department of paediatrics - PDF Free Download (2022)

1 department of paediatrics Annual Report

2 Paediatric Executive (left to right): Ben Vozzolo, Director of Operations; Rayfel Schneider, Education Chair; Marilyn Monk, VP, Clinical Programs; Meredith Irwin, Research Chair; Denis Daneman, Chair, Department of Paediatrics; Jeremy Friedman, Clinical Chair report from the chair table of contents 3 Report from the Chair 4 Profiles 6 Adolescent Medicine 12 Cardiology 26 Clinical and Metabolic Genetics 36 Clnical Pharmacology and Toxicology 44 Developmental Paediatrics 52 Endocrinology 60 Gastroenterology/Hepatology/Nutrition 70 Haematology/Oncology 92 Immunology and Allergy 98 Infectious Diseases 106 Neonatology 124 Nephrology 130 Neurology 148 Paediatric Emergency Medicine 156 Paediatric Medicine 168 Respiratory Medicine 178 Rheumatology 188 Transplant and Regenerative Medicine Centre 198 Grand Rounds 203 Awards Day 204 Promotions 205 Medical Education Training and Scholarship was a stellar year for the Department of Paediatrics in all spheres of academic activity: excellence in clinical care, education and education scholarship, and research. This report highlights some of the important milestones achieved during the year. Changes in departmental leadership Chaim Roifman stepped down as Head of the Division of Immunology/Allergy after almost 20 years in the position and has been replaced by Eyal Grunebaum, a clinician scientist in the division. O. Carter Snead III was replaced as Head of the Division of Neurology by Steven Miller who joined us Sep. 1, 2012 from UBC. Daune MacGregor s term as interim Head of the Division of Clinical and Metabolic Genetics ended with the arrival of Ronald Cohn from Johns Hopkins Sep. 1, These new Division Heads all come with the promise of ongoing development of their outstanding divisions to maintain and enhance their academic standing. We look forward to exciting times. At the same time, our profuse thanks go to Chaim, Carter and Daune for their enormous contributions. Promotion Ten members of the faculty were promoted to associate or full professor making 84 in the past six years. We congratulate all of the successful candidates in reaching this important milestone. Clinical excellence The demand for clinical care has increased there are more emergency and ambulatory care visits, more admissions, more complex patients. At the same time, the metrics of care have improved with shorter wait times in the ED, shorter lengths of stay, and better outcomes. These improvements are the result of close collaboration between medical and CHS leaders. The Academic Enterprise More than $47 million in research grants, numerous publications in high-impact journals, broad-based approach to research, hundreds of trainees, and more, all attest to a vibrant research environment. This will only get better when the Centre for Research & Learning opens a year from now. Our annual awards day session gets longer each year as more members of our faculty receive important awards from the department, the medical school, and national and international agencies. Finally, the department is in great shape thanks to the tireless efforts of all of our faculty, and the ongoing support of both the University of Toronto Faculty of Medicine and The Hospital for Sick Children. For this I remain immensely grateful. Sincerely, Denis Daneman, Department of Paediatrics Cover: Natasha Pisano department of paediatrics Annual Report

3 profiles overview Ronald Cohn Head, Division of Clinical and Metabolic Genetics and Co-Director of the Centre for Genetic Medicine Jeremy Friedman Associate Chair, Clinical Eyal Grunebaum Division of Immunology/Allergy Steven Miller Head, Division of Neurology, and Director of Centre for Brain and Behaviour Medicine has always been changing at a fast pace, but no aspect of medicine has changed as fast and fundamental as genetics. In the next few years, the concept of individualized medicine will become reality, and SickKids is preparing for the future with its new Centre for Genetic Medicine, co-led by Ronald Cohn and Stephen Scherer. Dr. Cohn joined SickKids in He received his medical degree from the University of Essen, Germany, and was the first combined resident in paediatrics and genetics at Johns Hopkins. He subsequently joined the faculty of the McKusick- Nathans Institute of Genetic Medicine at Johns Hopkins where he became the director of the worlds first multidisciplinary centre for hypotonia, and he was director of the medical genetics residency program at Johns Hopkins. As a leader of change, Dr. Cohn emphasizes the importance of active and open communication and a general willingness to look at genetic and genomic medicine with a different mindset. I will personally reach out to all divisions at SickKids and try to get to know every level of health-care provider to ensure that the Centre for Genetic Medicine represents a platform for all of SickKids and not only the divisional and research group of genetics. Jeremy Friedman will help to lead the Department of Paediatrics in its ongoing goal of providing outstanding clinical care to patients and their families. In collaboration with his colleagues he is continuously striving to find ways to improve care, achieve better outcomes, and share these improvements on a broad scale. My personal passion is to emphasize the art and science of medicine and how they are both integral to achieving the best care we can provide, Dr. Friedman says. My initial focus will be on quality improvement, patient safety, enhancing clinical systems and promoting clinical scholarship. Dr. Friedman graduated from the University of Cape Town, South Africa in He moved to Canada in 1987, spending his first three years as a general practitioner in rural Saskatchewan. He completed his residency training at SickKids from , and was appointed chief resident in his final year. He has worked as a general paediatrician on staff at SickKids since then, with a primary interest in hospitalist and complex care. He was appointed Division Head of Paediatric Medicine in 2004 and in 2011, was appointed Associate Paediatrician-in-Chief. He has won a number of departmental awards for clinical care, teaching and humanitarianism and was promoted to Professor in Eyal Grunebaum s goal is to support health-care providers who are treating patients with immune-related abnormalities. He will do this through education and by contributing to basic and translational research into immune abnormalities. His work complements the growing appreciation of the central role of the immune system in health and disease. Dr. Grunebaum will also address the significant increase of the frequency and severity of food allergy. His goal is to establish a centre of excellence that will use the superb research tools at SickKids to improve the care for children who are suffering from food allergy. To reach his goal, he will collaborate with families, physicians and researchers, recruit clinicians and scientists to help advance the field of food allergy, and share their findings with the medical and non-medical community. Dr. Grunebaum started his medical career at Hadassah Medical School, Hebrew University Jerusalem, Israel. He joined SickKids in 1998 for a fellowship in Immunology/Allergy, and in 2002 he joined the Division staff of Immunology and Allergy and Clinical Immunology as well as the Bone Blood and Marrow Transplantation Unit in the Division of Haematology/Oncology. In 2011 he became Director of Paediatric Allergy and Clinical Immunology Training at the University of Toronto. Steven Miller s vision is all about outcomes. While it s important to publish papers and attain research dollars, Dr. Miller believes that improving the care for children with neurological disease is the best outcome of all. To realize his vision, he needs a dynamic team, strong informatics infrastructure, and lots of partnerships inside and outside SickKids. He is busy building on all fronts and is excited to be at SickKids whose neurology department is outstanding with a depth and breadth that doesn t exist anywhere else. The SickKids Centre for Brain and Behaviour is the perfect place to improve patient care through research discovery, evaluate it and use evidence-based research to continue to move it forward, he says. The credit for the fine state of neurology at SickKids goes to his predecessor, Dr. Carter Snead, he says. A graduate of McGill School of Medicine, Dr. Miller studied and worked at University of California San Francisco in , after which he joined BC Children s Hospital, the Child & Family Research Institute and UBC as an associate professor of paediatrics and a Tier 2 Canada Research Chair in Neonatal Neuroscience. The focus of his collaborative research is using advanced imaging techniques to study brain development and injury in the newborn. 4 the hospital for sick children department of paediatrics Annual Report

4 ADOLESCENT MEDICINE overview Division Head Dr. Miriam Kaufman Full-Time Dr. Cathleen Steinegger Dr. Debra Katzman Part-Time Dr. Eudice Goldberg Dr. Kathy Hick Dr. Karen Leslie Dr. Rahul Saxena Dr. Joey Bonifacio Emeritus Dr. Miriam Rossi Clinical Fellows Dr. Nahed Jaber Subspecialty Residents Dr. Sarah Kearney Dr. Maya Kumar Dr. Alene Toulany Dr. Ian Chen Dr. Trisha Tulloch Administrative Staff Noreen Akhtar Joy Gouldbourne The Division of Adolescent Medicine at The Hospital for Sick Children is an interprofessional group committed to promoting and providing excellence in adolescent health care, and collaborating with local, national and international communities in the areas of patient care, training, education and research. The Division has five programs -- The Young Families Program (YFP); The Substance Abuse Program; The Good 2 Go Transition Program (G2G); and the SickKids Interprofessional Team for Youth (SITY) all of which continue to be active in clinical care, education, research, resource creation and international collaborations. The Young Families Program has integrated social paediatrics students into its clinical, research and educational programs. Research has increased and includes work in the areas of young fatherhood, contraception, literacy and sleep regulation. Moms on the Move is a two-phase project funded by Service Canada that combines employment and life skills training with job experience for adolescent mothers. During their work placement, the mothers continue to attend weekly workshops focusing on job maintenance and social support. The Eating Disorders Program had an extensive external review in 2011 that led to a major restructuring, with the program moving from a model of joint leadership from Adolescent Medicine and Psychiatry to a program with a single leader, supported by an executive committee that has representation from all players on the interprofessiional team. Throughout this, the program has continued to explore innovative treatments (and their outcomes) and to add to its research with studies on transition to adult care, evaluating a new refeeding protocol, and creating robust evidence to support a diagnostic addition to the DSM-5. department of paediatrics Annual Report

5 SITY is an important educational resource providing continuity clinics for fellows and teaching about a broad range of adolescent issues. Collaboration continues with clinics throughout the hospital, and data collection is complete for a study evaluating the model of providing adolescent health care for chronically ill teens that was developed at SickKids and has been in place for over 15 years. Plans to collaborate with more clinics are underway. SITY has optimized the involvement of a part-time psychologist and a post-doc who provide assessments, ongoing psychological treatment and important clinical and didactic teaching. The Substance Abuse Program provides continuum of care from outpatient to day treatment, incorporating best practices in clinical care, while providing learning for a variety of health professional trainees. A quality improvement project was initiated to ensure that effective assessment and treatment planning are in place for all patients. A validated assessment tool for concurrent disorders (GAIN-SS) was introduced, studied and is now being utilized. A study funded by Health Canada examining long-term health and psychosocial outcomes for day treatment patients was completed. Good 2 Go s new initiatives included a transition group focusing on early intervention for youth with special health care needs and their parents. With support from G2G, a number of programs became Good 2 Go including Marrow Failure and Neurosciences. Many G2G resources became readily accessible within the hospital, barcoded for easy access in EPC including the readiness checklist, transfer checklist and Transition Clinic assessment forms. Transition related research included a collaboration with McMaster Children s Hospital on the Transition to Adulthood with Cyberguide Evaluation (TRACE), a project to increase remote access to transition care and improve efficiency of transition support. G2G celebrated its fifth anniversary in a hospital-wide event including grand rounds and a half-day symposium. Honors and Awards Katzman, D: Inductee, 2012 McMaster University Alumni Gallery. Awarded May 2012 at McMaster University, Hamilton. Kaufman, M: Winner of 2012 Adelle Hoffman/Society for Adolescent Health and Medicine Visiting Professorship. Professorship took place in Sydney, Australia, November Publications Amaria K, Stinson J, Cullen-Dean G, Sappleton K, Kaufman M. Tools for addressing systems issues in transition. Health Care Quarterly 2011: 14 Special Issue (3): pp Cafazzo JA, Casselman M, Hamming N, Katzman DK, Palmert M. Design of an mhealth App for self-management of adolescent type I diabetes A pilot study. Medical Internet Journal of Research 2012: 8:14(3): pp e70. Fazeli PK, Katzman DK, Miller KK, Breggia A, Rosen CJ, Mendes N, Klibanski A, Misra M. Sclerostin levels and bone turnover markers in adolescents with anorexia nervosa and healthy adolescent girls. Bone 2012: June 15 (Epub ahead of print). Kaufman H, Horricks L, Kaufman M. Ethical considerations in transition. International Journal of Adolescent Medicine and Health 2011: 22 (4): pp Norris ML, Pinhas L, Nadeau P-O, Katzman DK. Delirium and refeeding syndrome in anorexia nervosa. International Journal of Eating Disorders 2011: October 19 (Epub ahead of print). Pinhas L, Morris A, Cosby R, Katzman DK. The incidence and age-specific presentation of restrictive eating disorders in children: A Canadian paediatric surveillance program study. Archives of Child and Adolescent Medicine 2011: 165 (10): pp Reeves S, Leslie K, Baker L, Egan-Lee E, Légaré F, Silver I, Rosenfield J, Hodges B, Curran V, Armson H, Kitto S. Study protocol for a pilot study to explore the determinants of knowledge use in a medical education context. Journal of Evaluative Clinical Practice 2012: May 15. Spencer M, Alexander L, Kaufman M. Geriatric MyHealth Passport: A pilot study of a portable health summary in an elderly population. Journal of the American Geriatrics Society 2011: 59 (11): pp Toulany A, Katzman DK, Goldberg E, Matlow A, Kaufman M. Chaperoning adolescents: A bygone era or modern medical necessity? Pediatrics and Child Health 2012: 17 (6): pp Turrell SL, Peterson-Badali M, Katzman DK. Consent to treatment in adolescents with anorexia nervosa. International Journal of Eating Disorders 2011: 44(8): pp Books and Chapters Bandiera G, Leslie K, Richardson D. Preparing good teachers for competency based education: The ABC s of faculty development. In: Education Design-A CanMEDS Guide for the Health Professions. (Sherbino J, Frank J, eds) Royal College of Physicians and Surgeons of Canada, Golden N, Katzman DK. Anorexia nervosa. In: American Academy of Pediatrics Textbook of Adolescent Health Care, First edition. (Fisher MM, Alderman EM, Kreipe RE, Rosenfeld WD, eds) The Donohue Group Inc, Katzman DK, Kanbur NÖ. Distress signals: Physical signs and symptoms associated with eating disorders. In: Restoring Our Bodies, Reclaiming Our Lives: Guidance and Reflections on Recovery from Eating Disorders, First edition. (Liu A, ed). Trumpeter Books, Boston, MA, Katzman DK, Neinstein LS. Adolescent medicine. In: Goldman s Cecil Medicine, 24th Edition. (Goldman L, Schafer AI, eds). Elsevier, Toulany A, Katzman DK. Eating disorders. In: When Something s Wrong: Ideas for Families. Healthy Minds Canada Handbook, Toronto, the hospital for sick children department of paediatrics Annual Report

6 Grants and Funding A centralized clinical outcome measurement of eating disorders in youth across Ontario: A multi-site partnership pilot study. Heinmaa M, Pinhas L, Katzman DK, Boachie A, Jasper J, Henderson K, Buchholz A, Spettigue W, Norris M, Barrowman N, Lafrance A. Psychiatry Endowment Fund. $25,000. Jan 2011 to Dec 2011 A pilot project to explore the determinants of knowledge use in a medical education context. Reeves S, Leslie K, Legare F, Silver I, Rosenfield J, Hodges B, Zwarenstein M, Armson H, Curran V. KT Canada SEED Funding Grant. $30, Balance in residency: Understanding how residents conceptualize balance. Kitney L, Leslie K. Paediatric Consultants Education Grant. $ CICH Profile: Knowledge synthesis to develop a chapter/module regarding children and youth mental health. Bennett K, Boyle M, Adair C, Boivin M, Boylan K, Cairney J, Cheung A, Davidson S, Georgiadis K, Hanvey H, Hertzman C, Katzman DK, LeBlanc J, Manassis K, Manion I, McGrath P, McLennan J, Pepler D, Peterman L, Popp J, Rush B, Santos R, Szatmari P. Canadian Institute of Health Research. $100,000. Mar 2012 to Feb 2013 Development and testing of a transition readiness questionnaire for youth with arthritis. Stinson J, Spiegel L, Watanabe K, Duffy L, Tucker, Stringer, Hazel B, Hochman J, Kaufman M. Canadian Arthritis Network: Discovery Advancement Program Grant. $50, to 2013 Development of an interprofessional adolescent health training program. Katzman DK, Spiegler B, Thompson G. Canadian Institute of Health Research Planning Grant. $24, to 2013 Defining competence for faculty developers. Leslie K, Baker L, Stubbs B, Panisko D, Meuser J, Walsh A, Marshall D, Lingard L, Myopoulous M. Royal College of Physicians and Surgeons of Canada Medical Education Grant. $44, Effects of anorexia nervosa on peak bone mass. Klibanski A, Katzman DK (Principal Investigator at SickKids site), Misra M, Herzog D, Levitsky L, Bouxsein M, Russell M. National Institutes of Health/CFAR Health Canada for Clinical Trials Application at SickKids site and for project at-large. $5,000, to 2016 Examining the long-term outcomes of adolescents receiving treatment in a substance abuse day treatment program. Leslie K, Brands B, Maser C. Health Canada. $37, to 2012 Examining transition from child to adult care in chronic kidney disease. Nicholas DB, Pinsk M, Molzahn A, Mantulak A, Vigneux A, MacNeil A, Dunseith C, Kaufman M. The Kidney Foundation of Canada. $100, to 2012 Family therapy in the treatment of adolescent anorexia nervosa. Agras S, Brandt H, Halmi K, Johnson C, Kaye W, Wilfley D, Woodside B, Pinhas L, Steinegger C, Katzman DK. National Institute of Mental Health. $6,250, to 2012 Further development of the MyHealth Passport. Good 2 Go Transition Program. John Deere Foundation. $50, to 2013 Improving the transition from paediatric to adult care services for adolescents with eating disorders. Toulany A, Dimitropoulos G, Samuels D, Leclerc A, Turrini T, Anderson J, Colton P, Kaufman M, Steinegger C. SickKids Paediatric International Patient Safety and Quality Care (PIPSQC) Funding. $4, Strengthening families for parents and youth Goldberg E. Health Canada Drug Strategy Community Initiatives Fund. $309, to 2012 Students and faculty as partners in innovation: The e-faculty Development Project. Leslie K, Law M, Ballon B, Yeung E, Shamji A, Lindzon G. University of Toronto Instruction Technology Incentive Fund (ITIF) ($15,000+matched funds of $15,000 from the Faculty of Medicine T-IME initiative) to 2013 The CHAPTER Study: Congenital heart adolescents participating in transition evaluation research. Mackie A, Rempel G, Macgill-Evans J, Nicholas D, Yasui Y, Kaufman M, McCrindle B, Anthony S, Sananes R, Kovacs A, Oechslin E. Canadian Institute of Health Research. $100, to 2013 The CHAPTER Study: Congenital heart adolescents participating in transition evaluation research. Mackie A, Rempel G, Macgill- Evans J, Nicholas D, Yasui Y, Kaufman M, McCrindle B, Anthony S, Sananes R, Kovacs A, Oechslin E. Heart and Stroke Foundation of Canada Grant-in-Aid. $358, to 2014 Understanding the role of Coach within faculty development. Leslie K, Ginsburg S, Egan-Lee E, Baker L, Reeves S. Royal College of Physicians and Surgeons of Canada Faculty Development Grant. $4, Vitamin D status in children and adolescents with anorexia nervosa. Steinegger C, Sherwood K, Segal S, Pinhas L, Katzman DK. Pediatric Consultants Partnership Grant for Creative Professional Activity. $9, to the hospital for sick children department of paediatrics Annual Report

7 cardiology overview Division Head Dr. Andrew Redington Full-Time Dr. Lee Benson Dr. Tim Bradley Dr. Rajiv Chaturvedi Dr. Jennifer Conway Dr. Anne Dipchand Dr. Andreea Dragulescu Dr. Mark Friedberg Dr. Fraser Golding Dr. Gil Gross Dr. Lars Grosse-Wortmann Dr. Robert Hamilton Dr. Edgar Jaeggi Dr. Paul Kantor Dr. Joel Kirsh Dr. Kyong-Jin Lee Dr. Brian McCrindle Dr. Luc Mertens Dr. Seema Mital Dr. Jennifer Russell Dr. Mike Seed Dr. Elizabeth Stephenson Part-Time Dr. Ra Han Dr. Anil Menon Dr. Lynne Nield Cross-Appointees Dr. Claudia Almeida Dr. Sarah Bowdin Dr. Jack Colman Dr. Rejane Dillenburg Dr. Alejandro Floh Dr. Tilman Humpl Dr. Alexandra Jevremovic Dr. Afrothite Kotsakis Dr. Tapas Mondal Dr. Norman Musewe Dr. Erwin Oechslin Dr. Steven Schwartz Dr. Ben Sivarajan Dr. Rachel Wald Dr. Roxanne Walker Dr. Eva Welisch Dr. Shi-Joon Yoo Clinical Fellows Majied Al-Ariefy Sharon Borik Frederic Dallaire Tom Einbinder Heynric Grotenhuis Steven Greenway Emilie Jean St. Michel Michael Mendelson Daryl Schantz Mohit Singla Maher Shalabi Catalina Tamayo Varsha Thakur Andrea Wan Subspecialty Residents Jonathan Forsey Dominic Hares Eias Kassem Marcelo Kozak Jillian Lewis Christian Turner Research Fellows Yoichi Iwamoto Kaarin Makikallio-Antila Naoki Ono Otto Rahkonen Dagmar Wertaschnigg Gyeong-Hee Yoo Adjunct Dr. Fred Keeley Administrative Staff Fe Berbano Theresa Braggs Esther Delea Katie Fox Judy Grundy Pamela Hardy Susan Jessop Shelley Kahn-Fortes Diana Kuzmanovski Deborah Lecourt Jacqueline Moonsammy Susan Morton Eleanor Paulino Ann Prosser The Division of Cardiology was formally established in the 1940s and is now one of the largest and most successful in the world. Its philosophy has remained unchanged since its inception over 60 years ago: to provide the highest quality care in an evidence-based environment of clinical innovation and scientific discovery. The Division is not only performing at the highest clinical standard, but is now better positioned to drive innovation and scientific discovery than at any other time in its history. Providing the highest quality care in an evidence-based environment of clinical innovation and scientific discovery. While the Division is an integral part of the Department of Paediatrics, it also forms a key part of the Labatt Family Heart Centre, which was officially opened two years ago. Incorporating the Division of Cardiology, the Division of Cardiac Surgery, the Division of Cardiac Critical Care and The Toronto Congenital Cardiac Centre for Adults, the heart centre is already one of the best organized and cohesive in the world. However, there is much more investment and development required before it will have reached its goals. Nonetheless, over the past decade, the divisional activities, in close collaboration with surgical and intensive care colleagues and all the other members of the heart centre, have contributed to our markedly improved preoperative diagnosis and postoperative outcomes for children undergoing cardiac surgery. In addition, our activities have led the field in development of therapeutic catheter techniques, made important contributions to our understanding of fetal cardiac medicine, established one of the largest paediatric cardiac transplantation centres worldwide, and won international recognition for contributions in the field of epidemiology of acquired and congenital heart disease and surgical outcomes. Each of these areas has provided the substrate for the large clinical research output of the Division which is underpinned by a world-class laboratory science program. One of the recent exciting clinical developments was the commissioning of an integrated suite of catheter laboratories and magnetic resonance scanning facilities. This unique fusion will allow simultaneous multi-mode research, investigation and treatment, setting the scene for MRI-guided interventional procedures and physiological measurements in children. department of paediatrics Annual Report

8 The cardiovascular research focus within SickKids Research Institute has made fundamental contributions to our understanding of the cellular biology and molecular genetics of pulmonary vascular bed, with recent diversification to include the study of the fetal myocyte, detailed study of the intercellular structure of the heart and vessels, electrophysiology of the developing heart and immunology of cardiac transplantation. High-quality basic and translational research, with an emphasis on direct clinical applicability, will continue to form the bedrock of our academic program. As an example, the biology of a new technique, remote ischemic preconditioning, has been explored and translated to clinical trials at SickKids. The heart centre is making a major investment in the development of biobanking facility in which DNA and tissue samples will be held for future fundamental, translational and clinical research investigations. Aside from this thematic funding, the heart centre supports many proposals for clinical innovation and seed-funding for scientific programs via its endowment, with the expectation that patient and family satisfaction will improve and the quality of research and its ability to garner external peer-reviewed funding will be enhanced. The Royal College-accredited Paediatric Cardiology Training Program is the largest in Canada and one of the largest and most sought after in North America. As such, its fellows have populated cardiology groups throughout the world, many going on to become leaders of our specialty. The single most important development of the last decade, however, has been the establishment of the Labatt Family Heart Centre. Following a series of important internal initiatives, the extraordinary generosity of the Labatt family has funded the redevelopment and future activities of the heart centre at SickKids. Honors and Awards Conway, J: ISHLT International Travelling Scholarship Award, Conway, J: Young Investigators Award, International Pediatric Transplant Association, Conway, J: Young Investigators Oral Abstract Winner, International Pediatric Transplant Association, McCrindle, B: Awarded the Dr. Harold N. Segall Award of Merit (Recognition of a significant contribution to the prevention of cardiovascular disease or the promotion of cardiovascular health in Canadians), Canadian Cardiovascular Society, Publications Abosoudah I, Mark L, Greenberg ML, Ness KK, Benson L, Nathan PC. Echocardiographic surveillance for asymptomatic late-onset anthracycline cardiomyopathy in childhood cancer survivors. Pediatric Blood and Cancer 2011: 57: pp Ackerman MJ, Priori SG, Williems S, Berul C, Brugada R, Calkins H, Camm AJ, Ellinor PT, Gollob M, Hamilton R, Hershberger RE, Judge DP, Le Marec H, McKenna WJ, Schulze-Bahr E, Semsarian C, Towbin JA, Watkins H, Wilde A, Wolpert C, Zipes DP. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies. European Society of Cardiology 2011: 13 (8): pp Ackerman MJ, Priori SG, Williems S, Berul C, Brugada R, Calkins H, Camm AJ, Ellinor PT, Gollob M, Hamilton R, Hershberger RE, Judge DP, Le Marec H, McKenna WJ, Schulze-Bahr E, Semsarian C, Towbin JA, Watkins H, Wilde A, Wolpert C, Zipes DP. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies. Heart Rhythm 2011: 8 (8): pp Ahmad N, Kantor PF, Grosse-Wortmann L, Seller N, Jaeggi ET, Friedberg MK, Mertens L. Influence of RV restrictive physiology on LV diastolic function in children after tetralogy of Fallot repair. Journal of American Society of Echocardiography 2012: (Epub ahead of print). Alghamdi MH, Grosse-Wortmann L, Ahmad N, Mertens L, Friedberg MK. Can simple echocardiographic measures reduce the number of cardiac magnetic resonance imaging studies to diagnose right ventricular enlargement in congenital heart disease? Journal of the American Society of Echocardiography 2012: 25 (5): pp Al-Saleh S, Dragulescu A, Manson D, Golding F, Traubici J, Mei-Zahav M, Maclusky IB, Faughnan ME, Carpenter S, Ratjen F. Utility of contrast echocardiography for pulmonary arteriovenous malformation screening in pediatric hereditary hemorrhagic telangiectasia. Journal of Pediatrics 2012: 160 (6): pp Alsoufi B, Manlhiot C, Al-Ahmadi M, Al-Halees Z, McCrindle BW, Mousa AY, Al-Heraish Y, Kalloghian A. Older children at the time of the Norwood operation have ongoing mortality vulnerability that continues after cavopulmonary connection. Journal of Thoracic and Cardiovascular Surgery 2011: 142 (1): pp Alsoufi B, Manlhiot C, Al-Ahmadi M, McCrindle BW, Kalloghlian A, Sibnini G, Bulbul Z, Al-Halees Z. Outcomes and associated risk factors for mitral valve replacement in children. European Journal of Cardiothoracic Surgery 2011: 40 (3): pp Amant F, Van Calsteren K, Halaska M, Mhallem M, Hui W, Lagae L, Willemsen M, Kapusta L, Van Calster B, Wouters H, Heyns L, Han S, Tomek V, Mertens L, Ottevanger P. Long-term cognitive and cardiac outcome after prenatal exposure to chemotherapy in children 18 months or older. Lancet Oncology 2012: 13 (3): pp Apitz C, Honjo O, Friedberg MK, Assad RS, Van Arsdell G, Humpl T, Redington AN. Beneficial effects of vasopressors on right ventricular function in experimental acute right ventricular failure in a rabbit model. The Thoracic and Cardiovascular Surgeon 2012: 60 (1): pp Atz AM, Zak V, Breitbart RE, Colan SD, Pasquali SK, Hsu DT, Lu M, Mahony L, Paridon SM, Puchalski MD, Geva T, McCrindle BW. Factors associated with serum brain natriuretic peptide levels after the Fontan procedure. Congenital Heart Disease 2011: 6 (4): pp Banka P, Sleeper LA, Atz AM, Cowley CG, Gallagher D, Gillespie MJ, Graham EM, Margossian R, McCrindle BW, Sang CJ, Williams IA, Newburger JW. Practice variability and outcomes of coil embolization of aortopulmonary collaterals before Fontan completion: A report from the Pediatric Heart Network Fontan cross-sectional study. American Heart Journal 2011: 162 (1): pp Banks I, Dipchand AI, Manlhoit C, Millar K, McCrindle B. Factors associated with low physical activity levels following pediatric cardiac transplantation. Pediatric Transplantation 2012: (Epub ahead of print). Bendiak GN, Kritzinger F, Dipchand AI, Ng VL, Solomon M, Grasemann H. Flow-independent exhaled nitric oxide parameters in pediatric lung and cardiac transplant recipients. Transplantation 2011: 91 (10): pp Bharucha T, Fernandes F, Slorach C, Mertens L, Friedberg MK. Measurement of effective aortic valve area using threedimensional echocardiography in children undergoing aortic balloon valvuloplasty for aortic stenosis. Echocardiography 2012: 29 (4): pp Bharucha T, Honjo O, Seller N, Atlin C, Redington A, Caldarone CA, Van Arsdell G, Mertens L. Mechanisms of tricuspid valve regurgitation in hypoplastic left heart syndrome: A case-matched echocardiographic-surgical comparison study. European Heart Journal of Cardiovascular Imaging 2012: (Epub ahead of print). Bharucha T, Mertens L. Are children with genetic disorders at increased risk of post-operative complications after cardiac surgery? Pediatric Critical Care Medicine 2011: 12 (5): pp Burchill L, Greenway S, Silversides C, Mital S. Genetic counseling in the adult with congenital heart disease: What is the role? Current Cardiology Reports 2011: 13 (4): pp Chahal N, Somji Z, Manlhiot C, Clarizia NA, Ashley J, Yeung RSM, McCrindle BW. Rate, associated factors and outcomes of recurrence of Kawasaki disease in Ontario, Canada. Pediatrics International 2012: 54 (4): pp Chaturvedi RR, Van Arsdell GS, Jacques F, Lee K-J. Delayed repair of right atrial isomerism with obstructed total anomalous pulmonary venous drainage by hybrid stent insertion between the left-sided atrium and pulmonary venous confluence. Journal of Thoracic and Cardiovascular Surgery 2012: (Epub ahead of print). Cherney DZI, Reich HN, Scholey JW, Lai V, Slorach C, Zinman B, Bradley TJ. Systemic hemodynamic function in humans with type 1 diabetes treated with protein kinase Cβ inhibition and renin angiotensin system blockade: A pilot study. Canadian Journal of Physiology and Pharmacology 2012: 90 (1): pp Chockalingam P, Jaeggi E, Rammeloo LA, Haak MC, Adama PN, Breur J, Bartelings MM, Clur SA, Blom NA. Maternal autoantibody mediated persistent sinus bradycardia. Journal of Rheumatology 2011: 38 (12): pp Collins N, Benson LN, Horlick EM. Iatrogenic ST elevation during percutaneous closure of a coronary artery fistula. Congenital Heart Disease 2012: 7: pp Connors S, Paredes FA, Parkash R, Krahn AD. Nonphysiologic noise early after defibrillator implantation in Canada: Incidence and implications. A report from the Canadian Heart Rhythm Society Device Committee. Heart Rhythm 2012: 9 (3): pp Conway J, Allain-Rooney T, McCrindle BW, Lau W, Dipchand AI. Development of donor-specific isohemagglutinins following pediatric ABO-incompatible heart transplantation. American Journal of Transplantation 2012: 12 (4): pp the hospital for sick children department of paediatrics Annual Report

9 Conway J, Christant MRK, West LJ, Ameduri RK, Alejos JC, Lamour JM, Das B, Gilbert D, Treler M, Naftel DC, Miyamoto SD. Outcomes of fetal listing for heart transplantation. Circulation 2011: 124 (Supp 21): A Conway J, Dipchand AI. Transplantation as a therapy for pediatric cardiomyopathies: When to list and risk factors for death while waiting. Progress in Pediatric Cardiology 2011: 32: pp Conway J, Manlhiot C, Allain-Rooney T, McCrindle BW, Lau W, Dipchand AI. Development of donor-specific isohemagglutinins following pediatric ABO-incompatible heart transplantation. American Journal of Transplant 2012: 12 (4): pp Conway J, Wilson GJ, Dipchand AI. A fatal case of eosinophilia following pediatric heart transplantation. Journal of Heart and Lung Transplantation 2011: 30 (5): pp Daly KP, Chakravarti SB, Foushee M, Naftel DC, Blume ED, Dipchand AI, Almond CS. Sudden death after pediatric heart transplantation: Analysis of data from the Pediatric Heart Transplantation Study Group. Journal of Heart and Lung Transplantation 2011: 30: pp Davies R, Russo M, Hong K, Mital S, Mosca R, Quaegebeur J, Chen J. Increased short-and long-term mortality at low-volume pediatric heart transplant centers: Should minimum standards be set? American Journal of Thoracic Surgery 2011: 253 (2): pp Dipchand AI. Decision-making in the face of endstage organ failure: High-risk transplantation and end-of-life care. Current Opinion in Organ Transplantation 2012: (Epub ahead of print). Dipchand AI. Transplanting across a positive crossmatch in pediatric heart transplantation. Pediatric Transplantation 2012: (Epub ahead of print). Dragulescu A, Golding F, Van Arsdell G, Caldarone C, Mertens L, Al-Radi O, Lee KJ. The impact of additional epicardial imaging to transesophageal echocardiography on intraoperative detection of residual lesions in congenital heart surgery. Journal of Thoracic and Cardiovascular Surgery 2012: 143 (2): pp Dragulescu A, Grosse-Wortmann L, Fackoury C, Mertens L. Echocardiographic assessment of right ventricular volumes: A comparison of different techniques in children after surgical repair of tetralogy of Fallot. European Journal of Echocardiography 2011: (Epub ahead of print). Dragulescu A, Grosse-Wortmann L, Fackoury C, Riffle S, Waiss M, Jaeggi E, Yoo S-J, Friedberg MK, Mertens L. Echocardiographic assessment of right ventricular volumes after surgical repair of tetralogy of Fallot: Clinical validation of a new echocardiographic method. Journal of the American Society of Echocardiography 2011: 24 (11): pp Dragulescu A, Kammache I, Fouillloux F, Amedro P, Métras D, Kreitmann B, Fraisse A. Long term results of pulmonary arteries rehabilitation in patients with pulmonary atresia, ventricular septal defect, pulmonary arteries hypoplasia and major aortopulmonary collaterals. Journal of Thoracic and Cardiovascular Surgery 2011: (Epub ahead of print). El-Khuffash A, Mertens L. Early treatment of the patent ductus arteriosus: Do we know what we are doing? Pediatric Critical Care Medicine 2012: 13 (3): pp Elmi M, Hickey EJ, Williams WG, Van Arsdell GS, Caldarone CA, McCrindle BW. Long-term tricuspid valve function following Norwood operation. Journal of Thoracic and Cardiovascular Surgery 2011: 142 (6): pp Essebag V, Champagne J, Birnie DH, Verma A, Healey JS, Simpson CS, Kus T, Thibault B, Mangat I, Tung S, Sterns L, Exner DV, Davies T, Coutu B, Crystal E, Stephenson EA, Connors S, Paredes FA, Parkash R, Krahn AD. Nonphysiologic noise early after defibrillator implantation in Canada: Incidence and implications: A report from the Canadian Heart Rhythm Society Device Committee. Heart Rhythm 2012: 9 (3): pp Everitt MD, Boyle GJ, Schechtman KB, Zheng J, Bullock EA, Kaza AK, Dipchand AI, Naftel DC, Kirklin JK, Canter CE. Early survival after heart transplant in young infants lowest after failed single ventricle palliation: A multi-institutional study. Journal of Heart and Lung Transplantation 2012: 31 (5): pp Farra N, Manickaraj AK, Ellis J, Mital S. Personalized medicine in the genomics era: Highlights from an international symposium on childhood heart disease. Future Medicine 2012: 8 (2): pp Feingold B, Zheng J, Law YM, Morrow WR, Hoffman TM, Schechtman KB, Dipchand AI, Canter CE. Risk factors for late renal dysfunction after pediatric heart transplantation: A multi-institutional study. Pediatric Transplant 2011: 15: pp Fernandes FP, Manlhiot C, Roche SL, Grosse-Wortmann L, Slorach C, McCrindle BW, Mertens L, Kantor PF, Friedberg MK. Impaired left ventricular myocardial mechanics and their relation to pulmonary regurgitation right ventricular enlargement and exercise capacity in asymptomatic children after repair of tetralogy of Fallot. Journal of American Society of Echocardiography 2012: 25 (5): pp Fernandes FP, Roche SL, Grosse-Wortmann L, Manlhiot C, Fackoury C, Slorach C, McCrindle BW, Mertens L, Kantor PF. Impaired right and left ventricular diastolic myocardial mechanics and filling in asymptomatic children and adolescents after repair of tetralogy of Fallot. European Heart Journal Cardiovascular Imaging 2012: (Epub ahead of print). Fernandes NM, Taylor GP, Manlhiot C, McCrindle B, Ho M, Miner S, Atkinson A, Jaeggi E, Nield L. The myocardium of fetuses with endocardial fibroelastosis contains fewer B and T lymphocytes than normal control myocardium. Pediatric Cardiology 2011: 32 (8): pp Ford-Jones L, Levin L, Schneider R, Daneman D, Social Pediatrics Working Group (Ford-Jones L, Atkinson A, Koren G, Jarvis A, Barozzino T, McCrindle B, Birken C, Hahn C, Uleryk E, Goldberg E, Johnstone J, Levin L, Feldman M, Shouldice M, Mistry N, Bernstein S, Cohen S, Yinge E, Cory E, Wittenberg J, Saxena R, Thompson G, Banerji A, Rossi M, Nulman I, Chapman LA, Williams R. A new social pediatrics elective - A tool for moving to life course developmental health. Journal of Pediatrics 2012: 160 (3): pp Forsey J, Mertens L. Atypical Kawasaki disease - A clinical challenge. European Journal of Pediatrics 2012: 171 (4): pp Friedberg M, Fernandes F, Roche S, Grosse-Wortmann L, Manlhiot C, Fackoury C, Slorach C, McCrindle B, Mertens L, Kantor P. Impaired right and left ventricular diastolic myocardial mechanics and filling in asymptomatic children and adolescents after repair of tetralogy of Fallot. European Heart Journal of Cardiovascular Imaging 2012: (Epub ahead of print). Frommelt PC, Guey LT, Minich LL, Bhat M, Bradley TJ, Colan SD, Ensing G, Gorentz J, Heydarian H, John JB, Lai WW, Levine J, Mahle WT, Miller S, Ohye RG, Pearson GD, Shirali G, Wong PC, Cohen MS, for the Pediatric Heart Network Investigators. Does initial shunt type for the Norwood procedure impact echocardiographic measures of cardiac size and function during infancy. The Single Ventricle Reconstruction Trial 2012: 125 (21): pp Gidding SS, Daniels SR, Kavey RE. Expert Panel on Cardiovascular Health and Risk Reduction in Youth: Developing the 2011 Integrated Pediatric Guidelines for Cardiovascular Risk Reduction. Pediatrics 2012: 129 (5): pp e1311-e1319. Gijsen VMGJ, Mital S, van Schaik RHN, Soldin OP, Soldin SJ, van der Heiden IP, Nulman I, Koren G, de Wildt SN. Age and CYP3A5 genotype affect tacrolimus dosing requirements after transplant in pediatric heart recipients. Journal of Heart and Lung Transplantation 2011: 30 (12): pp Gillis J, Burashnikov E, Antzelevitch C, Blaser S, Gross G, Turner L, Babul-Hirji R, Chitayat D. Long QT, syndactyly, joint contractures, stroke and novel CACNA1C mutation: Expanding the spectrum of Timothy syndrome. American Journal of Medical Genetics 2012: Part A 158A: pp Graham J, Banaschewski T, Buitelaar J, Coghill D, Danckaerts M, Dittmann RW, Dopfner M, Hamilton R, Hollis C, Hoitmann M, Rothenberger JA, Santosh P, Sergeant J, Simonoff E, Sonuga-Barke E, Wong ICK, Zuddas A, Steinhausen HC, Taylor E. European guidelines on managing adverse effects of medication for ADHD. European Child and Adolescent Psychiatry 2012: 20 (1): pp the hospital for sick children department of paediatrics Annual Report

10 Greenway SC, Yoo S-J, Baliulis G, Caldarone C, Coles J, Grosse-Wortmann L. Assessment of pulmonary veins after atriopericardial anastomosis by cardiovascular magnetic resonance. Journal of Cardiovascular Magnetic Resonance 2011: 21:13 (1): pp 72. Grosse-Wortmann L, Drolet C, Dragulescu A, Kotani Y, Chaturvedi R, Lee KJ, Mertens L, Taylor K, La Rotta G, Van Arsdell G, Redington A, Yoo SJ. Aortopulmonary collateral flow volume affects early postoperative outcome after Fontan completion: A multimodality study. Journal of Thoracic Cardiovascular Surgery 2012: (Epub ahead of print). Grosse-Wortmann L, Yoo SJ, Solomon M, Macgowan CK, Keshavjee S, Grasemann H. Cardiopulmonary magnetic resonance imaging in children after lung transplantation: Preliminary observations. Journal of Heart and Lung Transplantation 2011: 30 (11): pp Gruenwald CE, Manlhiot C, Abadilla AA, Kwok J, Maxwell S, Holtby HM, Brandao LR, Chan AK, Crawford-Lean L, Foreman C, Caldarone CA, Van Arsdell GS, McCrindle BW. Heparin brand is associated with postsurgical outcomes in children undergoing cardiac surgery. Annals of Thoracic Surgery 2012: 93 (3): pp Hamilton RM. Tinker, tailor: Foretelling the future in long QT syndrome. Heart Rhythm 2011: 8 (10): pp Hamilton RM. Risk vs. benefit for catheter ablation in the small child: When does the bough break? Canadian Journal of Cardiology 2011: 27 (4): pp 523 e1-2. Harrison JJ, Schiff JR, Coursol CJ, Daley CJ, Dipchand AI, Heywood NM, Keough-Ryan TM, Keown PA, Levy GA, Lien DC, Wichart JR, Cantarovich M. Generic immunosuppression in solid organ transplantation: A Canadian perspective. Transplantation 2012: 93 (7): pp Henderson HT, Canter CE, Mahle WT, Dipchand AI, LaPorte K, Schechtman KB, Zheng J, Asante-Korang A, Singh SK, Kanter KR. ABO-incompatible heart transplantation. Analysis of the pediatric heart transplant study (PHTS) database. Journal of Heart and Lung Transplantation 2012: 31 (2): pp Hickey E, Nosikova Y, Zhang H, Caldarone C, Benson L, Redington A, Van Arsdell GS. Very low-birth-weight infants with congenital cardiac lesions: Is there merit in delaying intervention to permit growth and maturation? Journal of Thoracic and Cardiovascular Surgery 2012: 143 (1): pp Ho J, Panagiotopoulos C, McCrindle BW, Grisaru S, Pringsheim T, for the CAMESA Guideline Group. Management recommendations for metabolic complications associated with second generation antipsychotic use in children and youth. Journal of the Canadian Academy of Child and Adolescent Psychiatry 2011: 20 (3): pp Honjo O, Atlin C, Mertens L, Al-Radi O, Redington A, Caldarone C, Van Arsdell G. Atrioventricular valve repair in patients with functional single ventricle physiology: Impact of ventricular function and morphology, valve morphology and mechanism of insufficiency on survival and reintervention. Journal of Thoracic Cardiovascular Surgery 2011: 142 (2): pp e2. Horenstein MS, Idriss SF, Hamilton RM, Kanter RJ, Webster PA, Karpawich PP. Efficacy of signal-averaged electrocardiography in the young orthotopic heart transplant patient to detect allograft rejection. Pediatric Cardiology 2012: 27 (5): pp Ilina MV, Kepron CA, Taylor GP, Perrin, DG, Kantor PF, Somers G. Undiagnosed heart disease leading to sudden unexpected death in childhood: A retrospective study. Pediatrics 2011: 128 (3): pp Kantor PF, Rusconi P, Lipshultz S, Mital S, Wilkinson JD, Burch M. Current applications and future needs for biomarkers in pediatric cardiomyopathy and heart failure: Summary from the second international conference on pediatric cardiomyopathy. Progress in Pediatric Cardiology 2011: 32 (1): pp Karamlou T, Diggs BS, McCrindle BW, Welke KF. A growing problem: Maternal death and peripartum complications are higher in women with grown-up congenital heart disease. Annals of Thoracic Surgery 2011: 92 (6): pp ; Discussion pp Kavey REW, Simons-Morton DG, dejesus JM, McCrindle B. (supplement editors). Expert panel on integrated guidelines for cardiovascular health and risk reduction in children and adolescents: Summary report. Pediatrics 2011: 128 (Supplement 5): pp S1-S44. Kaza AK, Colan SD, Jaggers J, Lu M, Atz AM, Sleeper LA, McCrindle BW, Lambert LM, Margossian R, Lacro RV, Richmond ME, Natarajan S, Minich LL, Pediatric Heart Network Investigators. Surgical interventions for atrioventricular septal defect subtypes: The Pediatric Heart Network Experience. Annals of Thoracic Surgery 2011: 92 (4): pp Gziri M, Amant F, Debieve F, Van Calsteren K, De Catte L, Mertens L. Effects of chemotherapy during pregnancy on the maternal and fetal heart. Prenatal Diagnosis 2012: 17: pp 1-6. Hahn CD, Manlhiot C, Schmidt MR, Nielsen TT, Redington AN. Remote ischemic per-conditioning: A novel therapy for evolving stroke? Stroke 2011: 42 (10): pp Hamilton RM, Rosenthal E, Hulpke-Wette M, Graham JG, Sergeant J. Cardiovascular considerations of attention deficit hyperactivity disorder medications: A report of the European Network on Hyperactivity Disorders work group, European Attention Deficit Hyperactivity Disorder Guidelines Group on attention deficit hyperactivity disorder drug safety meeting. Cardiology in the Young 2012: 22 (1): pp Hickey EJ, Caldarone CA, McCrindle BW. Left ventricular hypoplasia: A spectrum of disease involving the left ventricular outflow tract, aortic valve and aorta. Journal of the American College of Cardiology 2012: 3: 59 (Suppl): pp S43-S54. Hickey EJ, McCrindle BW, Larsen SH, Benson L, Manlhiot C, Caldarone CA, Van Arsdell GS, McCrindle BM, Williams WG. Hypertrophic cardiomyopathy in childhood: Disease natural history, impact of obstruction and its influence on survival. Annals of Thoracic Surgery 2012: 93 (3): pp Hickey EJ, Veldtman G, Bradley TJ, Gengsakul A, Webb G, Williams WG, Manlhiot C, McCrindle BW. Functional health status in adult survivors of operative repair of tetralogy of Fallot. American Journal of Cardiology 2012: 109 (6): pp Jacques F, Kotani Y, Deva DP, Moller T, Oechslin E, Horlick E, Osten O, Crean A, Benson LN, Wintersperger B, Caldarone CA. Left main coronary artery compression long term after repair of conotruncal lesions: The bow string conduit. Annals of Thoracic Surgery 2012: (Epub ahead of print). Jaeggi ET, Carvalho JS, De Groot E, Api O, Clur SA, Rammeloo L, McCrindle BW, Ryan G, Manlhiot C, Blom NA. Comparison of transplacental treatment of fetal supraventricular tachyarrhythmias with digoxin, flecanide and sotalol: Results of a non-randomized multicenter study. Circulation 2011: 124 (16): pp Jegatheeswaran A, Oliveira C, Batsos C, Moon-Grady AJ, Silverman NH, Hornberger LK, Coyte P, Friedberg MK. Costs of prenatal detection of congenital heart disease. American Journal of Cardiology 2011: 108 (12): pp Kirsh JA. Orbits and implants: Trends in indications, utilization and complications in pediatric device therapy. Heart Rhythm 2012: 9 (2): pp Koopman LP, McCrindle BW, Slorach C, Chahal N, Hui W, Sarkola T, Manliot C, Jaeggi ET, Bradley TJ, Mertens L. Interaction between myocardial and vascular changes in obese children: A pilot study. Journal of the American Society of Echocardiography 2012: 25 (4): pp Kozak M, Mertens L, Ho A, Yoo S, Grosse-Wortmann L. Quantification of ductal blood flow with magnetic resonance imaging in newborns with obstructive left heart disease. Journal of Cardiovascular Magnetic Resonance 2012: 1: 14 Suppl 1: pp the hospital for sick children department of paediatrics Annual Report

11 Krishnamurthy S, Adhisivam B, Hamilton RM, Baskin B, Biswal N, Kumar M. Arrhythmogenic dilated cardiomyopathy due to a novel mutation in the desmoplakin gene. Indian Journal of Pediatrics 2011: 78 (7): pp Kwong W, Neilson AL, Chiu CC, Gross GJ, Hamilton RM, Soucie L, Stephenson EA, Kirsh JA. The effect of NavX on fluoroscopy times in pediatric catheter ablation. Journal of Interventional Cardiac Electrophysiology 2012: 33 (1): pp Lee KJ, Yoo SJ, Holtby H, Grant B, Mroczek D, Wong D, Grosse-Wortmann L, Benson LN, Chaturvedi RR. Acute effects of the ACE inhibitor enalaprilat on the pulmonary, cerebral and systemic blood flow and resistance after the bidirectional cavopulmonary connection. Heart 2011: 97: pp Lee W, Yoo S-J, Roche SL, Kantor P, Van Arsdell G, Park EA, Redington A, Grosse-Wortmann L. Determinants and functional impact of restrictive physiology after repair of tetralogy of Fallot: New insights from magnetic resonance imaging. International Journal of Cardiology 2012: (Epub ahead of print). Mahle WT, Martin GR, Beekman RH III, Morrow WR, Rosenthal GL, Snyder CS, Minich LL, Mital S, Towbin JA, Tweddell JS. Endorsement of health and human services recommendation for pulse oximetry screening for critical congenital heart disease. Pediatrics 2012: 129 (1): pp Manlhiot C, Christie E, McCrindle BW, Rosenberg H, Chahal N, Yeung RS. Complete and incomplete Kawasaki disease: Two sides of the same coin. European Journal of Pediatrics 2012: 171 (4): pp Manlhiot C, McCrindle BW, Menjak IB, Yoon H, Holtby HM, Brandao LR, Chan AK, Schwartz SM, Sivarajan VB, Crawford-Lean L, Foreman C, Caldarone CA, Van Arsdell GS, Gruenwald CE. Longer blood storage is associated with suboptimal outcomes in high-risk pediatric cardiac surgery. Annals of Thoracic Surgery 2012: 93 (5): pp Manlhiot C, Menjak IB, Brandao LR, Gruenwald CE, Schwartz SM, Sivarajan VB, Yoon H, Maratta R, Carew CL, McMullen JA, Clarizia NA, Holtby HM, Williams S, Caldarone CA, Van Arsdell GS, Chan AK, McCrindle BW. Risk, clinical features, and outcomes of thrombosis associated with pediatric cardiac surgery. Circulation 2011: 124 (14): pp Mertens L, Seri IJ, Arlettaz R, Barker P, McNamara P, Moon-Grady AJ, Coon P D, Noori S, Simpson J, Lai W. Targeted neonatal echocardiography in the neonatal intensive care unit: Practice guidelines and recommendations training. Writing group of the American Society of Echocardiography (ASE) in collaboration with the European Association of Echocardiography (EAE) the Association for European Pediatric Cardiologists (AEPC) and the American Academy of Pediatrics (AAP). European Journal of Echocardiography 2011: 12 (10): pp Mertens L, Seri IJ, Arlettaz R, Barker P, McNamara P, Moon-Grady AJ, Coon PD, Noori S, Simpson J, Lai W. Targeted neonatal echocardiography in the neonatal intensive care unit: Practice guidelines and recommendations training. Writing group of the American Society of Echocardiography (ASE) in collaboration with the European Association of Echocardiography (EAE) the Association for European Pediatric Cardiologists (AEPC) and the American Academy of Pediatrics (AAP). Journal of the American Society of Echocardiography 2011: 24 (10): pp Mertens L, ASE Writing Group on Targeted Neonatal Echocardiography. Neonatologists and echocardiography: Time to move on. Journal of the American Society of Echocardiography 2012: 25 (3): pp Mertens L, ASE Writing Group on Targeted Neonatal Echocardiography. Assessment of right ventricular function in targeted neonatal echocardiography: Reply to letter by Koestenbergery and Ravekes. Journal of the American Society of Echocardiography 2012: 25 (2): pp 244. Mertens L. Neonatal echocardiography-different approaches in different continents: Reply to a letter by Evans and Kluckow. Journal of the American Society of Echocardiography 2012: 25 (2): pp Michelsen MM, Støttrup NB, Schmidt MR, Løfgren B, Jensen RV, Tropak M, St-Michel EJ, Redington AN, Bøtker HE. Exercise-induced cardioprotection is mediated by a bloodborne, transferable factor. Basic Research Cardiology 2012: 107 (3): pp 1-9. Millar K, Manlhiot C, Yeung RS, Somji Z, McCrindle BW. Corticosteroid administration for patients with coronary artery aneurysms after Kawasaki disease may be associated with impaired regression. International Journal of Cardiology 2012: 154 (1): pp Monagle P, Cocharane A, Roberts R, Manlhiot C, Weintraub R, Szechtman B, Hughes M, Andrew M, McCrindle BW. A multicenter randomized trial of heparin/warfarin versus acetylsalicylic acid as primary thromboprophylaxis for two years after Fontan procedure in children. Journal American College of Cardiology 2011: 58 (6): pp Moola FJ, Faulkner GEJ, Kirsh JA, Schneiderman J. Developing exercise interventions for children with cystic fibrosis and congenital heart disease: Learning from their parents. Psychology of Sport and Exercise 2011: 12: pp Morgan GJ, Waragai T, Eastaugh L, Chaturvedi RC, Lee K-J, Benson L. The fellows stitch: Large caliber venous hemostasis in pediatric practice. Catheterization and Cardiovascular Interventions 2011: 80 (1): pp Morinis J, Maguire J, Khovratovich M, McCrindle BW, Parkin PC, Birken CS. Paediatric obesity research in early childhood and the primary care setting: The TARGet Kids! Research Network. International Journal of Environmental Research and Public Health 2012: 9 (4): pp Newburger JW, Sleeper LA, Bellinger DC, Goldberg CS, Tabbutt S, Lu M, Mussatto KA, Williams IA, Gustafson KE, Mital S, Pike N, Sood E, Mahle WT, Cooper DS, Dunbar- Masterson C, Krawczeski CD, Lewis A, Menon S, Pemberton VL, Ravishankar C, Atz TW, Ohye RG, Gaynor JW for the Pediatric Heart Network Investigators. Early developmental outcome in children with hypoplastic left heart syndrome and related anomalies: The single ventricle reconstruction trial. Circulation 2012: 125 (17): pp O Gorman CS, Syme C, Bradley TJ, Hamilton JK, Mahmud FH. Impaired endothelial function in pediatric patients with Turner syndrome and healthy controls: A case-control study. International Journal of Pediatric Endocrinology 2012: (1): pp 5. Pahl E, Sleeper LA, Canter CE, Hsu DT, Lu M, Webber SA, Colan SD, Kantor PF, Everitt MD, Towbin JA, Jeffrieds JL, Kaufman BD, Wilkinson JD, Lipshultz SE, Pediatric Cardiomyopathy Registry Investigators. Incidence of and risk factors for sudden cardiac death in children with dilated cardiomyopathy: A report from the Pediatric Cardiomyopathy Registry. Journal of American College of Cardiology 2012: 7 (59): pp Pasquali SK, He X, Mohamad Z, McCrindle BW, Newburger JW, Li JS, Shah SS. Trends in endocarditis hospitalizations at US children s hospitals: Impact of the 2007 American Heart Association Antibiotic Prophylaxis Guidelines. American Heart Journal 2012: 163 (5): pp Pharis SC, Conway J, Warren AE, Bullock A, Mackie AS. The impact of 2007 Infective Endocarditis Prophylaxis Guidelines on the practice of pediatric and adult congenital cardiologists. American Heart Journal 2011: 161 (1): pp Porter A, Yamada D, Conway J, LeBlanc J, Shea S, Hancock Friesen C, Warren A. Prevelance of a positive screening score for attention deficit hyperactivity disorder in children with early repair of congenital heart disease. Canadian Journal of Cardiology 2011: 27: S the hospital for sick children department of paediatrics Annual Report

12 Prakash A, Lacro RV, Sleeper LA, Minich LL, Colan SD, McCrindle BW, Covitz W, Golding F, Hlavacek AM, Levine JC, Cohen MS. Challenges in echocardiographic assessment of mitral regurgitation in children after repair of atrioventricular septal defect. Pediatric Cardiology 2012: 33 (2): pp Ratnapalan S, Brown K, Benson L. Children presenting with acute pericarditis to the emergency department. Pediatric Emergency Care 2011: 27: pp Rauch R, Grosse-Wortmann L, Jones J, Burrill E, Welisch E, Rosenberg HC, Norozi K. Shone s complex and levoatriocardinal vein: A rare association. Journal of the American College of Cardiology 2012: 14: 59 (7): pp 698. Redington KL, Disenhouse T, Strantzas SC, Gladstone R, Wei C, Tropak MB, Dai X, Manlhiot C, Li J, Redington AN. Remote cardioprotection by direct peripheral nerve stimulation and topical Capsaicin is mediated by circulating humoral factors. Basic Research in Cardiology 2012: 107 (2): pp 241. Rhodes J, Margossian R, Sleeper LA, Barker P, Bradley TJ, Lu M, Fogel M, Harris MA, Lai WW, Powell AJ, Puchalski MD, Shirali G, Colan SD, for the Pediatric Heart Network Investigators. Non-geometric echocardiographic indices of ventricular function in patients with a Fontan circulation. Journal of the American Society of Echocardiography 2011: 24 (11): pp Sajan I, Manlhiot C, Reyes J, McCrindle BW, Humpl T, Friedberg MK. Pulmonary arterial capacitance in children with idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with congenital heart disease: Relation to pulmonary vascular resistance, exercise capacity, and survival. American Heart Journal 2011: 162 (3): pp Sananes R, Manlhiot C, Kelly E, Hornberger LK, Williams WG, MacGregor D, Buncic R, McCrindle BW. Neurodevelopmental outcomes following open heart surgery before 3 months of age. Annals of Thoracic Surgery 2012: 93 (5): pp Sarkola T, Abadilla AA, Chahal N, Jaeggi E, McCrindle BW. Feasibility of very high resolution ultrasound to assess elastic and muscular arterial wall morphology in adolescents attending an outpatient clinic for obesity and lipid abnormalities. Atherosclerosis 2011: 219 (2): pp Sarkola T, Redington AN, Slorach C, Hui W, Bradley T, Jaeggi E. Assessment of vascular phenotype using a novel very-highresolution ultrasound technique in adolescents after aortic coarctation repair and/or stent implantation: Relationship to central haemodynamics and left ventricular mass. Heart 2011: 97 (21): pp Schechter-Finkelstein T, Doyle J, Gassas A, Weitzman S, Alexander S, Dipchand AI, Grant D, Avitzur Y, Ali M, Pollock S. Hematopoetic stem cell transplantation following solid organ transplantation in children. Bone Marrow Transplantation 2011: 46: pp Semsarian C, Hamilton RM. Key role of the molecular autopsy in sudden unexpected death. Heart Rhythm 2012: 9 (1): pp Shariat M, Grosse-Wortmann L, Windram J, Yoo SJ. Pulmonary vein flow pattern in children with bidirectional cavopulmonary connection or Fontan circuit. Pediatric Radiology 2012: 42 (2): pp Stieber NA, Gilmour S, Morra A, Rainbow J, Robitaille S, Van Arsdell GS, McCrindle BW, Gibson BE, Longmuir PE. Feasibility of improving the motor development of toddlers with congenital heart defects using a home-based intervention. Pediatric Cardiology 2012: 33 (4): pp Sun AM, AlHabshan F, Cheung M, Bronzetti G, Redington AN, Benson LN, Macgowan C, Yoo SJ. Delayed onset of tricuspid valve flow in repaired tetralogy of Fallot: An additional mechanism of diastolic dysfunction and intraventricular dyssynchrony. Journal Cardiovascular Magnetic Resonance 2011: 13: pp 43. Tabbutt S, Ghanayem N, Ravishankar C, Sleeper L, Cooper D, Frank D, Lu M, Pizarro C, Frommelt P, Goldberg C, Graham E, Krawczeski C, Lai W, Lewis A, Kirsh J, Mahony L, Ohye R, Simsic J, Lodge A, Spurrier E, Stylianou M, Laussen P. Risk factors for hospital morbidity and mortality after the Norwood procedure: A report from the Pediatric Heart Network single ventricle reconstruction trial. Journal of Thoracic and Cardiovascular Surgery 2012: (Epub ahead of print). Tao J, Zhu W, Li Y, Xin P, Li J, Liu M, Li J, Redington AN, Wei M. Apelin-13 protects the heart against ischemia-reperfusion injury through inhibition of ER-dependent apoptotic pathways in a time-dependent fashion. American Journal of Physiology Heart Circulation Physiology 2011: 301 (4): pp H Taylor K, La Rotta G, McCrindle BW, Manlhiot C, Redington A, Holtby H. A comparison of cardiac output by thoracic impedance and direct fick in children with congenital heart disease undergoing diagnostic cardiac catheterization. Journal of Cardiothoracic Vascular Anaesthesia 2011: 25 (5): pp Taylor K, Manlhiot C, McCrindle BW, Grosse-Wortmann L, Holtby HM. Poor accuracy of noninvasive cardiac output monitoring using bioimpedance cardiography (Physioflow ) compared to magnetic resonance imaging in pediatric patients. Anesthesia and Analgesia 2012: 114 (4): pp Timberlake K, Kantor PF. Pharmacologic therapy of heart failure in children: (Part of a special series on paediatric pharmacology, guest edited by Gianvincenzo Zuccotti, Emilio Clementi, and Massimo Molteni). Pharmacological Research 2011: 64 (5): pp van Geldorp IE, Delhaas T, Gebauer RA, Frias P, Tomaske M, Friedberg MK, Tisma-Dupanovic S, Elders J, Früh A, Gabbarini F, Kubus P, Illikova V, Tsao S, Blank AC, Hiippala A, Sluysmans T, Karpawich P, Clur SA, Ganame X, Collins KK, Dann G, Thambo JB, Trigo C, Nagel B, Papagiannis J, Rackowitz A, Marek J, Nürnberg JH, Vanagt WY, Prinzen FW, Janousek J, Working Group for Cardiac Dysrhythmias and Electrophysiology of the Association for European Paediatric Cardiology. Impact of the permanent ventricular pacing-site on LV function in children: A retrospective survey. Heart 2011: 97 (24): pp Van Horn L, Banks L, Vincent E, McCrindle BW. The importance of physical activity for children. Pediatric Annals 2012: 41 (2): pp Vanderlaan RD, Conway J, Manlhiot C, McCrindle BW, Dipchand AI. Enhanced exercise performance and survival associated with evidence of autonomic reinnervation in pediatric heart transplant recipients. American Journal of Transplantation 2012: 12 (8): pp Vaslangiacomo Buechel E, Mertens L. Imaging the right heart The use of integrated multimodality imaging. European Heart Journal 2012: 33 (8): pp Villafane J, Young ML, Maury P, Wolpert C, Anttonen O, Hamilton R, Kannankeril PJ, Fischbach PS. Short QT syndrome in a pediatric patient. Pediatric Cardiology 2012: 30 (6): pp Walsh MA, Merat M, La Rotta G, Joshi P, Joshi V, Tran T, Jarvis S, Caldarone CA, Van Arsdell GS, Redington AN, Kavanagh BP. Airway pressure release ventilation improves pulmonary blood flow in infants after cardiac surgery. Critical Care Medicine 2011: 39 (12): pp Waragai T, Morgan G, Ralston T, Chaturvedi R, Lee K-J, Benson L. Vascular hemostasis bandage compared to standard manual compression after cardiac catheterization in children. Catheterization and Cardiovascular Interventions 2011: 78 (2): pp Weber RM, Ayala-Arnez R, Atiyah M, Etoom Y, Manlhiot C, McCrindle BW, Hickey EJ, Jaeggi ET, Nield LE. Fetal echocardiographic assessment of borderline small left ventricle scan predicts the need for postnatal intervention. Cardiology in the Young 2012: 5: pp 1-9. Wengle JG, Hamilton JK, Manlhiot C, Bradley TJ, Katzman DK, Sananes R, Adeli K, Birken CS, Abadilla AA, McCrindle BW. The golden keys to health: A healthy lifestyle intervention with randomized individual mentorship for overweight and obesity in adolescents. Pediatric and Child Health 2011: 16 (8): pp the hospital for sick children department of paediatrics Annual Report

13 Wertaschnigg D, Jaeggi M, Chitayat D, Shannon P, Ryan G, Thomson M, Yoo SJ, Jaeggi E. Prenatal diagnosis and outcome of absent pulmonary valve syndrome. Contemporary single center experience and review of the literature. Ultrasound in Obstetrics and Gynecology 2012: (Epub ahead of print). Windram J, Grosse-Wortmann L, Shariat M, Greer ML, Crawford MW, Yoo SJ. Cardiovascular MRI without sedation or general anesthesia using a Feed-and-Sleep technique in neonates and infants. Pediatric Radiology 2012: 42 (2): pp Ye L, Zhu W, Backx PH, Cortez MA, Wu J, Chow Y-H, McKerlie C, Wang A, Tsui L-C, Gross GJ, Hu J. Arrhythmia and sudden death associated with elevated cardiac chloride channel activity. Journal of Cellular and Molecular Medicine 2011: 15: pp Yoon G, Soman T, Wilson J, George K, Mital S, Dipchand AI, McCabe J, Logan W, Kantor P. Cardiac transplantation in Friedreich ataxia. Journal of Child Neurology 2012: (Epub ahead of print). Zaoutis T, Webber S, Naftel DC, Chrisant MA, Kaufman B, Pearce FB, Spicer R, Dipchand AI, on behalf of the Pediatric Heart Transplant Study Investigators. Invasive fungal infections in pediatric heart transplant recipients: Incidence, risk factors, and outcomes. Pediatric Transplant 2011: 15 (5): pp Books and Chapters Apitz C, Redington AN. Right ventricular assessment and function. In: Paediatric Pulmonary Hypertension (Beghetti M, Barst RJ, Berger RMF, Humpl T, Ivy D, Schulze-Neick I, eds). Elsevier 2011: pp Mertens L. Ultrasound in paediatric cardiology State of the art. In: Ultrasound in Clinical Diagnosis: From Pioneering Developments in Lund to Global Application in Medicine (Eklöf B, Lindström K, Persson S, eds). Oxford University Press, London 2012: pp Mital S. Mechanical circulatory support in infants and children. In: Mechanical Circulatory Support in Infants and Children. (Loebe M, ed) McGraw Hill 2011: pp Punn R, Friedberg MK, Silverman NH. Echocardiographyincluding fetal. In: Pediatric Cardiovascular Medicine, Second Edition. (Hoffman, Moller, eds) 2012: pp Grants and Funding A pharmacogenetic trial of tacrolimus after pediatric transplantation. Mital S. SickKids Transplant Centre Catalyst Grant. $150,000 CAD. Jan to Dec A proof of principal randomized controlled trial of chronic remote ischemic conditioning to modify post-mi remodeling. Redington AN, Overgaard C, Dzavik V, Farkouh M, Crean A. Canadian Institute of Health Research. $310,156 CAD. Jan to Dec A randomised, controlled, multidose, multicentre, adaptive phase II/III study in infants with proliferating infantile hemangiomas requiring systemic therapy to compare four regimens of propranolol (1 or 3 mg/kg for 3 or 6 months) to placebo (double blind). Pope E, Bradley TJ, Chakkittakandiyil A, Lara-Corrales I. Pierre Fabre Dermatology. $373,044 CAD. Jan to Dec A randomized controlled trial of hybrid and Norwood surgical strategies for single ventricle congenital heart disease. McCrindle B, Caldarone C. Heart and Stroke Foundation of Ontario. $228,234 CAD. Jan to Dec Adolescent type I diabetes cardio renal intervention trial (AdDIT) - Canada Site: (1) Randomized controlled trial; (2) Follow-up of non-randomized low risk subjects; (3) Ancillary study - urinary proteomics in adolescents with type 1 diabetes. Daneman D, Mahmud FH, Sochett EB, Curtis JR, Moineddin, Bradley TJ, Cherney DZI, Reich HN, Scholey JW. Juvenile Diabetes Research Foundation - Canadian Clinical Trials Network. $3,657,088 CAD. Jan to Dec Adolescent type 1 diabetes cardio-renal intervention trial. (AdDIT). Dunger DB, Daneman D, Curtis JR, Mahmud FH, Bradley TJ. British Heart Foundation 07CC14. $30,000 CAD. Jan to Dec Biologic determinants of right ventricular remodeling in tetralogy of Fallot. Mital S, Redington AN, McCrindle, B, Mertens L. Heart and Stroke Foundation of Canada. $150,634 CAD. Jan to Dec Can post-operative biventricular pacing improve cardiac output and decrease mechanical ventilation in children with electro-mechanical dyssynchrony after surgical repair of atrioventricular septal defect? Friedberg M, Stephenson E. Labatt Family Heart Centre Innovations Funding. $48,640 CAD. Jan to Dec Cardiovascular biomarker discovery in disease and development through predictive precision proteomics. Keller G, Liu P, Mital S. Ontario Research Fund - Global Leadership Round in Genomics and Life Sciences Competition (GL2 Competition). $6,641,774 CAD (Mital: $280,000 CAD). Jan to Dec Childhood cancer Late effects of treatment. Novel approaches to the prediction, diagnosis and treatment of cardiac late effects in survivors of childhood cancer. Nathan P, Mital S. Canadian Institutes for Health Research Team Grant. $2,797,749 CAD Genomics Core: $702,107 CAD. Jan to Dec CIHR emerging team in ARVC research. Hamilton R. Canadian Institute of Health Research. $2,499,925 CAD. Dec to Nov Clinical assessment of thrombosis in children and heart surgery: The CATCH study. McCrindle B, Mital S. Canadian Institutes for Health Research. $1,514,330 CAD (Mital: $161,277 CAD). Jan to Dec Closure of muscular ventricular septal defects with the Amplatzer muscular VSD occluder. Benson L, Lee KJ, Chaturvedi R, Stanton-Meijer B. AGA Medical Corporation (St. Jude Medical). $4,991 US/patient over 5 years. Jan to Dec Comparison of immune profiles of idiopathic dilated cardiomyopathy and maternal anti-ro and anti-la antibody associated endocardial fibroelastosis in the fetal and neonatal heart. Nield LE, Strachan K, Taylor G, Manlhiot C, McCrindle B, Atkinson A, Jaeggi E, Shannon P. Labatt Family Heart Centre Innovations Funding. $50,000 CAD. Jan to Dec Congenital heart block international research network (CIRN). Hamilton R, Hornberger L, Jaeggi E, Silverman E. Canadian Institute of Health Research. $311,172 CAD. Sep to Aug Creating a fetal lamb model of hypoplastic left heart syndrome. DeVrijer B, Chaturvedi R, Jaeggi E, Keunen J. Labatt Family Heart Centre Innovation Funds. $37,490 CAD. Jun to Dec CTOT-C (Clinical Trials in Organ Transplantation in Children): Alloantibodies in cardiac transplantation: Intervention, outcomes and mechanisms. Webber S, Dipchand AI, Blume E, Canter C, Naftel D, Hsu D. National Institutes of Health. $5,205,958 CAD. Jan to Dec Determinants of disease progression in pediatric cardiomyopathy. Kantor P, Mertens LM, Manlhiot C. Heart and Stroke Foundation of Canada. $140,376 CAD. Jan to Dec Echocardiographic quantification of right ventricular volumes, ejection fraction and regional RV shape in patients after tetralogy of Fallot repair. Use of novel echocardiographic technique. Mertens L, Dragulescu A, Gross-Wortmann L, Redington A, Friedberg M, Saver N, Claus P, d hooge J, Cohen M, Soriano B. Ventripoint Inc. $66, CAD. Jan to Dec Effects of remote ischemic preconditioning on clinical and genomic responses to open heart surgery in children. Redington AN, McCrindle BW. Heart and Stroke Foundation of Ontario. $570,399 CAD. Jan to Dec Establishment of normal values for echocardiography ventricular and vascular functional research. Mertens L, Bradley TJ, Friedberg MK, Jaeggi ET. The Labatt Family Heart Centre - Innovation Fund. $48,000 CAD. Jan to Dec the hospital for sick children department of paediatrics Annual Report

14 Exome sequencing to identify a new gene underlying Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC). Hamilton R. Rare Disease Foundation and the BC Children s Hospital Foundation (BCCNF). $3500 CAD. May to May Exome sequencing to identify the gene underlying alveolar capillary dysplasia, a rare cause of pulmonary hypertension lethal to infants. Hamilton R. Rare Disease Foundation and the BC Children s Hospital Foundation (BCCNF). $3500 CAD. May to May Exome sequencing to identify the gene underlying congenital junctional ectopic tachycardia (CJET). Hamilton R. Rare Disease Foundation and the BC Children s Hospital Foundation (BCCNF). $3500 CAD. May to May Genomic basis of congenital heart disease. Mital S. McLaughlin Centre Accelerator Grant in Genomic Medicine. $50,000 CAD. Jan to Dec Genotype-phenotype associations in paediatric cardiomyopathy. Lipshultz SE, Towbin JA, Ware S, Wilkinson J, Kantor PF, et al. National Institutes of Health (NHLBI). $8,759,446 CAD. Jan to Dec Glucose homeostasis and cardiac output after paediatic cardiac surgery. Mital S, Schwartz S, McCrindle B. Heart and Stroke Foundation of Ontario. $195,019 CAD. Jan to Dec Increasing physical activity among children with congenital heart defects. McCrindle B, Russell J, Dettmer E, Longmuir P, Faulkner G. Heart and Stroke Foundation of Ontario. $668,349 CAD. Jan to Dec Kawasaki disease: On our way to unraveling the pathogenesis. McCrindle B, Yeung R. Canadian Institutes of Health Research. $792,717 CAD. Jan to Dec Kawasaki disease: On our way to unraveling the pathogenesis. McCrindle B, Yeung R. Canadian Institutes of Health Research. $798,779 CAD. Jan Dec Large diameter Advanta V12 covered stent trial for coarctation of the aorta. Benson L, Chaturvedi R, Lee KJ, Golding F, Wan A. Atrium Medical Corporation. $4,550 US/patient over 5 years. Jan to Dec Melody transcatheter pulmonary valve post-market surveillance study. Benson L. Medtronic Inc. $8,800 US/patient over 5 years. Jan to Dec MRI assessment of pulmonary vascular remodeling and vasoconstriction in pulmonary hypertension. Macgowan K, Grosse-Wortmann L, Humpl T, Yoo S-J. Canadian Institutes of Health Research. $260,076 CAD. Sept to Aug Normal human fetal circulatory physiology by echocardiography and MRI. Seed M, Jaeggi E, Macgowan C, Yoo SJ, Grosse- Wortmann L. Labatt Family Heart Center Innovation Grant. $24,000 CAD. Jan ongoing Novel approaches to the prediction, diagnosis and treatment of cardiac late effects in survivors of childhood cancer. Nathan P, Kantor PF, Lui PP, Mertens LM, Mital SM, et al. Canadian Institute of Health Research. $2,797,748 CAD. Jan to Dec Ontario initiative in personalized stem cell medicine. Rossant J, Mital S. CFI. $932,011 CAD, Biorepository component - Mital S. $135,526 CAD. Jan to Dec Patterns and clinical significance of electro-mechanical dyssynchrony in pediatric dilated cardiomyopathy. Friedberg MK. Cardiomyopathy Foundation. $44,550 CAD. Feb to Feb Pediatric Heart Network The Hospital for Sick Children, Toronto. Schwartz S, Mital S, McCrindle B. NIH/NHLBI. $7,518,965 CAD. Jan to Dec Percutaneous venovenous oxygenation for support. Benson L. Labatt Family Heart Centre Innovation Funds. $15,072 CAD. Jan to Dec Personalized medicine in the genomics era: International Symposium on Childhood Heart Disease. Mital S. Mitacs, University of British Columbia. $7,000 CAD. Jan to Dec Pilot study to evaluate the effect of peri-operative diastolic function on post-surgical course in infants with single ventricle physiology. Friedberg M, Mertens L, Schwartz S, Jaeggi E, Dragulescu A, Petrovic M. SickKids Foundation. $44, CAD. Jan to Dec Placental insufficiency and aortic isthmus flow (PIAF Study). Fouron JC, Jaeggi E, Pasquier JC, Audibert F, Bujold E, Chetaille P, Morin L, Takser L, Weiss-Lambrou R. Canadian Institute of Health Research. $907,333 CAD. Jan to Dec Primary EBV infection in the immunosuppressed setting of organ transplantation: Determinants of virus-host interaction. Allen U, Beyene J, Dipchand A, Grunebaum E, Hebert D, Husain S, Mazulli T, Ng V, Read S, Robinson J, Tellier R, Weitzman S. Canadian Institutes of Health Research. $509,125 CAD. Jan to Dec PROMOTE: Preschoolers at Risk Obesity Trajectories and Cardiometabolic Factors: Towards early identification. McCrindle B, Birken C. Canadian Institutes of Health Research. $655,640 CAD. Jan to Dec Prospective cohort study of adults after tetralogy of Fallot: A Canadawide registry. Wald R, Redington AN, Benson L, Caldarone C, McCrindle B, Farkouh, Oechslin E, Silversides C, Vonder Muhll I, Grewal J, Nadeem N, Siu S, Therrien J. Canadian Institute of Heart Research. $771,662 CAD. Jan to Dec The biology of chronic preconditioning: Genomic and physiologic mechanisms of response. Redington A, McCrindle B, Wells G. Canadian Institutes of Health Research. $329,073 CAD. Jan to Dec The CHAPTER Study: Congenital heart adolescents participating in transition evaluation research. McCrindle B, Mackie A. Heart and Stroke Foundation, Multi-Provincal Grant in-aid. $358,115 CAD. Jan to Dec The use of positron emission tomography to determine survival in patients on cardiac extracorporeal membrane. Chaturvedi R, Floh A, Schwartz S, Sivarajan B, Charron M. SickKids Foundation. $13,000 CAD. Jan to Dec Thromboprophylaxis before Fontan for neonates and infants with single ventricle physiology. McCrindle B, Mital S, Schwartz S. NIH/NLHBI Pediatric Heart Network $7,518,965 CAD. Jan to Dec Thrombosis in pediatric heart transplant recipients and risk factors for outcomes. Dipchand AI, Manlhoit C. CSL Behring. $5000 CAD. Jan to Dec Ventricular function and arterial stiffness in the evaluation of pediatric heart failure. Bradley TJ, Kantor PF, Roche SL, Slorach C. Paediatric Consultants at the Hospital for Sick Children. $7,200 CAD. Jan to Dec Ventricular-ventricular interactions and their modulation for enhancement of right ventricular performance in pulmonary arterial hypertension. Friedberg M, Redington A, Humpl T, Mertens L. Canadian Institutes of Health Research. $298,500 CAD. Jan to Dec the hospital for sick children department of paediatrics Annual Report

15 CLINICAL AND METABOLIC GENETICS overview Division Head Dr. Daune MacGregor (Interim) Full Time Dr. Sarah Bowdin Dr. Melissa Carter Dr. David Chitayat Dr. Peter Kannu Dr. Saadet Mahmutoglu Dr. Roberto Mendoza Dr. Stephen Meyn Dr. Julian Raiman Dr. Komudi Siriwardena Dr. Andreas Schulze Dr. Rosanna Weksberg Dr. Grace Yoon Part Time Dr. Joe Clarke Dr. Leona Fishman Clinical Fellows Dr. Walla Al Hertani Dr. Ramses Badilla Dr. Shailly Jain Ghai Subspecialty Residents Dr. Almundher Al Maawali Dr. Ayeshah Chaudhry Dr. Soghra Jougheh Doust Dr. Bita Hashemi Dr. Andrea Guerin Dr. Rebekah Jobling Dr. Natalya Karp Dr. Sharon Moalem Dr. Maian Roifman Dr. Maha Saleh Administrative Staff Michelle Au Laurie Baciao Michelle Chu Michael Delaney Susan Dukhedlin-Lalla Denese Henry Allison Griffin Nasil Kim Melanie Lameront Kerry McPartland Andrea Pemberton Suzanne Prashad Deb Taylor Rozmin Visram Khadine Wiltshire The Division of Clinical and Metabolic Genetics is committed to providing excellence in patient care, research and education. The Division welcomed two new physicians in 2011: Dr. Melissa Carter in September and Dr. Saadet Mahmutoglu in October. Dr. Carter completed her medical genetics training at the Children s Hospital of Eastern Ontario in 2009 followed by a clinical fellowship in developmental paediatrics at Holland- Bloorview Kids Rehabilitation Hospital in Toronto. Her clinical interests include care of children with genetic developmental disabilities (in particular, chromosomal disorders and Rett syndrome). She established Toronto s first autism genetics clinic in October Research interests include delineation of new syndromes associated with recurrent copy number variants, in particular those reported in studies of autism spectrum disorder. Dr. Saadet Mahmutoglu received her medical degree at the Ege University, Medical Faculty in Turkey and Leopold-Franzens University, Medical Faculty, in Austria. She trained in paediatrics at the University of Istanbul in Turkey and University of Vienna in Austria. She undertook fellowship training in biochemical genetics and paediatric neurology at Children s Hospital, Vancouver, British Columbia. Saadet is in the final stages of writing her PhD on Creatine Deficiency Syndromes at the VU Medical Center, Amsterdam, The Netherlands. Her clinical interest is neurometabolic disorders and she has started the Neurometabolic Clinic in the Division of Clinical and Metabolic Genetics. Her research interests included creatine deficiency syndromes, metabolic epilepsies, white matter disorders and lysosomal storage disease. The Department has recruited a new Division Head, Dr. Ronald Cohn, who will be joining the Division in September In addition, Dr. Roberto Mendoza was promoted to the rank of Associate Professor. Committed to providing excellence in patient care, research and education. department of paediatrics Annual Report

16 Research endeavours in the Division are a vital part of our program and currently focus on the translation of new genomic information for patients and their families. There is continuing increase in the use of microarrays for genetic testing. The influx of genomic data for many of our patients has influenced our patterns of practice. There are many new challenges in the interpretation of entirely novel data in clinical settings. In March 2012, the Division hosted the first annual Divisional Research Day. Dr. Raymond Kim was awarded the Research Day Poster Award for Exome Sequencing in Primary Ciliary Dyskinesia. The Research Day Platform Award was awarded to Dr. Ramses Badilla-Porras for DNA Sequencing as a Diagnostic Tool for FBP1 Gene Mutations in Costa Rican Patients with Fructose-1, 6-Bisphosphatase Deficiency. The Eleventh Annual Starbucks Clinical Research Summer Students Competition were awarded to Dr. Melissa Carter (Delineation of Clinical Features of Individuals with Microduplication 22q11.2: A Questionnaire); Dr. Julian Raiman (Retrospective Study of Bone Health in Gaucher Disease at the Hospital for Sick Children (SickKids) and Mount Sinai Hospital (MSH)); Dr. Saadet Mahmutoglu (Retrospective review of patients with myoclonic epilepsy to generate a database: first steps to identify underlying aetiology); Dr. Peter Kannu and Ms. Andrea Shugar (Prevalence of Autism Spectrum Disorder (ASD) in Neurofibromatosis Type 1 (NF1) Patients and Implications for Screening Policies); and Ms. Sarah Herd and Ms. Laura Burr (Dietary Treatment with a High Medium-Chain Triglyceride Formula in Early Life and Nutritional Status of Children with Long Chain Fatty Acid Oxidation Defects). The Division of Clinical and Metabolic Genetics has developed and participated in numerous educational activities within SickKids. It has important links to the Research Institute and the Department of Paediatric Laboratory Medicine. The Division directs fellowship and residency training programs in genetic medicine as well as the MSc Program in Genetic Counselling. During the academic year, there were ten medical genetics residents and three clinical fellows, making our division one of the largest training programs in Canada. Dr. David Chitayat was awarded the Dr. Richard Rowe Award. Dr. Rowe was Head of the Division of Cardiology from 1973 until 1986, and demonstrated the very finest attributes of a clinical scholar. This award honors a physician in the Department for sustained clinical excellence and aimed at physicians who have demonstrated excellence in the delivery of clinical care in its broadest sense over a significant period of time. Dr. Ramses Badilla was awarded the CanMEDS Competencies Award. This award recognizes and honours trainees who exemplify one or more of the non-medical expert competencies embodied in the Royal College of Physicians and Surgeons of Canada CanMEDS 2005 Physician Competency Framework. Only two awards are handed out each year one to a resident in core paediatrics and one to a resident or fellow in a paediatric subspecialty program. Rozmin Visram received the Department s Administrative Award. This award acknowledges a administrative team member who demonstrates outstanding performance and excellence in all aspects of their role and beyond. As well, Dr. Julian Raiman was this year s recipient of the Robert Salter Humanitarian Award. This award recognizes and honours medical health professionals or support staff at SickKids who exemplify the qualities of compassion and humanitarianism in the provision of patient and family-centred care. Honours and Awards Carter, M: Winner of the Autism Research Training Program Fellowship, funded by Canadian Institutes of Health Research (CIHR) Strategic Training Initiative in Health Research (STIHR), 2009 to Chitayat, D: Winner of the Richard Rowe Award for excellence in clinical care, Toronto, Ontario, June, Mendoza, R: Winner of the Early Career Research Presentation, Tenth Biennial Meeting of the International Skeletal Dysplasia Society (ISDS), Palm Cove, Tropical North Queensland, Australia. Raiman, J: Winner of Robert Salter Humanitarian Award, SickKids Foundation, Toronto, Ontario, Canada, Publications Al Maawali A, Machado J, Fang P, Dupuis L, Faghfoury H, Mendoza-Londono R. Angelman syndrome due to a termination codon mutation of the UBE3A gene. Journal of Child Neurology 2012: (epub ahead of print). Al-Zaidy SA, Baskin B, Hawkins C, Yoon G, Ray PN, Vajsar J. Milder phenotype of congenital muscular dystrophy in a novel POMT1 mutation. Muscle and Nerve 2012: 45 (5): pp Anderson BH, Kasher PR, Mayer J, Szynkiewicz M, Jenkinson EM, Bhaskar SS, Urquhart JE, Daly SB, Dickerson JE, O Sullivan J, Leibundgut EO, Muter J, Abdel-Salem GM, Babul-Hirji R, Baxter P, Berger A, Bonaf e L, Brunstom-Hernandez JE, Buckard JA, Chitayat D, Chong WK, Cordelli DM, Ferreira P, Fluss J, Forrest EH, Franzoni E, Garone C, Hammans SR, Houge G, Hughes I, Jacquemont S, Jeannet PY, Jefferson RJ, Kumar R, Kutschke G, Lundberg S, Louren eo CM, Mehta R, Naidu S, Nischal KK, Nunes L, Ounap K, Philippart M, Prabhakar P, Risen SR, Schiffmann R, Soh C, Stephenson JB, Stewart H, Stone J, Tolmie JL, van der Knaap MS, Vieira JP, Vilain CN, Wakeling EL, Wermenbol V, Whitney A, Lovell SC, Meyer S, Livingston JH, Baerlocher GM, Black GC, Rice GI, Crow YJ. Mutations in CTC1, encoding conserved telomere maintenance component 1, cause Coats plus. Nature Genetics 2012: 44 (3): pp Brunel-Guitton C, Casey B, Coulter-Mackie M, Stockler- Ipsiroglu S, Vallance H, Hewes D, Mercimek-Mahmutoglu S. Late onset nonketotic hyperglycinemia caused by a novel homozygous missense mutation in the GLDC gene accepted. Molecular Genetics and Metabolism 2011: 103 (2): pp Cabral WA, Barnes AM, Adeyemo A, Cushing K, Chitayat D, Porter FD, Panny SR, Gulamali-Majid F, Tishkoff SA, Rebbeck TR, Gueye SM, Bailey-Wilson JE, Brody LC, Rotimi CN, Marini JC. A founder mutation in LEPRE1 carried by 1.5% of West Africans and 0.4% of African Americans causes lethal recessive osteogenesis imperfecta. Genetics in Medicine 2012: 14 (5): pp Campbell IM, Yatsenko SA, Hixson P, Reimschisel T, Thomas M, Wilson W, Dayal U, Wheless JW, Crunk A, Parkinson N, Fishman L, Riviello JJ, Nowaczyk MJM, Zeesman S, Rosenfeld JA, Bejjani BA, Shaffer LG, Cheung SW, Lupski JR, Stankiewicz P, Scaglia F. Novel 9q34.11 gene deletions encompassing combinations of four Mendelian disease genes: STXBP1, SPTAN1, ENG, and TOR1A. Genetics in Medicine 2012 (EPub). Carter MT, Blaser S, Papsin B, Meschino W, Reardon W, Klatt R, Babul-Hirji R, Milunsky J, Chitayat D. Middle and inner ear malformations in mutation-proven branchio-oculo-facial (BOF) syndrome: Case series and review of the literature. American Journal of Medical Genetics A 2012: 158A (8): pp Carter MT, Boycott KM. Microcephaly-capillary malformations syndrome A story of rapid emergence of a new recognizable entity. American Journal of Medical Genetics Part A 2011:155A(9): pp Chitayat D, Langlois S, Wilson RD. Prenatal screening for fetal aneuploidy in singleton pregnancies. Journal of Obstetrics and Gynaecology Canada 2011: 33 (7): pp Chung BHY, Mullegama S, Marshall CR, Lionel AC, Weksberg R, Dupuis L, Brick L, Li C, Scherer SW, Aradhya S, Stavropoulos DJ, Elsea SH, Mendoza-Londono R. Severe intellectual disability and autistic features in individuals with microduplication 2q23.1. European Journal of Human Genetics 2011: Nov 16. doi: /ejhg De Ru MH, Teunissen QG, van der Lee JH, Beck M, Bodamer OA, Clarke LA, Hollak CE, Lin SP, Munoz Rojas MV, Pastores GM, Raiman J, Scarpa M, Treacy EP, Tylki-Szymanska A, Wraith E, Zeman J, Wijburg FA. Capturing phenotypic heterogeneity in MPS I Results of an international consensus procedure. Orphanet Journal of Rare Diseases 2012: 23:7(1): pp 22. Duncan A, Langlois S, Wilson RD, Audibert F, Brock JA, Carroll J, Cartier L, Gagnon A, Johnson JA, Murphy-Kaulbeck L, Okun N, Pastuck M, Langlois S, Chitayat D, DeBie I, Demczuk S, D esilets VA, Geraghty MT, Marcadier J, Nelson TN, Siu V, Skidmore D. Use of array genomic hybridization technology in prenatal diagnosis in Canada. Journal of Obstetrics and Gynaecology Canada 2011: 33 (12): pp the hospital for sick children department of paediatrics Annual Report

17 Dunk CE, Roggensack AM, Cox B, Perkins JE, Asenius F, Keating S, Weksberg R, Kingdom JC, Adamson SL. A distinct microvascular endothelial gene expression profile in severe IUGR placentas. Placenta 2012: 33(4): pp Epub 2012 Jan 20. Engel K, Vuissoz JM, Eggimann S, Groux M, Berning C, Hu L, Klaus V, Moeslinger D, Mercimek-Mahmutoglu S, Stöckler S, Wermuth B, Häberle J, Nuoffer JM. Bacterial expression of mutant argininosuccinate lyase reveals imperfect correlation of in-vitro enzyme activity with clinical phenotype in argininosuccinic aciduria. Journal of Inherited Metabolic Disorders 2012: 35 (1):pp Fenwick A, Bowdin S, Klatt R, Wilkie A. A deletion of FGFR2 creating a chimeric IIIb/IIIc exon in a child with Apert syndrome. BMC Medical Genetics 2011: 12: pp 122. Furrow A, Theisen A, Velsher L, Bawle EV, Sastry S, Mendelsohn NJ, Jarvis K, Shaffer LG, Chitayat D. Duplication of the STS region in males is a benign copy-number variant. American Journal of Medical Genetics A 2011:155 (8): pp Gardiner K, Chitayat D, Choufani S, Shuman C, Blaser S, Terespolsky D, Farrell S, Reiss R, Wodak S, Pu S, Ray PN, Baskin B, Weksberg R. Brain abnormalities in patients with Beckwith- Wiedemann syndrome. American Journal of Medical Genetics Part A 2012: 158A(6): pp doi: /ajmg.a Gibson WT, Hood RL, Zhan SH, Bulman DE, Fejes AP, Moore R, Mungall AJ, Eydoux P, Babul-Hirji R, An J, Marra MA, Chitayat D, Boycott KM, Weaver DD, Jones SJ (FORGE Canada Consortium). Mutations in EZH2 cause Weaver syndrome. American Journal of Human Genetics 2012: 90 (1): pp Gillis J, Burashnikov E, Antzelevitch C, Blaser S, Gross G, Turner L, Babul-Hirji R, Chitayat D. Long QT, syndactyly, joint contractures, stroke and novel CACNA1C mutation. Expanding the spectrum of Timothy syndrome. American Journal of Medical Genetics A 2012: 158(1): pp Gribouval O, Morini ere V, Pawtowski A, Arrondel C, Sallinen SL, Saloranta C, Clericuzio C, Viot G, Tantau J, Blesson S, Cloarec S, Machet MC, Chitayat D, Thauvin C, Laurent N, Sampson JR, Bernstein JA, Clemenson A, Prieur F, Daniel L, Levy-Mozziconacci A, Lachlan K, Alessandri JL, Cartault F, Riviere JP, Picard N, Baumann C, Delezoide AL, Ortega MB, Chassaing N, Labrune P, Yu S, Firth H, Wellesley D, Bitzan M, Alfares A, Braverman N, Krogh L, Tolmie J, Gaspar H, Doray B, Majore S, Bonneau D. Spectrum of mutations in the renin-angiotensin system genes in autosomal recessive renal tubular dysgenesis. Human Mutation 2012: 33 (2): pp Goh E, Perez IC, Canales CP, Ruiz P, Agatep R, Yoon G, Chitayat D, Dror Y, Shago M, Goobie S, Sgro M, Walz K, Mendoza-Londono R. Definition of a critical genetic interval related to kidney abnormalities in the Potocki-Lupski syndrome. American Journal of Medical Genetics 2012: 158A(7): pp Injeyan MC, Shuman C, Shugar A, Chitayat D, Atenafu EG, Kaiser A. Personality traits associated with genetic counselor compassion fatigue: The roles of dispositional optimism and locus of control. Journal of Genetic Counseling 2011: 20 (5): pp Jain-Ghai S, Nagamani SC, Blaser S, Siriwardena K, Feigenbaum A. Arginase I deficiency: Severe infantile presentation with hyperammonemia: More common than reported. Molecular Genetics in Metabolism 2011: 104(1-2): pp Kannu P, O Rielly DD, Hyland JC, Ala Kokko L. Avascular necrosis of the femoral head due to a novel C propeptide mutation in COL2A1. American Journal of Medical Genetics Part A 2011: 155A(7): pp Khan MA, Rafiq MA, Noor A, Hussain S, Flores JV, Rupp V, Vincent AK, Malli R, Ali G, Khan FS, Ishak GE, Doherty D, Weksberg R, Ayub M, Windpassinger C, Ibrahim S, Frye M, Ansar M, Vincent JB. Mutation in NSUN2, which encodes an RNA methyltransferase, causes autosomal-recessive intellectual disability. American Journal of Human Genetics 2012: 90(5):pp Epub 2012 Apr 26. Matthew AG, Paradiso C, Currie KL, Finelli A, Hartman ME, Trachtenberg L, Shuman C, Horsburgh S, Chitayat D, Trachtenberg J, Ritvo P. Examining risk among men with a family history of prostate cancer. Patient Education and Counseling 2011: 85 (2): pp Mendoza-Londono R, Chitayat D, Kahr W, Hinek A, Blaser S, Dupuis L, Goh E, Badilla-Porras, Howard A, Mittaz L, Superti- Furga A, Unger S, Nishimura G, Bonafe L. Extracellular matrix and platelet function in patients with musculocontratural Ehlers-Danlos syndrome caused by mutations in the CHST14 gene. American Journal of Medical Genetics 2012: 158A(6): pp Mercimek-Mahmutoglu S, Al-Thihl K, Roland E. Is low serum creatine kinase a nonspecific screening marker for creatine deficiency syndromes? Molecular Genetics in Metabolism 2012:106(2): pp Mercimek-Mahmutoglu S, Dunbar M, Friesen A, Garret S, Hartnett C, Huh L, Sinclair G, Stockler S, Wellington S, Pouwels PJ, Salomons GS, Jakobs C. Evaluation of two year treatment outcome and limited impact of arginine restriction in a patient with GAMT deficiency. Molecular Genetics in Metabolism 2012:105 (1): pp Mercimek-Mahmutoglu S, Horvath GA, Coulter-Mackie M, Nelson T, Waters PJ, Sargent M, Struys E, Jakobs C, Stockler- Ipsiroglu S, Connelly MB. Profound neonatal hypoglycemia and lactic acidosis caused by pyridoxine-dependent epilepsy. Pediatrics 2012: 129(5): pp Mercimek-Mahmutoglu S, Tucker T, Casey B. Phenotypic heterogeneity in two siblings with methylglutaconic aciduria type I caused by a novel intragenic deletion. Molecular Genetics in Metabolism 2011: 104 (3): pp Merrill AE, Sarukhanov A, Krejci P, Idoni B, Camacho N, Estrada KD, Lyons KM, Deixler H, Robinson H, Chitayat D, Curry CJ, Lachman RS, Wilcox WR, Krakow D. Bent bone dysplasia-fgfr2 type, a distinct skeletal disorder, has deficient canonical FGF signaling. American Journal of Human Genetics 2012: 90 (3): pp Moalem S, Babul-Hirji R, Stavropolous DJ, Wherrett D, Bagli DJ, Thomas P, Chitayat D. XX male sex reversal with genital abnormalities associated with a de novo SOX3 gene duplication. American Journal of Medical Genetics 2012: 158A (7): pp Murji A, Proctor LK, Paterson AD, Chitayat D, Weksberg R, Kingdom J. Male sex bias in placental dysfunction. American Journal of Medical Genetics Part A. 2012: 158A(4): pp doi: /ajmg.a Epub 2012 Mar 9. Nennstiel-Ratzel U, Lueders A, Blankenstein O, Ceglarek U, Ensenauer R, Fingerhut R, Klein J, Lindner M, Mueller C, Peter M, Rauterberg E, Schneider I, Schultis W, Schulze A, Starke I, Stehn M, Stopsack M, Fusch C. National screening report Germany DGNS. PDF/Screeningreport_2009_e.pdf (2011). Niederhoffer KY, Peñaherrera M, Pugash D, Rupps R, Arbour L, Tessier F, Choufani S, Zhao C, Manokhina I, Shuman C, Robinson WP, Weksberg R, Boerkoel CF. Beckwith Wiedemann syndrome in sibs discordant for IC2 methylation. American Journal of Medical Genetics Part A. 2012: 158A(7): pp Rivière JB, Mirzaa GM, O Roak BJ, Beddaoui M, Conway RL, St-Onge J, Schwartzentruber JA, Karen W, Gripp KW, Nikkel SM, Worthylake T, Sullivan CT, Ward TR, Butler HE, Kramer NA, Albrecht B, Armour CM, Armstrong L, Caluseriu O, Cytrynbaum C, Drolet BA, Lauzon JL, Mancini GM, Meschino WS, Reggin JD, Sagie T, Weksberg R, Zirn B, Beaulieu CL, FORGE Canada Consortium, Majewski J, Bulman DE, Shendure J, Graham JM Jr, Boycott KM, Dobyns WB. De novo germline and postzygotic mutations in the PI3K/AKT pathway genes AKT3, PIK3R2 and PIK3CA cause a spectrum of related megalencephaly syndromes. New England Journal of Medicine 2012: Jun 24. doi: / ng (Epub ahead of print). 32 the hospital for sick children department of paediatrics Annual Report

18 Roscioli T, Kamsteeg EJ, Buysse K, Maystadt I, van Reeuwijk J, van den Elzen C, van Beusekom E, Riemersma M, Pfundt R, Vissers LE, Schraders M, Altunoglu U, Buckley MF, Brunner HG, Grisart B, Zhou H, Veltman JA, Gilissen C, Mancini GM, Delrée P, Willemsen MA, Ramadža DP, Chitayat D, Bennett C, Sheridan E, Peeters EA, Tan-Sindhunata GM, de Die-Smulders CE, Devriendt K, Kayserili H, El-Hashash OA, Stemple DL, Lefeber DJ, Lin YY, van Bokhoven H. Mutations in ISPD cause Walker-Warburg syndrome and defective glycosylation of α-dystroglycan. Nature Genetics 2012: 44(5): pp Rosenfeld JA, Traylor RN, Schaefer GB, McPherson EW, Ballif BC, Klopocki, Mundlos S, Shaffer LG, Aylsworth AS, 1q21.1 Study Group. Proximal microdeletions and microduplications of 1q21.1 contribute to variable abnormal phenotypes. European Journal of Human Genetics 2012: pp 1-8. Sato D, Lionel AC, Leblond CS, Prasad A, Pinto D, Walker S, O Connor I, Russell C, Drmic IE, Hamdan FF, Michaud JL, Endris V, Roeth R, Delorme R, Huguet G, Leboyer M, Rastam M, Gillberg C, Lathrop M, Stavropoulos DJ, Anagnostou E, Weksberg R, Fombonne E, Zwaigenbaum L, Fernandez BA, Roberts W, Rappold GA, Marshall CR, Bourgeron T, Szatmari P, Scherer SW. SHANK1 deletions in males with autism spectrum disorder. American Journal of Human Genetics 2012: 90(5): pp Epub 2012 Apr 12. Shaffer LG, Dabell MP, Rosenfeld JA, Neill NJ, Ballif BC, Coppinger J, Diwan NR, Chong K, Shohat M, Chitayat D. Referral patterns for microarray testing in prenatal diagnosis. Prenatal Diagnosis 2012: 32 (4): pp Shugar, S, Kivirikku S, Pöyhönen M, Messiaen L. Identification of copy number changes affecting the SPRED1 gene using RT-PCR, multiplex ligation-dependent probe amplification and quantitative PCR. American Journal of Medical Genetics 2011: 155A: pp Shuman C, Weksberg R. Beckwith-Wiedemann syndrome. Orphanet 2011: Dec ORPHA116. Skidmore DL, Chitayat D, Morgan T, Hinek A, Fischer B, Dimopoulou A, Somers G, Halliday W, Blaser S, Diambomba Y, Lemire EG, Kornak U, Robertson SP. Further expansion of the phenotypic spectrum associated with mutations in ALDH18A1, encoding Δ¹-pyrroline-5-carboxylate synthase (P5CS). American Journal of Medical Genetics A 2011: 155 A: pp Smith AC, Suzuki M, Thompson R, Higgins MJ, Chiu IW, Squire JA, Greally J, Weksberg R. Maternal gametic transmission of translocations or inversions of human chromosome 11p15.5 results in regional DNA hypermethylation and downregulation of CDKN1C expression. Genomics 2012: 99(1): pp Stockler S, Plecko B, Gospe SM Jr, Coulter-Mackie M, Connolly M, van Karnebeek C, Mercimek-Mahmutoglu S, Hartmann H, Scharer G, Struijs E, Tein I, Jakobs C, Clayton P, Van Hove JL. Pyridoxine dependent epilepsy and antiquitin deficiency clinical and molecular characteristics and recommendations for diagnosis, treatment and follow-up. Molecular Genetics in Metabolism 2011:104 (1-2): pp Ting J, Hochwald O, Au N, Mercimek-Mahmutoglu S. Blueberry muffin rash and thrombocytopenia in a newborn with mucolipidosis type II (I-cell disease) masquerading as congenital infections. Molecular Genetics in Metabolism 2011: 104 (4): pp Trottier M, Roberts W, Drmic I, Scherer SW, Weksberg R, Cytrynbaum C, Chitayat D, Shuman C, Miller FA. Parents perspectives on participating in genetic research in autism. Journal of Autism and Development Disorders 2012 (E pub ahead of print). Wan J, Yourshaw M, Mamsa H, Rudnik-Schöneborn S, Menezes MP, Hong JE, Leong DW, Senderek J, Salman MS, Chitayat D, Seeman P, von Moers A, Graul-Neumann L, Kornberg AJ, Castro-Gago M, Sobrido MJ, Sanefuji M, Shieh PB, Salamon N, Kim RC, Vinters HV, Chen Z, Zerres K, Ryan MM, Nelson SF, Jen JC. Mutations in the RNA exosome component gene EXOSC3 cause pontocerebellar hypoplasia and spinal motor neuron degeneration. Nature Genetics 2012: 44 (6): pp Wertaschnigg D, Jaeggi M, Chitayat D, Shannon P, Ryan G, Thompson M, Yoo SJ, Jaeggi E. Prenatal diagnosis and outcome of absent pulmonary valve syndrome: Contemporary single center experience and review of the literature. Ultrasound in Obstetrics and Gynecology 2012 (e Pub). Williams B, Meyn MS, Hitzler J. Transient leukemia in newborns without Down syndrome - Diagnostic and management challenges. Journal of Pediatric Hematology Oncology 2011: 33: pp Books and Chapters Baskin B, Bowdin S, Ray PN. Cherubism - comprehensive update posted live on September 1, In: GeneReviews (Internet). (Pagon RA, Bird TD, Dolan CR, Stephens K, eds). University of Washington. Seattle, WA Glanc P, Chitayat D. Prenatal diagnosis of lethal skeletal dysplasias. In: UpToDate, (Levine D, Wilkins-Haug L, Section eds) Glanc P, Chitayat D, Unger S. The fetal musculoskeletal system. In: Diagnostic Ultrasound, Fourth edition (Rumack CM, Wilson SR, Charboneau JW, Levine D, eds). Mosby Publishing, New York 2011: pp Schulze A. Creatine deficiency syndromes. In: Paediatric Neurology, Third series (Dulac O, Sarnat HB, Lassonde M, eds). Stoeckler-Ipsiroglu S, Mercimek-Mahmutoglu S, Salomons G. Creatine deficiency syndromes. In: Inborn Metabolic Diseases. Diagnosis and Treatment, Fifth edition (Fernandes J, Saudubray JM, van den Berghe G, eds). Springer Grants and Funding A pilot study to assess prevalence of GAMT deficiency in British Columbia. Mahmutoglu S. Rare Disease Foundation, Microgrant Program. $3,500 CAD to 2012 Aetiology of carpal and tarsal tunnel syndrome in children and in adolescents in British Columbia. Mahmutoglu S. Rare Disease Foundation, Microgrant Program. $3,500 CAD to 2012 An observational study of the clinical characteristics and disease progression of patients with Lysosomal acid lipase deficiency. Cholesteryl estersstorage disease phenotype. LAL-2-NH01. Raiman J. Synageva Biopharam Corporation. $1254 CAD per subject BMN004: A phase 3, randomized, double-blind, placebocontrolled, multinational clinical study to evaluate the efficiency and safety of 2.0mg/kg/week and 2.0 mg/kg/every other week BMN 110 in patients with Mucopolysaccharidosis IVA (Morquio A Syndrome). Raiman J. Biomarin Pharmaceutical. $56,690 CAD per patient (7 patients) to Present BMN005: A multicenter, multinational, extension study to evaluate long-term efficacy and safety of BMN 110 in patients with Mucopolysaccharidosis IVA (Morquio A Syndrome). Raiman J. Biomarin Pharmaceutical. $234,004 CAD per patient (7 patients) to Present Clinical and radiological investigation of cerebellar atrophy in childhood. Al-Maawali A, Yoon G. Rare Diseases Foundation Microgrant. $3,500 CAD to 2013 Common pathways of human DNA double strand break repair and Telomere maintenance. Meyn MS. Canadian Cancer Society. $122,877 CDN. Jul 2009 to Jun 2014 Cooperative multi-center program for research and treatment of autism. Roberts SW, Loh A, Roberts W, Anagnostou E, Marcon M, Weiss S, Brian J, Feigenbaum A, Schulze A, Logan W, Chitayat D, Weksberg R, Green P, Weiss J, Mitchell S, Berall G, Bradley E, Freeman N, Stinson J, Ornstein M, Atkinson A, Goldfarb C, Flanagan J, Superina D, Jiminez E, Jones Stokreef N, Orsino A, Isaacs B, Handley Derry M. Autism Treatment Network. $420,000 CAD to the hospital for sick children department of paediatrics Annual Report

19 Delineation of clinical features of individuals with microduplication 22q11.1: A questionnaire study. Carter M, Drmic I, Fishman L, Shugar A, Cytrynbaum C, Weksberg R, Roberts W, Anagnostou E. Rare Disease Foundation. $1,090. CAD to 2012 DNA damage responses in human hematopoietic stem and progenitor cells A pilot study. Meyn MS. SickKids Cancer Center New Ideas Grant. $49,600 CAD. Apr 2009 to Dec 2011 Effects of creatine supplementation on muscle pathophysiology in children with dermatomyositis. Feldman B, Schulze A, Stephens S, Wells G, Tein I. Rare Disease Foundation. $3,500 CAD to 2013 Emerging team in rare diseases: Achieving the triple aim for inborn errors of metabolism. CIHR Emerging Team Grant. Potter BK, Chakraborty P, Coyle D, Kronick J, Wilson K, Siriwardena K. $1,490,492 CAD to 2017 Epigenetic changes in DNA of infants exposed to gestational diabetes in utero. Hamilton J, Hanley A, Palmert M, Retnakaran R, Weksberg R. Canadian Diabetes Association. $297,734 CAD to 2015 Establishing DNA testing criteria for children with possible neurofibromatosis type 1. Kannu P, Shugar A. Rare Diseases Foundation Microgrant. $3500 CAD to 2011 Exome sequencing to identify disease modifying mutations in a cohort of patients with cardiomyopathy and right-sided congenital heart disease. Bowdin S, Greenway S, Shimizu M, Mital S, Redington A, Kantor P. Labatt Family Heart Centre Innovation Fund, SickKids Foundation. $30,000 CAD to 2012 Expanding the phenotypic spectrum of GLUT1 deficiency: A treatable metabolic epilepsy. Mahmutoglu S. Rare Disease Foundation, Microgrant Program. $3,500 CAD to 2013 Finding of rare disease genes in Canada (FORGE Canada). Canadian Pediatric Genetic Disorders Sequencing Consortium Competition. Mendoza R. Advancing Technology Innovation through Discovery Funders: Genome Canada, CIHR, Genome Quebec, Genome BC. $3,000,000 CAD. Mar 2011 to Aug 2012 First steps to the improvement of long-term outcome of GAMT deficiency by using an international database. Mahmutoglu S. Rare Disease Foundation, Microgrant Program. $3,500 CAD to 2012 First steps towards newborn screening for GAMT deficiency. Mahmutoglu S. Rare Disease Foundation, Microgrant Program. $3,500 CAD to 2012 Funding of an autism intervention research network for physical health. Roberts SW, Loh A, Feigenbaum A, Weiss S, Marcon C, Goldfarb C, Berall G, Bradley E, Brian J, Freeman N, Atkinson A, Taylor M, Stinson J, Mitchell S, Green P, Chitayat D. Health Resources and Services Administration. $400,882 CAD to 2011 Genomic basis of congenital heart disease. Mital S, Brudno, M, Bowdin S, Oechslin E, Chitayat D. McLaughlin Centre Accelerator Grant in Genomic Medicine. $50,000 CAD to 2012 Genomic technologies: Impact on clinical service delivery and strategies for rational and effective integration. Shuman C, Weksberg R, Guttmann A, Hayeems R. McLaughlin Centre. $200,000 CAD to 2013 Glucocorticoid induced osteoporosis (GIO) in the paediatric patient with chronic disease: Pathophysiology, prediction and management. Alman B, Shinya I, Mitchell J, Sochett E, Grynpas M, Biggar D, Feldman B, Thomas E, Grant R, Howard A, Mendoza-Londono R, Schneider R, Silverman E, Willett T. Team Grant: Bone Health. $10,000 CAD Glycogen storage disease research. Schulze A, Mouzaki M, Jain S, Faghfoury H, Bedford M, Pencharz P, Hamilton J, Feigenbaum A. SickKids Foundation. $37,310 CAD to 2012 Identification of new human imprinted genes: A novel approach. Weksberg R. National Sciences and Engineering Research Council of Canada. $390,630 CAD to 2017 Identification of novel iron-sulfur cluster assembly gene defects by exome sequencing. Robinson B, Schulze A. McLaughlin Centre. $20,000 CAD. May 2012 to Apr 2013 Interplay of genetic and epigenetic factors in the etiology of autism spectrum disorders. Weksberg R, Scherer S, Roberts W, Wodak S. The Ontario Mental Health Foundation. $149,758 CAD to 2014 Investigation of intrauterine growth restriction by gene expression and DNA methylation profiling. Weksberg, R, Chitayat D, Kingdom, Keating. CIHR. $383,883 CAD to 2011 Meeting the needs of children with skeletal dysplasia: Parents awareness, access to support and education services. Mendoza- Londono R, Dupuis L, Chitayat D, Shuman C, Trang H. Rare Diseases Fund. $2,045 CAD MetaKids. Mahmutoglu S. The Netherlands. $56,000 CAD to 2014 Modifying the biological late effects of pediatric and adolescent cancer treatments: Prevention and mitigation. Ito S, Guger S, Hitzler J, O Connor D, Schachar R, Spiegler B, Weksberg R, Carleton B. Canadian Institute of Health Research Team Grant. $2,109,312 CAD to 2016 Myoclonic epilepsy: Generating a database to identify treatable causes. Mahmutoglu S. Rare Disease Foundation, Microgrant Program. $3,500 CAD to 2013 Province of Ontario Neurodevelopmental Disorders Network (POND). Anagnostou E, Scherer S, Arnold P, Schacher R, Szatmari P, Marshall C, Nicolson R, Weksberg R, Lerch J, Beyene J, Reitzel J, Soreni N, Edwards A, Bountra C, Manassis K, Brian J, Roberts W, Mendlowitz S, Gorman D, Charach A, Woodbury- Smith M, Thorpe K, Ellis J, Foster J, Henkelman M, Taylor M. Ontario Brain Institute. $4,713,758 CAD to 2013 The FACTs Project: Fabry disease clinical research and therapeutics. Medin J. CIHR Emerging Team Grant in Rare Diseases. $2,500,000 CAD The GeneMed Program, Division of Clinical Pharmacology and Toxicology, The Hospital for Sick Children. Koren G, Chitayat D, Shinya I, Madadi P. McLaughlin Centre Accelerator Grant Competition. $60,000 CAD to 2012 The SickKids Research Institute (Infrastructure grant). Meyn MS. Canadian Foundation for Innovation. $91,119,176 CAD. Jun 2008 to May the hospital for sick children department of paediatrics Annual Report

20 CLINICAL PHARMACOLOGY AND TOXICOLOgY overview Division Head Dr. Shinya Ito Full-Time Dr. Gideon Koren Dr. Irena Nulman Dr. Margaret Thompson Part-Time Dr. David Juurlink Dr. Lisa Thurgur Cross-Appointees Dr. Christopher Parshuram Dr. Denis Scolnik Dr. Marco Sivilotti Dr. Savithiri Ratnapalan Dr. Sheila Jacobson Dr. Sylvain Baruchel Dr. Yaron Finkelstein Dr. Alejandro Nava- Ocampo Dr. Anna Taddio Dr. Bonnie Stevens Dr. Lidia Derewlany Research Fellows and Associates Dr. Ilan Matok Dr. Parvaneh Yazdani Brojeni Dr. Parvaz Madadi Dr. Taro Kamiya Dr. Katarina Aleksa Dr. Ernest Kopecky Dr. Smita Karande Clinical Fellows Dr. Bandar Baw Dr. Geert Willem Jong Dr. Hisaki Fujii Dr. Inna Svystonyuk Dr. Reo Tanoshima Dr. Rinat Hackmon Dr. Shahnaz Chaudhry Dr. Weerawadee Chandranipapongse Dr. Reuven Kedar Subspecialty Residents Dr. Joanne Ho Dr. Hagit Bergman Dr. Arif Manji Dr. Chris Lazongas Dr. Fatoumah Alabdulrazzaq Administrative Staff Niki Balamatsis Hila Halshtok Leah Moscato Shannon Vanderkooi Sarah Bondy Donna Tedesco The Division of Clinical Pharmacology and Toxicology at SickKids is one of the largest and most active paediatric pharmacology programs internationally. Our major clinical activities include a consultation service, the Motherisk program (a reproductive toxicology program), the Ontario Poison Centre, and DART (Drug Allergy, Reactions, and Toxicity) clinic in collaboration with the Division of Allergy and Immunology to provide specialized out-patient follow-up and management for those with various drug/toxin-related problems. The two information centres, Motherisk and Poison Centre, receive annually about 35,000 (Motherisk) and 75,000 (Poison Centre) inquires. The Motherisk Program directed by Dr. Gideon Koren continues generating and disseminating new knowledge in the field of drug safety for fetus and breastfeeding babies. The Ontario Poison Centre led by Dr. Margaret Thompson has now become a hub to medical toxicology training in Canada, attracting many residents and fellows nationally and internationally. In 2012, we have expanded our activity to provide toxicology/poisoning information to health professionals and the public in Manitoba. The division is one of the two major training sites of the Royal College. On the education front, the division is one of the two major training sites of the Royal College-accredited Clinical Pharmacology and Toxicology Residency Program of the University of Toronto. On the international scene, Dr. Shinya Ito, representing SickKids Clinical Pharmacology and Toxicology, co-leads the European Union-funded international initiative of paediatric pharmacology training to address global needs for paediatric clinical pharmacologists. Research by our members includes pharmacogenomics, drug metabolizing enzymes, drug transporters, mammary gland drug transport, reproductive toxicology and fetal alcohol syndrome. In particular, translational aspects of pharmacogenomics have become one of the important foci of our division. Recently, the pharmacogenomics-based personalized medicine program (GeneMed) has been launched as an Ontario Ministry funded research project to optimize drug therapy using patient s genetic information. department of paediatrics Annual Report

21 Honors and Awards Koren, G: Winner of Sumner J. Yaffe Lifetime Achievement Award, Paediatric Pharmacology and Therapeutics, Publications Abrahams R, Chase C, Desmoulin J, Kahan M, Knoppert D, Koren G, Lyons L, Ordean A, Roukema H, Uddin F. The opioid dependent mother and newborn - An update. Journal of Population Therapeutics and Clinical Pharmacology 2012: 19: pp e73-7. Alabdulrazzaq F, Koren G. Fetal safety of calcium channel blockers. Canadian Family Physician 2012: 58: pp Aleksa K, Carnevale A, Goodyer C, Koren G. Detection of polybrominated biphenyl ethers (PBDEs) in pediatric hair as a tool for determining in utero exposure. Forensic Science International 2011: Nov 10. Aleksa K, Liesivuori J, Koren G. Hair as a biomarker of polybrominated diethyl ethers exposure in infants, children and adults. Toxicology Letter 2011: Dec 29. Aleksa K, Walasek P, Fulga N, Kapur B, Gareri J, Koren G. Simultaneous detection of seventeen drugs of abuse and metabolites in hair using solid phase micro extraction (SPME) with GC/MS. Forensic Science International 2012: 218: pp Al-Maawali A, Walfisch A, Koren G. Taking angiotensinconverting enzyme inhibitors during pregnancy: Is it safe? Canadian Family Physician 2012: 58: pp Butte A, Ito S. Translational bioinformatics: Data-driven drug discovery and development. Clinical Pharmacology and Therapeutics 2012: 91: pp Cressman AM, Pupco A, Kim E, Koren G, Bozzo P. Smoking cessation therapy during pregnancy. Canadian Family Physician 2012: 58: pp de Wildt S, van Schaik R, Soldin OP, Soldin SJ, Brojeni PY, van der Heiden I, Parshuram C, Nulman I, Koren G. The interactions of age, genetics, and disease severity on tacrolimus dosing requirements after pediatric kidney and liver transplantation. European Journal of Clinical Pharmacology 2011: 67: pp Finkelstein Y, Soon GS, Acuna P, George M, Pope E, Ito S, Shear NH, Koren G, Shannon MW, Garcia-Bournissen F. Recurrence and long-term outcome of Stevens-Johnson syndrome and toxic epidermal necrolysis in children. Pediatrics 2011: 128: pp Gedeon C, Nava-Ocampo AA, Koren G. Ethical issues in pharmacologic research in women undergoing pregnancy termination: A systemic review and survey of researchers. Obstetrics Gynecology International 2012: Hutson JR, Garcia-Bournissen F, Davis A, Koren G. The human placental perfusion model: A systematic review and development of a model to predict in vivo transfer of therapeutic drugs. Clinical Pharmacology and Therapeutics 2011: 90: pp Ito S. A new dimension of clinical pharmacology: Induced pluripotent stem cells. Clinical Pharmacology and Therapeutics 2011: 89: pp Ito S. Pharmacokinetics 101. Paediatrics Child Health 2011: 16: pp Ito S. Placebo in clinical trials. Clinical Pharmacology and Therapeutics 2011: 90: pp Ito S, Nakamura H, Kobayashi T. Pediatric pharmacology in Japan. Pediatric Drugs 2012:14: pp Jong GW, Koren G. Maternal treatment with opioid analgesics and risk for birth defects. American Journal of Obstetrics and Gynecology 2011: 205: pp e10. Jong GW, Einarson T, Koren G, Einarson A. Antidepressant use in pregnancy and persistent pulmonary hypertension of the newborn (PPHN): A systematic review. Reproductive Toxicology 2012: May 5. (Epub ahead of print) Kahn SR, Almeida ND, McNamara H, Koren G, Genest J Jr, Dahhou M, Platt RW, Kramer MS. Smoking in preeclamptic women is associated with higher birthweight for gestational age and lower soluble fms-like tyrosine kinase-1 levels: A nested case control study. BMC Pregnancy Childbirth 2011: 11: pp 91. Kelly LE, Rieder MJ, Bridgman-Acker K, Lauwers A, Madadi P, Koren G. Are infants exposed to methadone in utero at an increased risk for mortality? Journal of Population Therapeutics and Clinical Pharmacology 2012: 19: pp e Kennedy D, Koren G. Identifying women who might benefit from higher doses of folic acid in pregnancy. Canadian Family Physician 2012: 58: pp Kim MW, Hong SC, Choi JS, Han JY, Oh MJ, Kim HJ, Nava-Ocampo A, Koren G. Homocysteine, folate and pregnancy outcomes. Journal of Obstetrics and Gynaecology 2012: 32: pp Klieger-Grossmann C, Weitzner B, Panchaud A, Pistelli A, Einarson T, Koren G, Einarson A. Pregnancy outcomes following use of escitalopram: A prospective comparative cohort study. Journal of Clinical Pharmacology 2012: 52: pp Koren G. Fetal risks of maternal pharmacotherapy: Identifying signals. Handbook of Experimental Pharmacology 2011: 205: pp Koren G. Folic acid and colorectal cancer: Unwarranted fears. Canadian Family Physician 2011: 57: pp Koren G. Pharmacokinetics in pregnancy: Clinical significance. Journal of Population Therapeutics and Clinical Pharmacology 2011: 18: pp e Koren G. Hypertension: ACE inhibitor use in pregnancy - Setting the record straight. Nature Reviews Cardiology 2011: 9: pp 7-8. Koren G, Elzagallaai A, Etwel F. Safety assessment in pediatric studies. Handbook of Experimental Pharmacology 2011: 205: pp Koren G, Nordeng H. SSRIs and persistent pulmonary hypertension of the newborn. British Medical Journal 2012: 344: d7642. Koren G, Nulman I, Aleksa K, Gareri J, Einarson A, Ito S. Essay for the 2011 CIHR/CMAJ award: Motherisk Caring for mothers, protecting the unborn. Canadian Medical Association Journal 2012: 184: pp E Lam J, Kelly L, Ciszkowski C, Landsmeer ML, Nauta M, Carleton BC, Hayden MR, Madadi P, Koren G. Central nervous system depression of neonates breastfed by mothers receiving oxycodone for postpartum analgesia. Journal of Pediatrics 2012: 160: pp Lee CY, Marcotte F, Giraldeau G, Koren G, Juneau M, Tardif JC. Digoxin toxicity precipitated by clarithromycin use: Case presentation and concise review of the literature. Canadian Journal of Cardiology 2011: 27: pp 870.e15-6. Lee JE, Han JY, Choi JS, Ahn HK, Lee SW, Kim MH, Ryu HM, Yang JH, Ocampo AA, Koren G. Pregnancy outcome after exposure to the probiotic Lactobacillus in early pregnancy. Journal of Obstetrics and Gynaecology 2012: 32: pp Levy A, Matok I, Gorodischer R, Sherf M, Wiznitzer A, Uziel E, Koren G. Bias toward the null hypothesis in pregnancy drug studies that do not include data on medical terminations of pregnancy: The folic acid antagonists. Journal of Clinical Pharmacology 2012: 52: pp Lo A, Sienna J, Mamak E, Djokanovic N, Westall C, Koren G. The effects of maternal supplementation of polyunsaturated fatty acids on visual, neurobehavioural, and developmental outcomes of the child: A systematic review of the randomized trials. Obstetrics and Gynecology International 2012: Mackenzie C, Syed J, Pollak PT, Koren G. Falling between the cracks: A case of amiodarone toxicity. Canadian Medical Association Journal 2011: 183: pp the hospital for sick children department of paediatrics Annual Report

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22 MacLeod S, Ito S. Walking the tightrope. Paediatrics Child Health 2011: 16: pp 527. Madadi P, Avard D, Koren G. Pharmacogenetics of opioids for the treatment of acute maternal pain during pregnancy and lactation. Current Drug Metabolites 2012: 13: pp Matlow J, Koren G. Is carbamazepine safe to take during pregnancy? Canadian Family Physician 2012: 58: pp Matok I, Levy A, Wiznitzer A, Uziel E, Koren G, Gorodischer R. The safety of fetal exposure to proton-pump inhibitors during pregnancy. Digestive Diseases and Sciences 2012: 57: pp Moller M, Koren G, Karaskov T, Garcia-Bournissen F. Examining the health and drug exposures among Canadian children residing in drug-producing homes. Journal of Pediatrics 2011: July 22. Moretti ME, Caprara D, Drehuta I, Yeung E, Cheung S, Federico L, Koren G. The fetal safety of angiotensin converting enzyme inhibitors and angiotensin II receptor blockers. Obstetrics Gynecology International 2012: Nash K, Koren G, Rovet J. A differential approach for examining the behavioral phenotype of fetal alcohol spectrum disorders. Journal of Population Therapeutics and Clinical Pharmacology 2011: 18: pp e Nurmohamed L, Moretti ME, Schechter T, Einarson A, Johnson D, Lavigne SV, Erebara A, Koren G, Finkelstein Y. Outcome following high-dose methotrexate in pregnancies misdiagnosed as ectopic. American Journal of Obstetrics and Gynecology 2011: 205: pp 533.e1-3. Osadchy A, Ratnapalan T, Koren G. Ocular toxicity in children exposed in utero to antimalarial drugs: Review of the literature. Journal of Rheumatology 2011: 38: pp Pupco A, Bozzo P, Koren G. Herpes zoster during pregnancy. Canadian Family Physician 2011: 57: pp Pupco A, Bozzo P, Koren G. Selective serotonin reuptake inhibitors and risk for major congenital anomalies. Obstetrics and Gynecology 2011: 118: pp Ross LE, Grigoriadis S, Mamisashvili L, Koren G, Steiner M, Dennis CL, Cheung A, Mousmanis P. Quality assessment of observational studies in psychiatry: An example from perinatal psychiatric research. International Journal of Methods in Psychiatric Research 2011: 20: pp Russell E, Koren G, Rieder M, Van Uum S. Hair cortisol as a biological marker of chronic stress: Current status, future directions and unanswered questions. Psychoneuroendocrinology 2011: Oct 3. Stade B, Beyene J, Buller K, Ross S, Patterson K, Stevens B, Sgro M, Ungar W, Watson W, Koren G. Feeling different: The experience of living with fetal alcohol spectrum disorder. Journal of Population Therapeutics and Clinical Pharmacology 2011: 18: pp e Stevens S, Major D, Rovet J, Koren G, Fantus E, Nulman I, Desrocher M. Social problem-solving in children with fetal alcohol spectrum disorders. Journal of Population Therapeutics Clinical Pharmacology 2012: 19: pp e Schwarz EB, Parisi SM, Handler SM, Koren G, Cohen ED, Shevchik GJ, Fischer GS. Clinical decision support to promote safe prescribing to women of reproductive age: A clusterrandomized trial. Journal of General Internal Medicine 2012: Feb 2 (Epub ahead of print) VanderVaart S, Berger H, Sistonen J, Madadi P, Matok I, Gijsen VM, de Wildt SN, Taddio A, Ross CJ, Carleton BC, Hayden MR, Koren G. CYP2D6 polymorphisms and codeine analgesia in postpartum pain management: A pilot study. Therapeutic Drug Monitoring 2011: 33: pp Vandervaart S, Berger H, Tam C, Goh YI, Gijsen VM, de Wildt SN, Taddio A, Koren G. The effect of distant reiki on pain in women after elective Caesarean section: A double-blinded randomized controlled trial. British Medical Journal 2011: 1: pp e Walfisch A, Matok I, Sermer C, Koren G. Weight-depression association in a high-risk maternal population. Journal of Maternal Fetal Neonatal Medicine 2011: Oct 4. Yazdani BP, Matok I, Garcia Bournissen F, Koren G. A systematic review of the fetal safety of interferon alpha. Reproductive Toxicology 2011: Dec 19. Zelner I, Hutson JR, Kapur BM, Feig DS, Koren G. Falsepositive meconium test results for fatty acid ethyl esters secondary to delayed sample collection. Alcohol Clinical and Experimental Research 2012: Mar 20. (Epub ahead of print) Zelner I, Shor S, Lynn H, Roukema H, Lum L, Eisinga K, Koren G. Clinical use of meconium fatty acid ethyl esters for identifying children at risk for alcohol-related disabilities: The first reported case. Journal of Population Therapeutics and Clinical Pharmacology 2012: 19: pp e Books and Chapters Nulman I, Citron S, Todorow M, Uleryk E. Neurodevelopment of children exposed to antidepressant and antipsychotic medications during pregnancy. In: Alcohol, Drugs and Medication in Pregnancy: The Long Term Outcome for the Child (Preece PM, Riley EP, eds). Mac Keith Press, London 2011: pp Natekar A, Pupco A, Bozzo P, Koren G. Safety of azathioprine use during pregnancy. Canadian Family Physician 2011: 57: Nulman I, Edell H. Paediatric outcomes following in utero exposure to the diagnosis and treatment of maternal malignancy. European Journal of Cancer 2011: 47 Suppl 3: pp S Nordeng H, van Gelder MM, Spigset O, Koren G, Einarson A, Eberhard-Gran M. Pregnancy outcome after exposure to antidepressants and the role of maternal depression: Results from the Norwegian Mother and Child Cohort Study. Journal of Clinical Psychopharmacology 2012: 32: pp Shrim A, Koren G, Yudin MH, Farine D. Maternal Fetal Medicine Committee. Management of varicella infection (chickenpox) in pregnancy. Journal of Obstetrics and Gynaecology Canada 2012: 34: pp Review. Sistonen J, Madadi P, Ross CJ, Yazdanpanah M, Lee JW, Landsmeer ML, Nauta M, Carleton BC, Koren G, Hayden MR. Prediction of codeine toxicity in infants and their mothers using a novel combination of maternal genetic markers. Clinical Pharmacology and Therapeutics 2012: 91: pp Verma P, Clark CA, Spitzer KA, Laskin CA, Ray J, Koren G. Use of non-aspirin NSAIDs during pregnancy may increase the risk of spontaneous abortion. Evidence Based Nursing 2012: 15: pp Walfisch A, Sermer C, Matok I, Einarson A, Koren G. Perception of teratogenic risk and the rated likelihood of pregnancy termination: Association with maternal depression. Canadian Journal of Psychiatry 2011: 56: pp Nulman I, Pulver A. Fetal alcohol spectrum disorder. Part I: Triumphs and challenges. In: Developmental Disabilities in Ontario (Brown I, Percy M, eds). Ontario Association on Developmental Disabilities, Toronto 2011: pp Nulman I, Tobias C, Uleryk E. Long term neurodevelopment of children exposed in utero to treatment for maternal cancer. In: Cancer in Pregnancy and Lactation: The Motherisk Guide (Koren G, Lishner M, eds). Cambridge University Press, Cambridge 2011: pp the hospital for sick children department of paediatrics Annual Report

23 Grants and Funding Access to data from the Norwegian Mother and Child Cohort. Koren G. The University of Oslo and the Foundation for the Promotion of Norwegian Pharmacies. 36,000 Euro to 2011 Bioavailability of amoxicillin dissolved in human milk. Ito S. Women Health Organization. $481,188 USD Biologically-based outcome predictors in juvenile radiopathic arthritis. Koren G. Canadian Institute of Health Research. $1,250,000 CAD to 2012 Canadian Pharmacogenetic Network for Drug Safety. Koren G. $24,000,000 CAD to 2014 Development of clinical practice guideline for pharmacogenetic testing. Koren G. Canadian Institute of Health Research. $83,940 CAD to 2012 Developing GeneMed for Canada: Pilot study. Ito S. University of Toronto Center for Applied Genomics. $60,000 CAD to 2012 Drug in Lactation Analyses Consortium (DLAC). Ito S. CIHR and DSEN (Catalyst grant). $97,150 CAD to 2013 Drugs and human milk (MT13747: renewal (1996,1999,2002,2007,2010). Ito S. Canadian Institutes of Health Research. $602,292 CAD to 2015 Genotype specific approaches to therapy in childhood (GATC); Canadian Pharmacogenomic Network for Drug Safety (CPNDS): for SickKids. Ito S. Canadian Institute of Health Research. $105,000/yr CAD to 2014 Global Research in Paediatrics (GRiP). Ito S. European Union. 120,300 EUR to 2016 International registry for Gilenya in multiple sclerosis. Koren G. Novartis Inc. $595,000 CAD to 2017 Long term neurodevelopmental outcome after exposure to psychotropic drugs during pregnancy. Koren G. The Norwegian Research Council. 460,000 Euro to 2014 Medication use in pregnancy with focus on attitudes perception of risk and mental well-being. Koren G. The University of Oslo. 225,000 Euro to 2013 Neurocognitive-phenome, genome, epigenome and nutriome in childhood leukemia survivors: N-PhenoGENICS. Childhood Cancer-Late Effects of Treatment Team grant competition. Ito S. Canadian Institute of Health Research (CIHR). $1,898,381 CAD to 2017 Neurodevelopment of children exposed in utero to chemotherapy for maternal breast cancer. Nulman I. Canadian Breast Cancer Foundation (CBCF). $436,257 CAD to 2013 Pilot project--diagnosis of FASD in children in the care of Children s Aid Society of Toronto and Catholic Children s Aid Society. Nulman I. Mothercraft. $55,000/yr CAD to 2011 Pre-emptive use of diclectin. Koren G. Duchesnay Inc. Canada. $135,000/yr CAD to 2011 Program of paediatric pharmacogenomics medicine: GeneMed. Ito S. Ontario Ministry of Health and Long term Care Drug Innovation Fund. $589,697 CAD to 2014 Studies on pregnancy exposure to medications and vaccines. Koren G. Organization of Teratology Information Services (OTIS). $5,300,000 US to 2012 Testing the fetal programming hypothesis. Koren G. Canadian Institute of Health Research: CHIP-Child. $1,150,000 CAD to 2016 The association between fatty acid ethyl esters in meconium and the diagnoses of FASD in an at-risk Canadian population. Nulman I. Canadian Foundation on Fetal Alcohol Research. $54,950 CAD to 2011 The Ivey Chair in Molecular Toxicology. Koren G. Canadian Institute of Health Research. $250,000/yr CAD to 2013 The role of placenta in fetal toxicology. Koren G. Canadian Institute of Health Research. $584,309 CAD to 2011 Environmental team grant: Effects of brominated flame retardants on reproductive health on development. Koren G. Canadian Institute of Health Research. $2,500,000 CAD to 2015 GeneMed in children. Koren G. Ontario Ministry of Health. $255,000 CAD to 2014 Pain in preterm infants in development and effects. Koren G. National Institute of Health Research. $1,304,369 CAD to 2012 Pharmacokinetics of folate in pregnancy. Koren G. Duchesnay Inc. Canada. $96,000/yr CAD to 2012 The role of the placenta in fetal toxicology. Ito S. Canadian Institute of Health Research. $140,000/yr CAD to 2011 The Vulnerable Infant Population (VIP) perinatal stress study. Koren G. American Association of Critical Nurses. $72,000 US to 2012 Use of antidepressant medications during pregnancy and lactation: Development of an evidence-based decision tool. Koren G. Ontario Ministry of Health. $294,357 CAD to the hospital for sick children department of paediatrics Annual Report

24 DEVELOPMENTAL PAEDIATRICS overview Division Head Dr. Darcy Fehlings Full Time Dr. Evdokia Anagnostou Dr. Douglas Biggar Dr. Jolene Huber Dr. Ryan Hung Dr. Elizabeth Jimenez Dr. Anne Kawamura Dr. Alvin Loh Dr. Laura McAdam Dr. Golda Milo-Manson Dr. Angela Orsino Dr. Wendy Roberts Dr. Jay Rosenfield Dr. Peter Rumney Dr. Sharon Smile Part Time and/or Community Physicians Dr. Paige Church Dr. Janine Flanagan Dr. Cynthia Goldfarb Dr. Mark Handley-Derry Dr. Carolyn Hunt Dr. Anita Jethwa Dr. Nicola Jones-Stokreef Dr. Peggy Kirkpatrick Dr. Zina Levichek Dr. Anne Richards Dr. Lilly Rojnica Dr. Clive Schwartz Dr. David Shawn Dr. Diann Superina-Bell Cross Appointees/ Scientific Staff Dr. Jessica Brian Dr. Melissa Carter Dr. Patricia McKeever Fellows Dr. Andrea Hoffman Dr. Joelene Huber Dr. Shannon Knights Dr. Deepali Mankad Dr. Ripu Minhas Dr. Melanie Penner Administrative Staff at The Hospital for Sick Children Maureen Hodge Barbara Zimnowodzki Administrative Staff at Holland Bloorview Kids Rehabilitation Hospital Merlene Chin Joanne Jenah Romana Karnetova Sharon Ouellette The Division of Developmental Paediatrics is situated at the Holland Bloorview Kids Rehabilitation Hospital. The division consists of full-time academic clinicians as well as a community section of developmental paediatricians. The main clinical focus of the division is on childhood neuro-developmental and neuro-rehabilitation disorders. Comprehensive assessment and treatment programs are run for children and youth with conditions such as cerebral palsy, acquired brain injury, neuromuscular disorders and spina bifida. Inpatient services are provided for children with medical conditions requiring complex continuing care. Specialized assessment clinics provide diagnostic assessments for children and youth with autism spectrum disorders with clinics located at Holland Bloorview as well as regional developmental clinics situated throughout the metropolitan Toronto area. The division runs a large psychopharmacology clinic providing consultation for children and youth with complex neuro-behavioural needs. We are pleased to welcome Dr. Sharon Smile to our division to provide academic leadership in preschool developmental disorders. The main clinical focus of the division is on childhood neuro-developmental and neuro-rehabilitation disorders. Within the division, there is a strong commitment to education. Under the leadership of Dr. Anne Kawamura as program director, the fully accredited subspecialty residency program in Developmental Paediatrics is the largest in Canada. There is also a commitment to training paediatric, physiatry, and neurology residents and medical students in applied childhood disability. A highlight of the divisions educational activities is the Child Development Update a national conference held once every three years chaired by Dr. Golda Milo-Manson and supported by the continuing educational office of The Hospital for Sick Children and the Department of Paediatrics. The conference attracts attendees from across Canada. The Division members actively lead research projects in applied childhood disability studies, in association with the Bloorview Research Institute. Highlights include the creation of two Ontariobased research networks funded by the Ontario Brain Institute one focused on autism spectrum disorders led by Dr. Evdokia Anagnostou and one focused on cerebral palsy led by Dr. Darcy Fehlings. department of paediatrics Annual Report

25 Honours and Awards Penner, M: Chronic Pain in Children and Youth with Cerebral Palsy - Winner of best Developmental Paediatrics Abstract Award at the Canadian Paediatric Society Annual Conference (Supervisor: D. Fehlings), McAdam, L: Elsevier WMS Membership Award, World Muscle Society, Algarve, Portugal, October Roberts, W: Continuing Education Award, Department of Paediatrics, The Hospital for Sick Children, June Publications Ameis SH, Fan J, Rockel C, Voineskos AN, Lobaugh NJ, Soorya L, Wang AT, Hollander E, Anagnostou E. Impaired structural connectivity of socio-emotional circuits in autism spectrum disorders: A diffusion tensor imaging study. PLoS One 2011: 6 (11): e Epub 2011 Nov 23. Anagnostou E, Hansen R. Medical treatment overview: Traditional and novel psycho-pharmacological and complementary and alternative medications. Current Opinion in Pediatrics 2011: Dec 23 (6): pp Anagnostou E, Chaplin W, Watner D, Silverman JM, Smith CJ, Zagursky K, Kryzak LA, Corwin TE, Feirsen N, Tanel N, Hollander E. Factor analysis of repetitive behaviors in autism as measured by the Y-BOCS. Journal of Neuropsychiatry and Clinical Neuroscience 2011: 23 (3): pp Beaudet AL. Clinical practice pathways for evaluation and medication choice for attention deficit hyperactivity disorder symptoms in Autism Spectrum Disorders. Special supplement for Pediatrics. Proceedings of the National Academy Sciences, USA 2012: 109 (21): pp Bennett T, Boyle M, Georgiades K, Georgiades S, Thompson A, Duku E, Bryson S, Fombonne E, Vaillancourt T, Zwaigenbaum L, Smith I, Mirenda P, Roberts W, Volden J, Waddell C, Szatmari P, and The Pathways in ASD Study Team. Influence of reporting effects on the association between maternal depression and child autism spectrum disorder behaviors. Journal of Child Psychology and Psychiatry 2012: 53 (1): pp Carter MT, Boycott KM. Microcephaly capillary malformation syndrome: A story of rapid emergence of a new recognizable entity. American Journal of Medical Genetics Part A 2011: 155 (9): pp Carter MT, Geraghty MT, De La Cruz L, Reichard RR, Boccuto L, Schwartz CE, Clericuzio CL. New syndrome with multiple capillary malformations, intractable seizures, and brain and limb anomalies. American Journal of Medical Genetics Part A 2011: 155: pp Celestino-Soper PB, Violante S, Crawford EL, Luo R, Lionel AC, Delaby E, Cai G, Sadikovic B, Lee K, Lo C, Gao K, Person RE, Moss TJ, German JR, Huang N, Shinawi M, Treadwell-Deering D, Szatmari P, Roberts W, Fernandez B, Schroer RJ, Stevenson RE, Buxbaum JD, Betancur C, Scherer SW, Sanders SJ, Geschwind DH, Sutcliffe JS, Hurles ME, Wanders RJ, Shaw CA, Leal SM, Cook EH Jr, Goin-Kochel RP, Vaz FM, Beaudet AL. A common X-linked inborn error of carnitine biosynthesis may be a risk factor for nondysmorphic autism. Special supplement for Pediatrics. Proceedings of the National Academy Sciences, USA 2012: 109 (21): pp Epub 2012 Duerden EG, Mak-Fan KM, Taylor MJ, Roberts SW. Regional differences in grey and white matter in children and adults with autism spectrum disorder: An activation likelihood estimate (ALE) meta-analysis. Autism Research 2011: Dec 2 doi / aur.235 (Epub ahead of print). Duerden EG, Oatley, HK, Mak-Fan KM, McGrath PA, Taylor MJ, Roberts SW, Szatmari P. Risk factors associated with self-injurious behaviors in children and adolescents with autism spectrum disorders. Journal of Autism and Developmental Disorders 2012: Mar 16 (Epub ahead of print). Fehlings D, Narayanan U, Andersen J, Beauchamp R, Gorter JW, Kawamura A, Kiefer G, Mason M, McCormick A, Mesterman R, Switzer L, Watt J. Botulinum toxin-a use in paediatric hypertonia: Canadian practice patterns. Canadian Journal of Neurological Science 2012: 39 (4): pp Fehlings D, Switzer L, Agarwal P, Wong C, Sochett E, Stevenson R, Sonnenberg L, Smile S, Young E, Huber J, Milo-Manson G, Abu Kuwaik G, Gaebler D. Informing evidence-based clinical practice guidelines for children with cerebral palsy at risk of osteoporosis: A systematic review. Developmental Medicine and Child Neurology 2012: 54: pp Gibson BE, Teachman G, Wright V, Fehlings D, Young NL, McKeever P. Children s and parents beliefs regarding the value of walking: Rehabilitation implications for children with cerebral palsy. Child: Care, Health and Development 2012: 38 (1): pp Girgis RR, Slifstein M, Xu X, Frankle WG, Anagnostou E, Wasserman S, Pepa L, Kolevzon A, Abi-Dargham A, Laruelle M, Hollander E. The 5-HT(2A) receptor and serotonin transporter in Asperger s disorder: A PET study with (¹¹C)MDL and (¹¹C)DASB. Psychiatry Research 2011: 194 (3): pp Hollander E, Soorya L, Chaplin W, Anagnostou E, Taylor BP, Ferretti CJ, Wasserman S, Swanson E, Settipani C. A double-blind placebo-controlled trial of fluoxetine for repetitive behaviors and global severity in adult autism spectrum disorders. American Journal of Psychiatry 2012: 169d: (3): pp Erratum in American Journal of Psychiatry 2012: 9(5): pp 540. Howcroft J, Fehlings D, Zabjek K, Fay L, Liang J, Biddiss E. Wearable wrist activity monitor as an indicator of functional hand use in children with cerebral palsy. Developmental Medicine and Child Neurology 2011: 53(11): pp Howcroft J, Klejman S, Fehlings D, Wright D, Zabjek K, Andrysek J, Biddiss E. Active video game play in children with cerebral palsy: Potential for physical activity promotion and rehabilitation therapies Archives of Physical Medicine and Rehabilitation 2012: (doi: apmr ). Kawamura S, Klejman D, Fehlings D. Reliability and validity of the Kinematic Dystonia Measure for children with upper extremity dystonia. Journal of Child Neurology 2012: 27: pp Kushki A, Chau T, Anagnostou E. Handwriting difficulties in children with autism spectrum disorders: A scoping review. Journal of Autism and Developmental Disorders 2011: 41 (12): pp Review. Erratum in Journal of Autism and Developmental Disorders 2011: 41 (12): pp Lionel AC, Crosbie J, Barbosa N, Goodale T, Thiruvahindrapuram B, Rickaby J, Gazzellone M, Carson AR, Howe JL, Wang Z, Wei J, Stewart AFR, Roberts R, McPherson R, Fiebig A, Franke A, Schreiber S, Zwaigenbaum L, Fernandez BA, Arnold PD, Szatmari P, Marshall CR, Schachar R, Scherer SW. Rare copy variation discovery and cross-disorder comparisons identify risk genes for ADHD. Science Translational Medicine 2011: 3 (95): pp 95 ra75. Mak-Fan KM, Morris D, Vidal J, Anagnostou E, Roberts W, Taylor MJ. White matter and development in children with an autism spectrum disorder. Autism 2012: Jun 14. (Epub ahead of print) PubMed PMID: Ohasi JK, Mirenda P, Marinova-Todd S, Hambly C, Fombonne E, Szatmari P, Bryson S, Roberts W, Smith I, Vaillancourt T, Volden J, Waddell C, Zwaigenbaum L, Georgiades S, Duku E, Thompson A, The Pathways in ASD Study Team. Comparing early language development in monolingual and bilingual exposed young children with autism spectrum disorders. Research in Autism Spectrum Disorders 2012: 6: pp Sato D, Lionel AC, Leblond CS, Prasad A, Pinto D, Walker S, O Connor I, Russell C, Drmic IE, Hamdan FF, Michaud JL, Endris V, Roeth R, Delorme R, Huguet G, Leboyer M, Rastam M, Gillberg C, Lathrop M, Stavropoulos DJ, Anagnostou E, Weksberg R, Fombonne E, Zwaigenbaum L, Fernandez BA, Roberts W, Rappold GA, Marshall CR, Bourgeron T, Szatmari P, Scherer SW. SHANK1 deletions in males with autism spectrum disorder. American Journal of Human Genetics 2012: 90 (5): pp Epub 2012 Apr the hospital for sick children department of paediatrics Annual Report

26 Scahill L, McCracken JT, Bearss K, Robinson F, Hollander E, King B, Bregman J, Sikich L, Dukes K, Sullivan L, Anagnostou E, Donnelly C, Kim YS, Ritz L, Hirtz D, Wagner A. Design and subject characteristics in the federally-funded citalopram trial in children with pervasive developmental disorders. Journal of Autism and Developmental Disorders 2012: 42 (3): pp Vaags AK, Lionel AC, Sato D, Goodenberger M, Stein QP, Curran S, Ogilvie C, Ahn JW, Drmic I, Senman L, Chrysler C, Thompson A, Russell C, Prasad A, Walker S, Pinto D, Marshall CR, Stavropoulos DJ, Zwaigenbaum L, Fernandez BA, Fombonne E, Bolton PF, Collier DA, Hodge JC, Roberts W, Szatmari P, Scherer SW. Rare deletions at the neurexin 3 locus in autism spectrum disorder. American Journal of Human Genetics 2012: 90 (1): pp Zaidman-Zait A, Mirenda P, Zumbo B, Georgiades S, Szatmari P, Bryson S, Fombonne E, Roberts W, Smith I, Vaillancourt T, Volden J, Waddell C, Zwaigenbaum L, Duku E, Thompson A, and the Pathways in ASD Study Team (2011). Factor analysis of the Parenting Stress Index-Short Form with parents of young children with autism spectrum disorders. Autism Research 2011: 4 (5): pp Zwaigenbaum L, Brian J, Bryson S, Smith I, Vaillancourt T, Szatmari P, Roberts W, Roncadin C. Sex differences in children with autism spectrum disorder identified within a high-risk infant cohort. Journal of Autism and Developmental Disorders 2012: Mar 28 (Epub ahead of print). Books and Chapters Anagnostou E, Bear M, Dawson G. Commentary: Future directions in the treatment of autism spectrum disorders. In: Autism Spectrum Disorders (Amaral D, Dawson G, Geschwind D, eds.). Oxford University Press. Anagnostou E, Rusoff J. Anticonvulsants in the treatment of autism. In: Autism Textbook of Autism Spectrum Disorders (Hollander E, Kolevzon A, Coyle J, eds.). PPI Press Machalek K, Percy M, Carter M, Brown I. Genetics of developmental disabilities. In: Developmental Disabilities in Ontario, Third edition. (Brown I, Percy M, eds.). Toronto: Ontario Association on Developmental Disabilities Perry A, Wells K, Carter M, Loh A. Autism and related disorders. In: Developmental Disabilities in Ontario, Third edition. (Brown I, Percy M, eds.). Toronto: Ontario Association on Developmental Disabilities Grants and Funding A pilot dose finding study of pioglitazone in children with ASD. Anagnostou E, Brian J. PSI. $127,700 CAD. Sep 2010 to Aug 2012 Active video games for promotion of physical activity in children and youth with disabilities: Risks and benefits. Biddiss E, McKeever P, Wright V, Chau T, Fehlings D. Canadian Institutes of Health Research. $147,272 CAD. Feb 2010 to Jan 2013 Advancing vocational opportunities for persons with autism spectrum disorders. Workshop Brain Camp. Nicholas D, Roberts SW. NeuroDevNet. $15,000 CAD An evaluation of the long-term effectiveness of Botulinum toxin in children with spastic cerebral palsy. Fehlings D, Goldsmith C, Narayanan U, Rosenbaum P, Wright W. Canadian Institutes of Health Research. $442,307 CAD. Apr 2008 to Mar 2012 Autism comes to the hospital: Experiences of hospital care from the perspectives of children and adolescents with autism spectrum disorder, their parents and health care providers. Muskat B, Roberts S, Nicholas D, Stoddart K, Zwaigenbaum L. Sick Kids Foundation and The Institute of Human Development, Child and Youth Health-CIH. $190,000 CAD to 2012 Autism spectrum and associated neurodevelopmental disorders: Genomes to outcomes. Scherer SW, Szatmari P, Anagnostou E, Roberts W, Arnold P, Bassett A, Brudno M, Holden J, Paterson A, Schachar R, Vincent JB, Weksberg R. Ontario Research Fund (ORF). $8,920,652 CAD. Oct 2010 to Sep 2015 Autism spectrum disorders: Pathways to better outcomes (Phase II). Szatmari P, Roberts W, Bryson SE, Fombonne E, Zwaigenbaum L, Smith I, Waddell C, Vaillancourt T, Mirenda P, Volden J. Canadian Institutes of Health Research. $1,712,750 CAD to 2014 Autism Treatment Network Registry. Loh A, Roberts W, Anagnostou E, Marcon M, Weiss S, Brian J, Feigenbaum A, Schulze A, Logan W, Chitiyat D, Weksberg R, Green P, Weiss J, Mitchell S, Berall G, Bradley E, Freeman N, Stinson J, Ornstein M, Atkinson A, Goldfarb C, Flanagan J, Superina D, Jimenez E, Jones Stokreef N, Orsino A, Isaacs B, Handley-Derry M. Autism Speaks. $420,000 CAD. Jan 2011 to Dec 2013 Biometric authentication using handwriting biomechanics. Chau T, Anagnostou E, Fleury A. Natural Sciences and Engineering Research Council of Canada (NSERC). $340,000 CAD. Jun 2010 to May 2013 Canadian Cerebral Palsy Registry. Goldowitz D, Shevell M, Fehlings M, Fehlings D, Scherer S, Majnemer A, Yager J. NeuroDevNet, Networks Centre of Excellence. $210,000 CAD. Jan 2010 to Dec 2014 Case formulation in developmental paediatrics: Seeing the process through the eyes of the expert. Kawamura A, Orsino A, Mylopoulos M. Pediatric Consultants Medical Education Research Grant, Hospital for Sick Children. $4,878 CAD to 2013 Childhood Hemiplegic Cerebral Palsy Integrated Neuroscience Discovery Network CP-NET. Fehlings D, Kawamura A, Wright V, Biddiss E, Chau T, Young N, Rosenbaum P, Mesterman R, Gorter JW, Pelland L, Scott S, Samdup D, deveber G, Scherer S, Taylor M, Shroff M, Henkelman M, Paterson A, Wintle R, Fehlings M, Chen R, Morshead C, van der Kooy D, Menon R, Campbell C. Ontario Brain Institute. $1,500,000 CAD. Apr 2012 to Mar 2013 CIHR training grant in autism research. Fombonne E, Roberts W, Bryson S, Paterson A, Scherer S, Szatmari P, Smith I, Moore C. Canadian Institutes of Health Research. $1,950,000 CAD to 2015 CP cycle to fun. Graham N, Fehlings D, Mandryk R, Warburton D, Rhodes R, Wright V. Natural Sciences and Engineering Research Council of Canada (NSERC), CIHR, CHIR-IMHA. $510,978 CAD. Apr 2012 to Mar 2015 CP fit n fun project equipment grant. Fehlings D, Switzer L, Graham N. TELUS. $17,500 CAD. Apr 2012 to Mar 2013 CP fit n fun project: Health and social benefits of virtual exercise games in adolescents with cerebral palsy. Fehlings D, Graham N, Wright V. NeuroDevNet Networks of Centres of Excellence of Canada (NCE). $150,000 CAD. Dec 2010 to Nov 2012 Delineating the neurodevelopmental phenotype and health outcomes in microduplication 22q11.2 syndrome. Carter M, Weksberg R, Drmic I, Butcher D, Roberts W, Anagnostou E, Scherer SW, Cytrynbaum C, Shugar A, Fishman L. Rare Disease Foundation Microgrant. $1,090 CAD to 2012 Dissociating cognitive and motor components of healthy and dysfunctional expressive language development in children. Pang E, Roberts W. Cheyne D, De Nil L. Canadian Institutes of Health Research. $776,210 CAD to 2013 Does current practice align with best practice for intensive hand function training in children with hemiplegia? A national environmental scan. Majnemer A, Shikako-Thomas D, Germain M, Fehlings D, Gordon A, Maynard D. School of Physical and Occupational Therapy, McGill University, Edith Strauss Rehabilitation Research Projects. $12,924 CAD. Feb 2012 to Jan 2013 Enhancement of developmental motor plasticity in perinatal stroke with TDCS. Kirton A, Hill MD, Fehlings D, Andersen J, Goodyear B, Dukelow S, Beaulieu C, Dewey D, Chen R, Majnemer A. Heart and Stroke Foundation. $270,000 CAD. Apr 2012 to Mar 2015 Evaluating outcomes of hip interventions in children with severe non-ambulatory cerebral palsy using the CPCHILD: A prospective cohort study. Narayanan U, Fehlings D, Weir S. Ontario Federation for Cerebral Palsy. $44,445 CAD. Feb 2010 to Jan the hospital for sick children department of paediatrics Annual Report

27 Functional outcomes following orthopaedic surgery based on gait laboratory versus observational gait analysis in ambulatory children with cerebral palsy: A multi-centre pilot randomized controlled trial. Narayanan U, Willan AR, Fehlings DL, Wedge JH, Zabjek KF. Canadian Institutes of Health Research. $314,633 CAD. Oct 2009 to Mar 2013 Genomic influences on brain and behavioural trajectories in autism spectrum disorders. Zwaigenbaum L, Anagnostou E, Scherer S, Evans A, Roberts W, Bryson S, Fombonne E, Mirenda P, Hall G, Szatmari P. NeuroDevNet. $2,643,200 CAD to 2013 Identifying risk factors associated with pain in children and youth with cerebral palsy and effectiveness of spasticity interventions in alleviating pain and improving health-related quality of life in children with cerebral palsy. Fehlings D, Switzer L, Narayanan U, Dupuis A. Allergan Canada. $109,953 CAD. Dec 2010 to Nov 2012 Improving early diagnosis and treatment for autism spectrum disorders. The Canadian Infant Sibling Project. Zwaigenbaum L, Brian J, Bryson S, Szatmari P, Roberts W, Smith I. Autism Speaks Canada. $1,008,704 CAD to 2014 Intranasal oxytocin for the treatment of children and adolescents with autism spectrum disorders. Anagnostou E, Roberts W, Brian J, Dupuis A, Jacobs S, Cook E. Department of Defense $805,791 CAD. Sep 2010 to Aug 2013 Intranasal oxytocin for social cognition/function in adults with autism. Anagnostou E, Szatmari P. Canadian Institutes of Health Research. $1,080,000 CAD. Jan 2012 to Dec 2016 Lokomat trial in robotic therapy. Fehlings D, Wright V. CIBC. $500,000 CAD; Three to Be Foundation. $450,000 CAD; KRG Children s Charitable Foundation $950,000 CAD. Jun 2012 to May 2014 Networks of Centres of Excellence (NCE). Goldowitz D, Zwaigenbaum L, Scherer SW, Szatmari P, Fombonne E, Collaborators: Roberts W, Brudno M, Cargonetto S, Craig AM, Hyde K, Evans A, Anagnostou E, Hall G, Bryson S, Smith I, Brian J, Volden J, Mirenda P, Bailey A, Vaillancourt T, Miller F, Nicholas D, Fernandez B. NeuroDevNet. $19,572,000 CAD ($3,248,000 June 2010 to May 2013) OptEC: Optimizing early child development in the primary care practice setting: Pragmatic randomized trial of iron treatment for young children with non-anemic iron deficiency. Parkin P, Birken C, Fehlings D, Macarthur C, Maguire J, Mamak E, Mamdan M, Thorpe K, Zlotkin S. Canadian Institutes of Health Research. $541,567 CAD. Jun 2011 to May 2016 OptEC: Optimizing early child development in the primary care practice setting: Pragmatic randomized trial of iron treatment for young children with non-anemic iron deficiency. Parkin P, Birken C, Maguire J, DeGroot J, Zlotkin S, Macarthur C, Fehlings D, Mamdani M, Thorpe K, Azad A. Physicians Services Inc. $168,359 CAD. May 2011 to Apr 2013 Province of Ontario Neurodevelopmental Disorders Network. Anagnostou, E, Scherer S, Szatmari P, Arnold P, Schachar R. Ontario Brain Institute. $3,500,000 CAD. Oct 2011 to Mar 2013 The art of waiting: Evaluation of an interactive media experience in a children s hospital clinic waiting space. Biddiss E, Fehlings D, McKeever P, McPherson A, Chau T, Rigby P. Canadian Institutes of Health Research. $138,714 CAD. Jul 2011 to Jun 2013 The Challenge Module for evaluating advanced motor skills of children with cerebral palsy: From measurement to childcentred goal setting. Wright V, Gibson B, Rosenbaum P, Fehlings D, Anderson J, Wood E, Mesterman R, Glazebrook C. Canadian Institutes of Health Research. $339,435 CAD. Apr 2012 to Mar 2015 The genetic epidemiology of autism: Family and molecular studies. Szatmari P, Zwaigenbaum L, Goldberg J, Roberts W, Bryson SE, Patterson J, Mahoney A, Merette W, Tuff L, Vincent J, Scherer S. Canadian Institutes of Health Research. $779,731 CAD to 2013 The Study of Toddlers with Autism and Regression (STAR) Protocol: Biomarkers of inflammation and immune activation in ASD. Loh A, James J, Menon D, Anagnostou E, Pardo C, Benseler S, Schulze A, Feigenbaum A, Logan W, Brian J, Burns K, Asghar S, Banwell B, Spence S, Bauman M, Zimmerman A, Beversdorf D, Sohl K. Health Resources and Services Administration (HRSA award UA3MC11054), Autism Intervention Research Network for Physical Disorders (AIR-P). $213,854 CAD to 2012 Therapists beliefs regarding the value of walking. Gibson B, Fehlings D, Narayanan U, Wright V, Teachman G. Connaught New Researcher Award, University of Toronto. $45,294 CAD. Apr 2012 to Mar 2013 Treatment of overweight induced by antipsychotic medication in young people with ASD. Anagnostou E, Veenstra-VanderWeele J, Handen B, Aman MG. HRSA. $1,800,000 CAD to 2015 Typical and atypical development of frontal lobe systems and the maturation of social cognitive function. Taylor M, Anagnostou E. Canadian Institutes of Health Research. $450,000 CAD. Jan 2012 to Jan 2017 Understanding early developmental trajectories in autism: From infancy to age 8. Zwaigenbaum L, Bryson S, Roberts SW Szatmari P, Smith I. Canadian Institutes of Health Research $1,098,859 CAD to 2015 Working memory function in children with autism. Taylor M, Anagnostou E. Canadian Institutes of Health Research. $353,921 CAD. Oct 2010 to Sep the hospital for sick children department of paediatrics Annual Report

28 ENDOCRINOLOGY overview Division Head Dr. Mark R. Palmert Full-Time Dr. Denis Daneman Dr. Jill Hamilton Dr. Farid Mahmud Dr. Kusiel Perlman Dr. Etienne Sochett Dr. Diane Wherrett Part-Time Dr. Jacqueline Curtis Dr. Susan Kirsch Dr. Dror Koltin Dr. Stacey Urbach Gynecology Dr. Lisa Allen Dr. Nicolette Caccia Dr. Sari Kives Dr. Alexandra Nevin-Lam Dr. Rachel Spitzer Dr. Anjali Aggarwal Fellows - Gynaecology Dr. Yolanda Kirkham Cross-Appointees Dr. Amira Klip Dr. Joanne Rovet Honorary Dr. Robert Ehrlich Subspecialty Residents/ Clinical Fellows Dr. Fahed Aljaser Dr. Sareea Al Remeithi Dr. Ereny Bassilious Dr. Michal Cohen Dr. Andrea Ens Dr. Jennifer Harrington Dr. Paola Luca Dr. Asaf Oren Dr. Rayzie Shulman Dr. Jonathan Wasserman Research Fellow Dr. Caroline Zuijdwijk Administrative Staff Shahla Amiri Angela Edoo Mary Mathew Elizabeth Rodgers Minet Smith Zarine Tilak was another productive year for the Division of Endocrinology with ongoing development of existing programs and new innovations. We continued our efforts to train future endocrinologists from Canada and from around the world. This year, we had 11 trainees in our subspecialty training program: Seven Canadians, two from Israel, one from Kuwait, one Australian and one from United Arab Emirates. Our areas of largest clinical volume continue to be: (i) Diabetes: our comprehensive program provides ongoing care to over 1,000 children and teens with diabetes, including patients with type 1 and type 2 diabetes; patients with diabetes that results from treatment of other disorders with agents such as glucorticoids that lead to insulin resistance; and patients with diabetes secondary to cystic fibrosis. We also have a program that provides comprehensive assessment and treatment of adolescents with chronically poor diabetes control. (ii) General Endocrinology: emphasizing the areas of growth, thyroid disease, disorders of sex development, pubertal disorders and reproductive health. (iii) Calcium/Bone: the Calcium-Bone and Bone Health Clinics assess children with abnormal bone health from a wide variety of causes as part of the multidisciplinary Bone Health Centre. (iv) Gynaecology: the Section of Paediatric and Adolescent Gynaecology provides care to pre-teen and adolescent girls across a wide spectrum of disorders and also provides care to pregnant adolescents. Our areas of largest clinical volume are diabetes, general endocrinology, calcium and bone and gynaecology. We also developed further our pathways for the care of children with thyroid cancer in collaboration with Oncology, Otolaryngology and Nuclear Medicine and expanded our programs to care for children with other endocrine tumors, such as adrenal cortical carcinoma and pheochromocytoma in collaboration with Oncology and Genetics. We also continued to provide care to survivors of paediatric cancer who develop endocrine abnormalities. department of paediatrics Annual Report

29 The SickKids Team Obesity Management Program (STOMP) continues to flourish. STOMP is an interdisciplinary medicalsurgical program for obese children and youth.. The program is intended for children and adolescents with complex, severe obesity defined as: greater than the 99th percentile for BMI, or greater than the 95th with an associated severe medical or psychosocial co-morbidity or additional chronic illness impacted by obesity (e.g. solid organ transplant, brain tumour-related hypothalamic obesity). STOMP is an intensive two-year program focusing on healthy lifestyle psychoeducation and cognitive behavioural therapy with access to surgical intervention (lap band bariatric procedure). The interdisciplinary team consists of three physicians (endocrinology, general paediatrics & general surgery), an exercise therapist, psychologist, dietitian, social worker, information coordinator and nurse practitioner. Our research efforts have targeted the following areas: (i) prediction and prevention of type 1 diabetes through involvement in the multinational TrialNet and TRIGR study groups; (ii) implications and treatment of insulin resistance and obesity among children and adolescents and the evolution of these abnormalities in young children; (iii) identification of factors affecting metabolic control and the early complications of diabetes; (iv) prevention and treatment of the medical complications of diabetes; (v) psychosocial and psychological outcomes in diabetes and other endocrine disorders; (vi) evaluation of disorders of calcium and bone mineral metabolism in pediatrics; and (vii) clinical and laboratory based investigation of the regulation and disorders of pubertal timing. Honors and Awards Allen, L: National Faculty Teaching Award, American College of Obstetricians and Gynecologists. The Council on Resident Education in Obstetrics and Gynecology, Daneman, D: Fellow, Canadian Academy of Health Sciences. Hamilton, J: Award for Excellence in Clinical Teaching, Hospital for Sick Children, Department of Paediatrics, Kives, S: Chair s Award for Research Facilitation, Department of Obstetrics and Gynaecology, Faculty of Medicine, University of Toronto(Continuing Education), Kives, S: Paul McCleary Award, St Michaels Hospital Medical Education Award, Toronto, (Distinction), Klip, A: Canada Research Chair in Cell Biology of Insulin Action, Tier I Palmert, M: Elected President of the Canadian Pediatric Endocrine Group Palmert, M: Elected to Board of Directors, Pediatric Endocrine Society (formerly LWPES) Publications Allen LM, Benacci JC, Trane RN III, Driscoll RJ. A case of neonatal compartment syndrome: Importance of early diagnosis in a rare and debilitating condition. American Journal Perinatology 2012: 27 (2): pp Allen LM, Lam AC. Premenstrual syndrome and dysmenorrhea in adolescents. Adolescent Medicine State of the Art Reviews 2012: 23 (1): pp Amed S, Dean H, Sellers EA, Panagiotopoulos C, Shah BR, Booth GL, Laubscher TA, Dannenbaum D, Hadjiyannakis S, Hamilton JK. Risk factors for medication-induced diabetes and type 2 diabetes. Journal of Pediatrics 2011: 159 (2): pp Amed S, Hamilton JK, Sellers EA, Panagiotopoulos C, Hadjiyannakis S, Shah BR, Booth GL, Laubscher TA, Dannenbaum D, Dean H. Differing clinical features in Aboriginal vs. non-aboriginal children presenting with type 2 diabetes. Pediatric Diabetes 2012 (Epub ahead of print). Borgono CA, Hamilton JK, Ye C, Hanley AJ, Connelly PW, Sermer M, Zinman B, Retnakaran R. Determinants of insulin resistance in infants at age 1 year: Impact of gestational diabetes mellitus. Diabetes Care 2012: 35 (8): pp Cafazzo JA, Eng P, Casselman M, Hamming N, Katzman DK, Palmert MR. Design of an mhealth App for self-management of adolescent type 1 diabetes A pilot study. Journal Medicine Internet Research 2012: 14(3): e70. Cohen M, Guger S, Hamilton J. Long term sequelae of pediatric craniopharyngioma - Literature review and 20 years of experience. Frontiers in Endocrinology (Lausanne) 2011: 2: pp 81. Daneman D, Daneman M. What has attachment theory got to do with diabetes care. Diabetes Management 2012: 2: pp Daneman D, Kellner J. Navigating the various stages of an academic career in pediatrics. Paediatrics and Child Health 2012: 17: pp Dumont T, Allen L, Kives S. Can von Willebrand disease be investigated on combined hormonal contraceptives? Journal of Pediatric and Adolescent Gynecology 2011: pp (Electronic). Dumont T, Caccia N, Allen L. Pediatric synchronous bilateral ovarian torsion: A case report and review of the literature. Journal of Pediatric Surgery 2011: 46 (12): pp e Gilbert ME, Rovet J, Chen ZP, Koibuchi N. Developmental thyroid hormone disruption: Prevalence, environmental contaminants and neurodevelopmental consequences. Neurotoxicology 2011: e 25. Kewalramani G, Fink LN, Asadi F, Klip A. Palmitate-activated macrophages confer insulin resistance to muscle cells by a mechanism involving orotein kinase C θ and ε. PLoS One 2011: 6: pp e Our newest research endeavour, the Juvenile Diabetes Research Foundation s (JDRF s) new Canadian Clinical Trial Network (CCTN), is now a year old. The CCTN was created in partnership with the Government of Canada s Federal Economic Development Agency for Southern Ontario (FedDev Ontario) and is charged with conducting trials aimed at identifying better treatments for type 1 diabetes. SickKids is one of three lead sites for the CCTN, and the initial trials are now underway. Abla O, Palmert MR. Reversal of LCH-related diabetes insipidus and appearance of posterior pituitary bright spot with low-dose chemotherapy. Pediatric Blood Cancer 2012: 59(1): pp Epub 2012: Jan 4. Allen LM. Prenatal 3-dimensional imaging techniques in the sonographic evaluation of an oral mass: Comparison with postnatal imaging modalities. Journal of Ultrasound Medicine 2012: 30 (4): pp Chan Y-M, Broder-Fingert A, Paraschos S, Lapatto R, Au M, Hughes V, Bianco ADC, Min L, Plummer L, Cerrato F, De Guillebon A, Wahab F, Dwyer A, Kirsch S, Quinton R, Cheetham T, Ozata M, Ten S, Chanoine JP, Pitteloud N, Crowley WF, Martin KA, Schiffman RA, Hall JE, Kaiser UB, Seminara SB. GnRH-deficient phenotypes in humans and mice with heterozygous variants in KISS/kiss 1. Journal of Clinical Endocrinology and Metabolism 2011: 96(11): pp E Kirkham YA, Kives S. Ovarian cysts in adolescents: Medical and surgical management. Adolescent Medicine State of the Art Reviews 2012: 23 (1): pp Kirkham YA, Lacy JA, Kives S, Allen L. Characteristics and management of adnexal masses in a Canadian pediatric and adolescent population. Journal of Obstetrics and Gynaecology Canada 2011: 33 (9): pp the hospital for sick children department of paediatrics Annual Report

30 Koltin D, Sung L, Naqvi A, Urbach SL. Medication induced diabetes during induction in pediatric acute lymphoblastic leukemia: Prevalence, risk factors and characteristics. Supportive Care in Cancer 2012: Epub 2011 Nov 8: pp Nash K, Koren G, Rovet J. A differential approach for examining the behavioural phenotype of fetal alcohol spectrum disorders. Journal of Population Therapeutics and Clinical Pharmacology 2011: 18: e pp Sud S, Marcon M, Assor E, Daneman D, Mahmud FH. Caregiver attitudes regarding celiac disease screening and treatment in pediatric type 1 diabetes. Canadian Journal of Diabetes 2011: 35(4): pp Yeshayahu Y, Tallett S, Pacak K, De Souza C, Palmert MR. When is a pheao not a pheao? Depression in an adolescent leading to a pheaochromocytoma-like biochemical profile. Clinical Endocrinology 2011: 75(4): pp Kulik D, Mahmud FH. A true case of neonatal salt wasting? Pediatrics in Review 2011: 32(3): pp 123. Laffel L, Chang N, Grey M, Hale D, Higgins L, Hirst K. Izquierdo R, Larkin M, Macha C, Pham T, Wauters A, Weinstock RS, TODAY Study Group. Metformin monotherapy in youth with recent onset type 2 diabetes: Experience from the prerandomization run-in phase of the TODAY study. Pediatric Diabetes 2012: Feb 27 Epub. Lara-Corrales I, Parkin PC, Stephens D, Hamilton J, Koren G, Weinstein M, Sibbald RG, Pope E. The efficacy of trimethoprim in wound healing of patients with epidermolysis bullosa: A feasibility trial. Journal of American Academy of Dermatology 2012: 66 (2): pp Lee WL, Klip A. Shuttling glucose across brain microvessels, with a little help from GLUT1 and AMP kinase: Focus on AMP kinase regulation of sugar transport in brain capillary endothelial cells during acute metabolic stress. American Journal of Physiology Cell Physiology 2012: PMID (Epub ahead of print). Luca P, Birken C, Grewal P, Dettmer E, Hamilton J. Complex obesity. Current Pediatric Reviews 2011: 8 (2): pp (Epub ahead of print). Murphy C, Allen L, Jamieson MA. Ambiguous genitalia in the newborn: An overview and teaching tool. Journal of Pediatric and Adolescent Gynecology 2011: 24 (5): pp Murphy KE, Hanna, ME, Willan AR, Ohlsson A, Kelly EN, Matthews SG, Saigal S, Asztalos E, Ross S, Delisle MF, Tomat L, Amankwah K, Guselle P, Gafni A, Lee SK, Armson BA, MACS Collaborative Group. Maternal side-effects after multiple courses of antenatal corticosteroids (MACS): The three month follow-up of women in the randomized controlled trial of MACS for preterm birth study. Journal Obstetrics and Gynaecology Canada 2011: 33 (9): pp O Gorman CS, Syme C, Bradley T, Hamilton J, Mahmud FH. Impaired endothelial function in pediatric patients with Turner syndrome and healthy controls: A case-control study. International Journal of Pediatric Endocrinology 2012: (1): pp 5. Palmert MR, Dunkel L. Delayed puberty. New England Journal of Medicine 2012: 366: (5): pp Patel BP, Bellissimo N, Thomas SG, Hamilton JK, Anderson GH. Television viewing at mealtime reduces caloric compensation in peripubertal, but not postpubertal, girls. Pediatric Resources 2011: 70 (5): pp Pulman KJ, Kives S, Jamieson MA. A case of vaginoplasty without grafting. Journal of Pediatric and Adolescent Gynecology 2012: 25 (2): pp e Rachmiel M, Rubio-Cabezas O, Ellard S, Hattersley AT, Perlman K. Early-onset, severe lipoatrophy in a patient with permanent neonatal diabetes mellitus secondary to a recessive mutation in the INS gene. Pediatric Diabetes 2011: Sept 13. doi: /j (Epub ahead of print). Retnakaran R, Ye C, Hanley AJ, Connelly PW, Sermer M, Zinman B, Hamilton JK. Effect of maternal weight, adipokines, glucose intolerance and lipids on infant birth weight among women without gestational diabetes mellitus. Canadian Medical Association Journal 2012 (Epub ahead of print). Shulman R, Palmert MR, Daneman D. Insulin pump therapy in youth with type 1 diabetes: Uptake and outcomes in the real world. Future Medicine - Diabetes Management 2012: 2(2): pp Stevens S, Major D, Rovet J, Koren G, Fantus E, Nulman I, Desrocher M. Social problem solving in children with fetal alcohol spectrum disorders. Journal of Population Therapeutics and Clinical 2012: 19: pp Teixeira SS, Tamrakar AK, Goulart-Silva F, Serrano-Nascimento C, Klip A, Nunes MT. Triiodothyronine acutely stimulates glucose transport into L6 muscle cells without increasing surface GLUT4, GLUT1, or GLUT3. Thyroid 2012: 22: pp TODAY Study Group, Zeitler P, Hirst K, Pyle L, Linder B, Copeland K, Arslanian S, Cuttler L, Nathan DM, Tollefsen S, Wilfley D, Kaufman F. A clinical trial to maintain glycemic control in youth with type 2 diabetes. New England Journal of Medicine 2012: 366(24): pp Epub 2012: Apr 29. Turkbey EB, Backlund JY, Genuth S, Jain A, Miao C, Cleary PA, Lachin JM, Nathan DM, van der Geest RJ, Soliman EZ, Liu CY, Lima JA, Bluemke DA, DCCT/EDIC Research Group. Myocardial structure, function, and scar in patients with type 1 diabetes mellitus. Circulation 2011: 124: pp Epub 2011: Sep 26. Urbach SL, Guger S, Nathan PC. Long-term survivors of cancer in childhood and adolescence. Current Pediatric Reviews 2012: 8 (2): pp Wheeler S, Stevens S, Sheard E, Rovet J. Facial memory deficits in children with fetal alcohol spectrum disorders. Child Neuropsychology 2012: 18 (4): pp Wheeler SM, McAndrews MP, Sheard E, Rovet J. Visuospatial associative memory and hippocampal functioning in congenital hypothyroidism. Journal International Neuropsychological Society 2012: 18: pp Wheeler SM, Willoughby KA, McAndrews MP, Rovet JF. Hippocampal size and memory functioning in children and adolescents with congenital hypothyroidism. Journal Clinical Endocrinology Metabolism 2011: 96: pp Willoughby KA, Desrocher M, Levine B, Rovet J. Normative development of episodic and semantic autobiographical memory across childhood and adolescence. Frontiers in Developmental Science 2012: 3:53: e. Yu J, Shi L, Wang H, Bilan PJ, Yao Z, Samaan MC, He Q, Klip A, Niu W. Conditioned medium from hypoxia-treated adipocytes renders muscle cells insulin resistant. European Journal of Cell Biology 2011: 90: pp Books and Chapters Amed S, Perlman K, Daneman D. Hypoglycaemia in people with type 1 diabetes mellitus. In: BMJ Learning. (updated April 2012) Daneman D, Friedman J, eds: The child who is medically fragile. In: Current Pediatric Review 2012: 8(2): pp Vaidya B, Negro R, Poppe K, Rovet J. Thyroid and pregnancy. In: Special issue of Journal of Thyroid Research, SAGE-Hindawi Access to Research (Vaidya B, Negro R, Poppe K, Rovet J, eds.) 2011: pp 1-3. Grants and Funding Adolescent Type 1 Diabetes Cardio-Renal Intervention Trial (AdDIT) Canadian Expansion. Mahmud FH, Daneman D, Sochett EB. Juvenile Diabetes Research Foundation (JDRF). $3,885,000 CAD. Apr to Mar Adolescent type 1 diabetes cardio-renal intervention trial (AdDIT). Daneman D. The Hospital for Sick Children. $91,020 CAD. Jul to Jun An early developmental model of overeating, obesity and executive dysfunction. Levitan R, Meany M, Davis C, Dubé L, Gruber R, Hamilton J, Kennedy J, Matthews S, Soares C, Steiner M. Canadian Institutes of Health Research $763,130 CAD. May to Apr Autonomic nervous system regulation in adolescents and children treated for craniopharyngioma. Hamilton J, McCrindle B, Syme C. Brain Tumour Research Assistance and Information Network (b.r.a.i.n.child). $20,632 CAD. Apr to Mar the hospital for sick children department of paediatrics Annual Report

31 CANadian Pediatric Weight management Registry (CANPWR). Identifying the characteristics of successful change in Canadian pediatric weight management programs. Principal Applicant: Morrison K. Co-Applicants: Hamilton J, Ball G, Berall G, Chanoine JP, Hadjiyannakis S, Legault L, Lambert M, Tremblay M, Xie C. Canadian Institutes of Health Research. $100,000 CAD. Aug to Jul CANadian Pediatric Weight management Registry (CANPWR). Identifying the characteristics of successful change in Canadian pediatric weight management programs Principal Applicant: Morrison K. Co-Applicants: Hamilton J, Ball G, Berall G, Buchholz A, Chanoine JP, Legault L, Thabane L, Tremblay M. Canadian Institutes of Health Research. $943,636 CAD. Apr to Mar Celiac Disease and Diabetes: Dietary Intervention and Evaluation Trial (CD-DIET). Mahmud FH, Marcon M. Juvenile Diabetes Research Foundation (JDRF). $3,000,000 CAD. May to Apr Deciphering the bases of sex differences in the brain and their role in psychiatric disorders. Palmert M, Lerch J, Arnold P, Foster J. Canadian Institute of Health Research. $779,590 CAD. Oct to Sep DNA methylome study in type 1 diabetes. Petronis A, Palmert MR. National Institutes of Health/National Institute of Child Health and Human Development. $2,939,808 CAD. Sep to Aug Epigenetic changes in DNA of infants exposed to gestational diabetes in utero. Palmert M, Hamilton J. Canadian Diabetes Association. $272,967 CAD. Jul to Jun Evaluation of the SickKids Team Obesity Management Program (STOMP) Hamilton J, Birken C, Dettmer E, Grewal P, Jeffery A, Knox K, Bar-Dayan A, McGuire M. Cathexis Consulting Group and Provincial Council for Maternal and Child Health. $26, CAD. May to Apr Food intake control in children. Anderson GH, Co-Applicants: Hamilton J, Luhovyy B, Thomas S. Canadian Institutes of Health Research. $660,000 CAD. Oct to Sep Genome wide DNA methylation alterations in infants exposed to gestational diabetes in utero. Principal Applicant: Hamilton J, Co-Applicants: Retnakaran R, Hanley A, Palmert M, Weksberg R. Canadian Diabetes Association. $272,973 CAD. Jul to Jun High Impact Strategies Towards Overweight Reduction in Youth (HISTORY) Team Grant. McCrindle B, Hamilton J, Parkin P, Pencharz P, Anderson G, Birken C, Wells G, Riddell M, Hanley A, O Connor D, Dettmer E, Bellissimo N, Langer J, Tein I. Canadian Institutes of Health Research. $2,322,370 CAD. Mar to Feb Impact of maternal metabolic abnormalities in pregnancy on breast milk composition and subsequent infant metabolic development. O Connor DL, Ley S, Hanley A, Hamilton J, Sermer M, Retnakaran R, Zinman B. Canadian Diabetes Association. $225,429 CAD. Jun to May Insulin resistance and beta cell dysfunction in early childhood: The role of maternal and infant metabolic risk factors. Hamilton J, Retnakaran R, Hanley A, Zinman B. Canadian Institutes of Health Research. $511,886 CAD. Oct to Sep Interplay between fatty acids and macrophages in the genesis and relief of muscle cell insulin resistance. Principal Applicant: Klip A. Canadian Diabetes Association. $255,000 CAD. Jul to Jun Investigating brain and behavioural plasticity following selfregulation training in children with fetal alcohol spectrum disorders. Rovet J, Lerch J, Taylor M, Koren G. Canadian Institutes of Health Research. $341,000 CAD. Apr to Mar Investigation of how paternal hyperglycaemia alters metabolic outcomes in offspring. Palmert M. Institute of Medical Science. $2,400 CAD. May to Jul JDRF (Juvenile Diabetes Research Foundation) and Diabetes UK (AdDIT) Study. Daneman D, Mahmud F, Curtis J, Sochett E. Juvenile Diabetes Research Foundation and Diabetes UK. $85,000 CAD. Jul to Jun Molecular convergence of insulin and muscle contraction signalling at Rab proteins: Implications for type 2 diabetes. Canadian Institutes of Health Research. China-Canada Joint Health Research Initiative. (CIHR) and National Natural Sciences Foundation of China (NSFC). Nominated Principal Applicant: Klip A. Co-Investigator: Bilan PJ (Canada). Co-Applicant: Niu W (China). $217,281 CAD. Jan to Dec PROMOTE: Preschoolers at Risk - Obesity and Cardiometabolic Risk Factors: Towards early identification. Birken CS, Adeli K, Hamilton J, Hanley A, Maguire J, McCrindle B, Parkin PC. Physicians Services Incorporated Foundation. $168,200 CAD. Apr to Mar PROMOTE: Preschoolers at Risk - Obesity trajectories and cardiometabolic factors: Towards early identification. Principal Applicant: Birken C. Co-Applicants: Adeli K, Hamilton J, Hanley A, Maguire J, Mamdani M, McCrindle B, Parkin P, Thorpe K. Canadian Institutes of Health Research. $645,963 CAD. Dec to Nov Prospective analysis of a standardized treatment and follow-up protocol for pediatric non-medullary thyroid cancer. Wasserman J, Rachmiel M, Gupta A, Hamilton J. Rare Disease Foundation. $3500 CAD. Jan to Dec Regulation of muscle glucose transporters by Rac and Rabs during insulin action and lipotoxicity. Klip A. Canadian Institutes of Health Research. $724,217 CAD. Oct to Sep SickKids Multi-Study Clinical Center. Mahmud FH, Wherrett, DW. Juvenile Diabetes Research Foundation Canadian Clinical Trial Network (CCTN). Start-Up Funding: $195,000 CAD. Core Funding $540,000 CAD. Apr to Mar Social cognitive processing in fetal alcohol spectrum disorders. Stevens S, Rovet J. Canadian Foundation for Fetal Alcohol Research. $64,000 CAD. Nov to Oct The effect of milk products on glycemic regulation, satiety and food intake in children. Anderson GH, Bellissimo N, Hamilton J, Luhovyy B. Dairy Farmers of Canada. $150,000 CAD. Dec to Nov The GLUT4 interactomes and their metabolic implications. Principal Applicant: Klip A. Co-Applicant: Foster L. Canadian Institutes of Health Research. $818,206 CAD. Oct to Sep The prevalence of albuminuria in youth with type 2 diabetes. Sellers E, Hadjiyannakis S, Hamilton J, Dean H, Amed S, Panagiotopoulos D, Dyck R, Langlois V, Dart A. Public Health Agency of Canada $40,000 CAD. Jan to Dec TIME Trial: RCT of sensor-augmented pump vs standard pump initiation. Mahmud FH, Curtis J. Juvenile Diabetes Research Foundation (JDRF). $250,000 CAD. Sep to Aug TRIGR (Trial to Reduce Insulin-dependent diabetes in the Genetically at Risk). Daneman D. National Institutes of Health. $450,000 CAD. Jul to Jun Type 1 Diabetes TrialNet. Toronto Clinical Centre. Wherrett DK. National Institutes of Health. $545, CAD. May to Apr Understanding the importance of newly identified diabetes risk factors among Aboriginal Canadians: The Sandy Lake Health and Diabetes Project. Principal Applicant: Hanley A. Co-Applicants: Hamilton J, Harris S, Rae J, Zinman B. Canadian Institutes of Health Research $100,000 CAD. Aug to Jul Use of ACASI to gather information on risk behaviours in a referral population of pregnant Canadian adolescents. Spitzer RF, Allen L. Physician Services Incorporated. $37,800 CAD. Mar to Feb the hospital for sick children department of paediatrics Annual Report

32 GASTROENTEROLOGY/HEPATOLOGY/NUTRITION overview Division Head Dr. Anne Griffiths Full-Time Dr. Yaron Avitzur Dr. Peter Durie Dr. Tanja Gonska Dr. Nicola Jones Dr. Binita Kamath Dr. Simon Ling Dr. Margaret Marcon Dr. Aleixo Muise Dr. Vicky Ng Dr. Philip Sherman Dr. Thomas Walters Dr. Stanley Zlotkin Part-Time Dr. Glenn Berall Dr. Carol Durno Dr. Paul Kortan Dr. Karen Liquornik Dr. Gary May Dr. Paul Pencharz Cross-Appointees Dr. Latifa Yeung Dr. Stephen Freedman Clinical Fellows Dr. Rammy Abu-Assi Dr. Achiya Amir Dr. Amit Assa Dr. Cynthia Popalis Research Fellows Dr. Karolina Burghardt Dr. Peter Church Dr. Abdul Elkadri Dr. Marialena Mouzaki Dr. Juan Ossa Dr. Christiane Sokollik Dr. Pamela Valentino Dr. Johan Van Limbergen Dr. Catharine Walsh Nurse Practitioners Maria D Angelis Karen Frost Kim Milbury Krista Murch Constance O Connor Ambulatory Nurses Zenaida Chinea Debra Harrison Julia Kelly Vikki Scaini Alison Stewart GI Procedure Nurses Carol Fitzmaurice Donna Stevenson Wendy Taylor Kelly Wimbush Administrative Staff Chona Callejo Tamara Forde Margaret Johnson Sherry Joy Nnenna Ngoka Lilatool Shakur Suzanne Shek The Division of Gastroenterology, Hepatology and Nutrition provides subspecialty clinical care to children and adolescents with gastrointestinal, pancreatic, hepatologic and nutritional disorders, including those who have undergone liver and/or small bowel transplantation. Clinical volumes and patient complexity have increased steadily over the past few years, with busy in-patient, ambulatory, and procedural practices. Providing clinical care to children and adolescents with gastrointestinal, pancreatic, hepatologic and nutritional disorders The past five years have seen a lot of changes in Divisional faculty related to transitions and new opportunities. Incoming faculty are highly valued by the Division and Department, as recognized this year by the receipt by relatively new faculty of two key Department of Pediatrics awards. Dr. Aleixo Muise received the 2012 Early Career Research Award and Dr. Yaron Avitzur, the 2012 Early Career Clinical Excellence Award. Specialty nursing and allied health professionals play a valuable and increasing role in patient care, including organization of patient support groups (e.g. IBD teen support group) and family education days (e.g. Claire Smith IBD day for families and Liver Transplant Family Education Day). Divisional faculty conduct research which spans the spectrum from basic and translational to patient-based in such areas as inflammatory bowel disease, exocrine pancreatic diseases, liver diseases, GI microbiology and immunology, nutritional metabolism and nutrient requirements. Faculty members collaborate in or lead large multi-centre North American and international research consortia addressing both pathogenesis and treatment of childhood liver disease, cystic fibrosis and inflammatory bowel disease. Locally, the establishment of the Northbridge Financial Corporation Chair in Inflammatory Bowel Disease recognizes the importance of IBD clinical care, education and research at SickKids and will undoubtedly enhance the activities of the IBD team. The paediatric GI/Nutrition fellowship training program has been directed by Dr. Simon Ling since early 2010, and has always been central in the Division. Divisional faculty with varied subspecialty interests unite around the common goal of providing optimal training department of paediatrics Annual Report

33 for the next generation of colleagues. The program, which usually accepts four new fellows annually, consists of one year of intensive clinical training and two or more years of training in basic or clinical research. The clinical component includes training in endoscopic procedures, in-patient management of complex patients including those awaiting and undergoing liver transplantation and those with inflammatory bowel disease; ambulatory clinics (including general GI, IBD, hepatology, transplantation, feeding disorders, and nutrition). To complement their clinical or basic science research training, fellows are encouraged to complete a post-graduate degree (MSc or PhD) at either the Faculty of Health Promotion, Management and Epidemiology (HPME) or the Institute for Medical Sciences at the University of Toronto. The emphasis placed on academic scholarly work during training is aimed at continuing to prepare fellows for careers in academic paediatric gastroenterology both in Canada and around the world. Honors and Awards Staff Avitzur, Y: Junior faculty award for clinical excellence in paediatric medical care, The Hospital for Sick Children, Toronto, June Muise, A: Junior Faculty Research Award Department of Pediatrics, University of Toronto, Jones, N: Winner of Canadian Association of Gastroenterology Visiting Research Scientist Award, Ontario, Kamath, B: Teaching and Mentorship Award, Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, Muise, A: Winner of Junior Faculty Research Award, Department of Pediatrics, University of Toronto, March Walters, T: Winner of Visiting Lecturer Runner-up, Division of Paediatric Emergency Medicine, Faculty of Medicine, University of Toronto, Trainees Church, P: Resident Research Award, Takeda Canada and the Canadian Association of Gastroenterology, Toronto, Church, P: Trainee Travel Award, The Hospital for Sick Children, Research Institute, Toronto, Church, P: Travel Grant Award, School of Graduate Studies, University of Toronto, Walsh, C: Postgraduate Medical Trainee Leadership Award, Postgraduate Medical Education, University of Toronto, Toronto, June Walsh, C: School of Graduate Studies Conference Grant. University of Toronto, Toronto, November, Publications Amir A, Shapiro R, Shulman LM, Mor E, Steinberg R, Fleishhacker H, Bergerin R, Avitzur Y. BK virus infection and its effect on renal function in pediatric liver transplant recipients: A cross sectional, longitudinal, prospective study. Transplantation 2011: 92: pp Anderson CA, Boucher G, Lees CW, Franke A, D Amato M, Taylor KD, Lee JC, Goyette P, Imielinski M, Latiano A, Lagacé C, Scott R, Amininejad L, Bumpstead S, Baidoo L, Baldassano RN, Barclay M, Bayless TM, Brand S, Büning C, Colombel JF, Denson LA, De Vos M, Dubinsky M, Edwards C, Ellinghaus D, Fehrmann RS, Floyd JA, Florin T, Franchimont D, Franke L, Georges M, Glas J, Glazer NL, Guthery SL, Haritunians T, Hayward NK, Hugot JP, Jobin G, Laukens D, Lawrance I, Lémann M, Levine A, Libioulle C, Louis E, McGovern DP, Milla M, Montgomery GW, Morley KI, Mowat C, Ng A, Newman W, Ophoff RA, Papi L, Palmieri O, Peyrin-Biroulet L, Panés J, Phillips A, Prescott NJ, Proctor DD, Roberts R, Russell R, Rutgeerts P, Sanderson J, Sans M, Schumm P, Seibold F, Sharma Y, Simms LA, Seielstad M, Steinhart AH, Targan SR, van den Berg LH, Vatn M, Verspaget H, Walters T, Wijmenga C, Wilson DC, Westra HJ, Xavier RJ, Zhao ZZ, Ponsioen CY, Andersen V, Torkvist L, Gazouli M, Anagnou NP, Karlsen TH, Kupcinskas L, Sventoraityte J, Mansfield JC, Kugathasan S, Silverberg MS, Halfvarson J, Rotter JI, Mathew CG, Griffiths AM, Gearry R, Ahmad T, Brant SR, Chamaillard M, Satsangi J, Cho JH, Schreiber S, Daly MJ, Barrett JC, Parkes M, Annese V, Hakonarson H, Radford-Smith G, Duerr RH, Vermeire S, Weersma RK, Rioux JD. Meta-analysis identifies 29 additional ulcerative colitis risk loci, increasing the number of confirmed associations to 47. Nature Genetics 2011: 43 (9): pp 919. Benchimol E, Guttman A, To T, Rabeneck L, Griffiths AM. Changes in surgical and hospitalization rates in pediatric inflammatory bowel disease in Ontario, Canada ( ). Inflammatory Bowel Diseases 2011: 17 (10): pp Benchimol E, Manuel DG, To T, Rabeneck L, Griffiths AM, Guttman A. Development and use of reporting guidelines for assessing the quality of validation studies of health administrative data. Journal of Clinical Epidemiology 2011: 64 (8): pp Bitton A, Buie D, Enns R, Feagan BG, Jones JL, Marshall JK, Whittaker S, Griffiths AM, Panaccione R on behalf of the Canadian Association of Gastroenterology Severe Ulcerative Colitis Consensus Group. Treatment of hospitalized adult patients with severe ulcerative colitis: Toronto Consensus Statements. American Journal of Gastroenterology 2012: 107 (2): pp Brode SK, Ling SC, Chapman KR. Alpha-1 antitrypsin deficiency: A commonly overlooked cause of lung disease. Canadian Medical Association Journal 2012 (Epub ahead of print). Choi P, Stephenson A, Ellis L, Martin S, Solomon M, Dupuis A, Dorfman R. Zielenski J, Ooi CY, Weiser W, Durie PR, Tullis E. Role of cystic fibrosis transmembrane conductance regulator (CFTR) in patients with chronic sinopulmonary disease. Chest 2012: March (Epub ahead of print). DeGreef E, Avitzur Y, Grant D, De-Angelis M, Ng V, Jones NL, Ngan B, Shapiro R, Steinberg R, Gana JC. Infliximab as salvage therapy in paediatric intestinal transplant with steroidand thymoglobulin-resistant late acute rejection. Journal of Pediatric Gastroenterology and Nutrition 2012: 54 (4): pp Diamond I, de Silva N, Tomlinson G, Pencharz P, Feldman B, Moore A, Ling SC, Wales P. The role of parenteral lipid in the development of advanced intestinal failure associated liver disease in neonates A multiple variable analysis. Journal of Parenteral and Enteral Nutrition 2011: 35: pp Gana JC, Turner D, Mieli-Vergani G, Davenport M, Miloh T, Avitzur Y, Yap J, Morinville V, Brill H, Ling SC. A clinical prediction rule and platelet count predict esophageal varices in children. Gastroenterology 2011: 141 (6): pp Gareau M, Ho N, Brenner D, Sousa A, LeBourhis L, Mak T, Girardin S, Philpott D, Sherman PM. Enterohemorrhagic, but not enteropathogenic, escherichia coli infection of epithelial cells disrupts signaling responses to tumour necrosis factor-alpha. Microbiology UK (formerly Journal of General Microbiology) 2011: 157 (Pt 10): pp Gonska T. Early in vivo testing to assess new therapeutic interventions in CF patients. Current Pharmaceutical Design 2012: 18 (5): pp Guttman OR, Roberts EA, Schreiber RA, Barker CC, Ng VL, The Canadian Pediatric Hepatology Research Group. Biliary atresia with associated structural malformations in Canadian children. Liver International 2011: 31 (10): pp Ho NK, Crandall I, Sherman PM. Identifying mechanisms by which escherichia coli O157:H7 subverts interferon-γ mediated signal transducer and activator of transcription-1 activation. PLoS One 2012: 7 (1): pp e Ho NK, Ossa JC, Silphaduang U, Johnson R, Johnson-Henry KC, Sherman PM. Enterohemorrhagic escherichia coli O157: H7 shiga toxins inhibit gamma interferon-mediated cellular activation. Infection and Immunity 2012: 80 (7): pp Hyams JS, Damaraju L, Blank M, Johanns J, Guzzo C,Winter HS, Kugathasan S, Cohen S, Markowitz J, Veereman G, Crandall W, Baldassano R, Griffiths AM. Induction and maintenance therapy with Infliximab for children with moderate to severe ulcerative colitis. Clinical Gastroenterology and Hepatology 2012: 10 (4): pp Hyams JS, Griffiths AM, Markowitz J, Baldassano RN, Faubion WA Jr, Colletti RB, Dubinsky M, Kierkus J, Wang J, Huang B, Bittle B, Marshall M, Lazar A. Safety and efficacy of adalimumab for moderate to severe Crohn s disease in children. Gastroenterology 2012:143 (2): pp Jones NL on behalf of Canadian Helicobacter study group. Helicobacter pylori in First Nation and recent immigrant populations in Canada. Canadian Journal of Gastroenterology 2012: 26 (2): pp Jones NL, Chiba N, Fallone C, Hunt R, Thomson A. Helicobacter pylori and immigrant health. Canadian Medical Association Journal 2012: 184 (1): pp the hospital for sick children department of paediatrics Annual Report

34 Jones NL, Croitoru K, Vanner S, Soni R, Hope L. Research topics in gastrointestinal disease: A report on the 10th meeting and an announcement of the 11th meeting. Canadian Journal of Gastroenterology 2011: 25 (8): pp Jones NL. Helicobacter pylori treatment in the hospital setting: A potential model for developing quality improvement initiatives to prevent missed test results. Canadian Journal of Gastroenterology 2011: 25 (10): pp 542. Kamath BM, Bauer RC, Loomes KM, Chao G, Gerfen J, Hutchinson A, Hardikar W, Hirschfield G, Jara P, Krantz ID, Lapunzina P, Leonard L, Ling S, Ng VL, Hoang PL, Piccoli DA, Spinner NB. NOTCH2 mutations in Alagille syndrome. Journal of Medical Genetics 2012: 49 (2): pp Kamath BM, Podkameni G, Hutchinson AL, Leonard LD, Gerfen J, Krantz ID, Piccoli DA, Spinner NB, Loomes KM, Meyers K. Renal anomalies in Alagille syndrome. American Journal of Medical Genetics 2011: Nov (Epub ahead of print). Kamath BM, Yin W, Miller H, Anand R, Rand EB, Alonso E, Bucuvalas J and the SPLIT Group. Outcome of Alagille syndrome after liver transplantation: The SPLIT experience. Liver Transplantation 2012: March (Epub ahead of print). Koletzko S, Jones NL, Goodman K, Gold B, Rowland M, Cadranel S, Chong S, Colletti RB, Casswall T, Elitsur Y, Guarner J, Kalach N, Madrazo A, Megraud F, Oderda G on behalf of the H. Pylori Working Groups of ESPGHAN and NASPGHAN. Evidence-based guidelines from ESPGHAN and NASPGHAN for Helicobacter pylori infection in children. Journal of Pediatric Gastroenterology and Nutrition 2011: 53 (2): pp Kumagi T, Drenth JPH, Guttman O, Ng V, Lilly L, Therapondas G, Hiasa Y, Michitaka K, Onji M, Watanabe Y, Sen S, Griffiths W, Roberts E, Heathcote EJ, Hirschfield GM. Biliary atresia and survival into adulthood without transplantation: A collaborative multi-centre clinic review. Liver International 2012: 32 (3): pp Lam GY, Fattouh R, Muise AM, Grinstein S, Higgins DE, Brumell JH. Listeriolysin O suppresses phospholipase C-mediated activation of the microbiocidal NADPH oxidase to promote Listeria monocytogenes infection. Cell Host Microbes 2011:10 (6): pp Levin D, Marcon MA, Rintala RJ, Jacobson D, Langer JC. Inflammatory bowel disease manifesting after surgical treatment for Hirschsprung disease. Journal of Pediatric Gastroenterology and Nutrition 2012: Feb 4 (Epub ahead of print). Lin HC, Hoang L, Hutchinson A, Chao G, Gerfen J, Loomes KM, Krantz I, Kamath BM, Spinner NB. Alagille syndrome in a Vietnamese cohort: Mutation analysis and assessment of facial features. American Journal of Medical Genetics 2012: 158A (5): pp Michail S, Durbin M, Turner D, Griffiths AM, Mack Dr. Hyams J, Leleiko N, Kenche H, Stolfi A, Wine E. Alterations in the gut microbiome of children with severe ulcerative colitis. Inflammatory Bowel Diseases Online 2011: doi: / ibd Moran CJ, Walters TD, Guo CH, Kugathasan S, Klein C, Turner D, Wolters VM, Bandsma RH, Mouzaki M, Zachos M, Langer JC, Cutz E, Benseler SM, Roifman CM, Silverberg MS, Griffiths AM, Snapper S, Muise AM. IL-10R polymorphisms are associated with very early-onset ulcerative colitis. Inflammatory Bowel Diseases 2012: May 3 (Epub ahead of print). Muise A, Snapper SB, Kugathasan S. The age of gene discovery in very early onset inflammatory bowel disease. Gastroenterology 2012: June (Epub ahead of print). Muise A, Walters T, Xu W, Shen-Tu G, Guo CH, Fattouh R, Lam G, Wolters V, Bennitz J, Van Limbergen J, Renbaum P, Kasirer Y, Ngan BY, Turner D, Denson L, Sherman PM, Duerr R, Cho J, Lees C, Satsangi J, Wilson D, Paterson A, Griffiths A, Glogauer M, Silverberg M, Brumell J. Single nucleotide polymorphisms that increase expression of the gaunosine triphosphatase RAC1 are associated with colonic inflammatory bowel disease. Gastroenterology 2011: 141 (2): pp Muise AM, Fattouh R, Lam GY, Dhillon S, Brummel JH. The author s reply. Gut 2012: 61 (7): pp Muise AM, Xu W, Guo CH, Walters TD, Wolters VM, Fattouh R, Lam GY, Murchie R, Sherlock M, Gana JC, Russell RK, Glogauer M, Duerr RH, Cho JH, Lees CW, Satsangi J, Wilson DC, Paterson AD, Griffiths AM, Silverberg MS, Brumell JH. NADPH oxidase complex and IBD candidate gene studies: Identification of a rare variant in NCF2 that results in reduced binding to RAC2. Gut 2012: 61 (7): pp Ng VL, Alonso E, Bucuvalas J, Cohen G, Limbers C, Varni J, Blonski D, Mazariegos G, Magee J, McDiarmid S, Anand R for the Studies of Pediatric Liver Transplantation (SPLIT) Research Group. Health status of children alive 10 years after pediatric liver transplantation performed in the US and Canada: Report of the SPLIT experience. Journal of Pediatrics 2012: 160 (5): pp Nightingale S, McEwan-Jackson FD, Hawker GA, Macarthur C, Khambalia AZ, Fecteau A, Ng VL. Corticosteroid exposure not associated with long-term bone mineral density in pediatric liver transplantation. Journal of Pediatric Gastroenterology and Nutrition 2011: 53 (3): pp O Connor PM, Lapointe TK, Jackson S, Beck P, Jones NL, Buret AG. Helicobacter pylori activates calpain via toll-like receptor 2 to disrupt adherens junctions in human gastric epithelial cells. Infection and Immunity 2011: 79 (10): pp Ooi CY, Dupuis A, Ellis L, Jarvi K, Martin S. Gonska T, Dorfman R, Kortan P, Solomon M, Tullis E. Durie PR. Comparing the American and European diagnostic guidelines for cystic fibrosis: Same disease, different language? Thorax 2012: 67 (1): pp Otley A, LeLeiko N, Langton C, Lerer T, Mack D, Evans J, Michail S, Pfefferkorn M, Carvalho R, Rosh J, Griffiths A, Oliva-Hemker M, Kay M, Bousvaros A, Stephens M, Samson C, Grossman A, Keljo D, Markowitz J, Hyams J. Budesonide use in pediatric Crohn s disease patients. Journal of Pediatric Gastroenterology and Nutrition 2012: 55 (2): pp Raju D, Hussey S, Ang M, Terebiznik MR, Gupta V, Blanke SR, Delgado A, Romero-Gallo J, Ramjeet M, Mascarenhas H, Peek RM, Correa P, Streutker C, Hold G, Kunstmann E, Yoshimori T, Silverberg M, Philpott DJ, El Omar E, Jones NL. Vacuolating cytotoxin and variants in Atg16L1 that disrupt autophagy promote Helicobacter pylori infection in humans. Gastroenterology 2012: 142 (5): pp Rivas MA, Beaudoin M, Gardet A, Stevens C, Sharma Y, Zhang CK, Boucher G, Ripke S, Ellinghaus D, Burtt N, Fennell T, Kirby A, Latiano A, Goyette P, Green T, Halfvarson J, Haritunians T, Korn JM, Kuruvilla F, Lagacé C, Neale B, Lo KS, Schumm P, Törkvist L, National Institute of Diabetes and Digestive Kidney Diseases Inflammatory Bowel Disease Genetics Consortium (NIDDK IBDGC), United Kingdom Inflammatory Bowel Disease Genetics Consortium, International Inflammatory Bowel Disease Genetics Consortium, Dubinsky MC, Brant SR, Silverberg MS, Duerr RH, Altshuler D, Gabriel S, Lettre G, Franke A, D Amato M, McGovern DP, Cho JH, Rioux JD, Xavier RJ, Daly MJ. Deep resequencing of GWAS loci identifies independent rare variants associated with inflammatory bowel disease. Nature Genetic 2011: 43 (11): pp Rodrigues D, Sousa A, Johnson-Henry K, Sherman PM, Gareau MG. Probiotics are effective for the prevention and treatment of Citrobacter rodentium-induced colitis in mice. Journal of Infectious Diseases 2012: 206 (1): pp Rodrigues D, Sousa A, Vong L, Hawley S, Gareau M, Kumar S, Johnson-Henry K, Sherman PM. Matrix metalloproteinase-9 contributes to gut microbe homeostasis in a model of infectious colitis. BMC Microbiology 2012: 12 (1): pp 105. Schechter T, Gassas A, Weitzman S, Grant D, Pollock-BarZiv S, Dipchand A, Alexander S, Ali M, Avitzur Y, Doyle J. Hematopoietic stem cell transplantation following solid organ transplantation in children. Bone Marrow Transplantation 2011: 46: pp Sherlock M, Griffiths AM. Medical therapy for pediatric inflammatory bowel disease. Current Gastroenterology Reports 2012: 14 (2): pp Sherlock ME, Bandsma R, Ota K, Kirby-Allen M, Griffiths AM. Severe neutropenia following infliximab treatment in a child with ulcerative colitis. Inflammatory Bowel Diseases 2011: 17 (2): pp E the hospital for sick children department of paediatrics Annual Report

35 Sherman PM, Brown S, Rose K, Murphy T, Makarchuk MJ, Paterson W, Belanger P. Workshop report: Developing a pediatric inflammatory bowel diseases network and data platform for Canada. Journal of Pediatric Gastroenterology and Nutrition 2012: May 27 (Epub ahead of print). Sherman PM, Makarchuk MJ, Belanger P, Roberts E. Strategic plan of the CIHR Institute of Nutrition, Metabolism, and Diabetes. Canadian Journal of Gastroenterology 2011: 25 (10): pp Shneider BL, Bosch J, De Franchis R, Emre SH, Groszmann RJ, Ling SC, Lorenz JM, Squires RH Jr, Superina RA, Thompson AE, Mazariegos GV. Portal hypertension in children: Expert pediatric opinion on the report of the Baveno V Consensus Workshop on methodology of diagnosis and therapy in portal hypertension. Pediatric Transplantation 2012: 16 (5): pp Sibony M, Jones NL. Recent advances in Helicobacter pylori pathogenesis. Current Opinion in Gastroenterology 2012: 28 (1): pp Sokollik C, Ang M, Jones NL. Autophagy: A primer for the gastroenterologist/hepatologist. Canadian Journal of Gastroenterology 2011: 25 (12): pp Sokollik C, Chan KJ, Sherman PM. Ingested magnets Catch or let go? Gastroenterology 2012: 142 (4): pp 701, Sud S, Marcon M, Assor E, Daneman D, Mahmud FH. Health professional attitudes regarding celiac disease screening and treatment in pediatric type 1 diabetes. Canadian Journal of Diabetes 2011: 35 (4): pp Sud S, Marcon M, Assor E, Daneman D, Mahmud FH. Quality of life in children with diabetes and celiac disease: Minimal impact of the double diagnosis. Pediatric Diabetes 2012: 13 (2): pp Traubici J, Somers GR, Ling SC, Pearl RJ, Nathan PC. Pleuropulmonary blastoma in a child with autosomal-recessive polycystic kidney disease. Pediatric Radiology 2011: 41: pp Turner D, Griffiths AM, Walters TD, Seah T, Markowitz J, Pfefferkorn M, Keljo D, Waxman J, Otley A, Leleiko NS, Mack D, Hyams J, Levine A. Mathematical weighting of the pediatric Crohn s disease activity index (PCDAI) and comparison with its other short versions. Inflammatory Bowel Disease 2012: 18 (1): pp Turner D, Mack Dr. Hyams J, Leleiko N, Otley A, Markowitz J, Kasirer Y, Muise A, Seow CH, Silverberg MS, Crandall W, Griffiths AM. C-reactive protein (CRP), erythrocyte sedimentation rate (ESR) or both? A systematic evaluation in pediatric ulcerative colitis. Journal of Crohn s and Colitis 2011: 5 (5): pp Van Limbergen J, Philpott D, Griffiths AM. Genetic profiling in inflammatory bowel disease: From association to bedside. Gastroenterology 2011: 141 (5): pp Vong L, Ferraz J, Dufton N, Panaccione R, Beck P, Sherman PM, Perretti M, Wallace J. Up-regulation of annexin-1 and lipoxin A4 in individuals with ulcerative colitis may promote mucosal homeostasis. PLoS One 2012: 7 (6): pp e Walsh C, Rose D, Dubrowski A, Ling S, Grierson L, Backstein D, Carnahan H. Learning in the simulated setting: A comparison of expert-, peer-, and computer-assisted learning. Academic Medicine 2011: 86: pp S12-S16. Walsh CM, Sherlock ME, Ling SC, Carnahan H. Virtual reality simulation training for health professions trainees in gastrointestinal endoscopy. Cochrane Database of Systematic Reviews 2012: 6. Article No.: CD DOI: / CD pub2. Walters TD, Steinhart AH, Bernstein CN, Tremaine W, McKenzie M, Wolff BG, McLeod RS. Validating Crohn s disease activity indices for use in assessing postoperative recurrence. Inflammatory Bowel Diseases 2011: 17 (7): pp Books and Chapters Mouzaki M, Kamath BM. Alagille syndrome. In: Concise Pediatric and Adolescent Hepatology (Branski D, Kiess W, eds.). Karger, Basel Switzerland 2012: pp Sherlock M, Griffiths A. Enteral nutrition: Impact on disease activity and growth in pediatric IBD, advanced therapy of inflammatory bowel disease. In: IBD and Crohn s disease, Third edition. Volume II. (Bayless TM, Hanauer SB, eds.). People s Medical Publishing House, Shelton Connecticut, USA 2011: pp Sokollik C, Gupta A, Ling SC. Hepatocellular carcinoma in children. In: Primary Liver Cancer: Surveillance, Diagnosis and Treatment (Reau N, Poordad F, eds.) Springer Science, New York 2011: pp Grants and Funding A comparative study of the antiviral efficacy and safety of entecavir (ETV) versus placebo in pediatric subjects with chronic hepatitis B virus (HBV) infection who are HBeAg-positive. Ling SC. Bristol-Myers Squibb. Patient-based stipend. $20,000 CAD/ year to 2015 A cross-sectional study of the health-related quality of life and neurocognitive status of survivors of pediatric liver transplantation (2009 ARRA Award). Squires R, Alonso E, Ng V. 3 U01 DK S2. $14,000 US. Jul to Jun A model to predict outcome of liver disease in Alagille syndrome. Kamath B. American Liver Foundation. $50,000 USD/year. Jan to Dec A multi-center group to study acute liver failure in children (PALF-II). Ng V, Fecteau A, Kamath B. U01 DK $270,852 USD. Sept to Aug A multi-center group to study acute liver failure in children (PALF). Squire R, Ng V, Kamath B. National Institute of Health/ National Institute of Diabetes and Digestive and Kidney Diseases. $15,000,000 CAD. Sept to Aug A multi-disciplinary human study on the genetic, environmental and microbial interactions that cause IBD. Croitoru K, Griffiths AM, Ropelski M, Moyeddi P. Crohn s and Colitis Foundation of Canada. $5,600,000 CAD to 2012 A pharmacogenetic trial of tacrolimus after pediatric transplantation. Mital S, Kamath B. Transplant Center Catalyst Grant. $150,000 CAD. Dec to Dec A pilot study to assess the role of frailty among candidates for liver transplantation: Moving beyond PELD. Kamath B, Ng V, Cormier K, Patterson C, So S. The Hospital for Sick Children, Pediatric Consultants Creative Professional Activity (CPA). $5,825 CAD to 2012 A randomized controlled trial to evaluate the efficacy and safety of a gluten-free diet in patients with asymptomatic celiac disease and type 1 diabetes, celiac disease and diabetes - Dietary intervention and evaluation trial (CD-DIET). Mahmud F, Assor E, Cutz E, Marcon M. Juvenile Diabetes Research Foundation. $3,000,000 CAD. May to Sep A randomized, placebo-controlled trial of omega -3 fatty acids in the treatment of young children with autism. Anagnostou E, Brian J, Roberts W, Dupuis A, Marcon M. Alva Foundation. $150,000 CAD. Jan to Dec Adherent and invading Escherichia coli engagement of CEA-CAM6: Defining the link to Crohn s disease. Gray-Owen S, Sherman PM. Crohn s and Colitis Foundation of Canada. $375,000 CAD. Jul to Jun Assessment of school readiness and health-related quality of life (HRQOL) in children transplanted under the age of 2 years at The Hospital for Sick Children. Ng V, Rogers A, Gold A, Kamath B, SickKids Transplant Center. Ashley s Angels Foundation Pilot Grant. $13,553 CAD to 2013 Autism Treatment Network Registry. Loh A, Roberts W, Anagnostou E, Marcon M, Weiss S, Brian J, Feigenbaum A, Schulze A, Logan W, Chitiyat D, Weksberg R, Green P, Weiss J, Mitchell S, Berall G, Bradley E, Freeman N, Stinson J, Ornstein M, Atkinson A, Goldfarb C, Flanagan J, Superina D, Jiminez E, Jones Stokreef N, Orsino A, Isaacs B, Handley Derry M. Autism Speaks. $420,000 CAD. May 2011 to June 2013 Canadian Institutes of Health Research Canadian Child Health Clinician Scientist Training Program. Jones NL, Stevens B, Han VKM, Lee SK, Chemtob S. Canadian Institutes of Health Research. $325,000 CAD/year. Apr to Mar Cell signaling in mucosal inflammation and pain. McCullouch C, Sherman PM. Canadian Institutes for Health Research. $325,000 CAD/year. Jan to Dec Certolizumab in pediatric Crohn s disease: A multicenter study. Griffiths AM. UCB Pharma. Per patient remuneration: Anticipated $60,000 CAD to the hospital for sick children department of paediatrics Annual Report

36 Childhood Liver Disease and Research Network (ChiLDREN) Consortium. Sokol R, Ng V, Kamath B. National Institute of Health/National Institute of Diabetes and Digestive and Kidney Diseases. $146,076 CAD/year. Oct to May Constipation and toilet training in autism. Marcon M, Green P, Barreira L, Moroz L, Brian J. Autism Intervention Network for Physical Health (AIR-P Network). $39,783 USD. Jan to Dec Delineating the role of autophagy in ischemia reperfusion injury. Jones NL. Ashley s Angels Grant, The Hospital for Sick Children Transplant Centre. $15,000 CAD. Apr to Mar Determinants of the microbiome in IBD by the Harvard Broad Institute Toronto IBD Consortium. Xavier L, Silverberg M, Muise AM, Croitoru S, Bousvaros K. Crohn s and Colitis Foundation of America Team Grant. $1,000,000 CAD. Jan to Dec Development and establishment of a novel microfluidic-optic method for sweat testing. Gonska T, Li D. SickKids Foundation, CF Centre Catalyst Research Grant. $50,000 CAD. Apr to Mar Development of a prognostic score of severe liver disease in children with intestinal failure. Avitzur Y, Burghardt K. Transplant Centre Research Fellowship Grant, The Hospital for Sick Children. $70,000 CAD to 2012 Emerging team grant in cystic fibrosis therapy team grant. Ratjen F, Bear C, Ellis J, Rotin D, Durie P, Grasemann H, Hu J, Rossant J, Viirre R, Gonska T. Canadian Institutes of Health Research $2,401,449 CAD. Sep to Aug Epithelial cell signaling responses to attaching-effacing intestinal pathogens. Sherman PM. Canadian Institutes of Health Research. $777,000 CAD. Sep to Aug Evaluation of the pharmacokinetics, safety, tolerability and efficacy of entecavir (ETV) in pediatric subjects with chronic hepatitis B virus (HBV) infection who are HBeAg-positive. Ling SC. Bristol-Myers Squibb. Patient-based stipend $4,000 CAD/year to 2013 Funding of an autism intervention research network for physical health (AIR-P). Roberts SW, Loh A, Feigenbaum A, Weiss S, Marcon M, Goldfarb C, Berall G, Bradley E, Brian J, Freeman N, Atkinson A, Taylor M, Stinson J, Mitchell S, Green P. Health Resources and Services Administration. $400,882 CAD. Jan to Dec Gastric flora in gastrostomy fed children with neurologic impairment on acid suppression medication. Rapoport A, Avitzur Y. Creative Professional Activity Grant, Department of Pediatrics, The Hospital for Sick Children. $10,000 CAD Genetic and functional studies in very early onset-ibd (NEOPICS). Muise AM. Canadian Institutes of Health Research Operating Grant (MOP ). $866,005 CAD. Jan to Mar Hepatitis B Research Network: Entecavir and interferon-α2a combination therapy for children immune tolerant to chronic hepatitis B infection. Ling SC, Heathcote J. National Institutes for Health. $278,000 USD /year to 2015 Immune regulation and biomarker development in pediatric and adult onset human autoimmune diseases. Bar-Or A, Sherman PM, Banwell B, Piccirillo CA, Polychronakos C. Canadian Institutes of Health Research Team Grant. $2,018,445 CAD. Sep to Aug Immuno-genetic determinants of linear growth in paediatric IBD. Denson LA, Walters TD, Griffiths AM. Crohn s and Colitis Foundation of America Paediatric Bone and Skeletal Health Research Award. $100,000 USD/year. Jul to Jun Immunosuppression Withdrawal after Pediatric Liver Transplantation (iwith). Feng S, Ng V, Fecteau A, Avitzur Y, Jones N, Kamath B. National Institutes of Health U34 DK $5,300 CAD to 2012 ips stem cell models of Alagille syndrome. Kamath B. Rare Disease Foundation Microgrant. $3500 CAD. Dec to Dec North American IBD Genetics Consortium. Cho J, Duerr R, Silverberg M, Griffiths AM, Rotter J, Brandt S, Rioux J. National Institutes of Health. $3,000,000 CAD to 2011 Pediatric longitudinal study of elemental diet and stool microbiome composition. Wu G, Griffiths AM. National Institutes of Health. $40,000 CAD to 2012 Predicting response to standardized paediatric colitis therapy: The PROTECT Study. Hyams J, Denson L, Kugathasan S, Sylvester F, Gupta N, Walters TD. National Institutes of Health (NIDDK) U34 Multi-centre Clinical Study Implementation Planning Grant. $250,000 USD. Sep to Apr Predicting response to standardized paediatric colitis therapy: the PROTECT Study. Hyams J, Denson L, Kugathasan S, Sylvester F, Gupta N, Walters TD. National Institutes of Health (NIDDK) U01 Multi-centre Clinical Study. $1,500,000 USD. May to Apr Prediction by ultrasound of the risk of hepatic cirrhosis in cystic fibrosis ( PUSH ). Ling SC, Durie P, Navarro O. Cystic Fibrosis Foundation and National Institutes of Health $73,000 USD/year to 2013 Research topics in GI disease. Jones NL. Canadian Association of Gastroenterology. $25,000 CAD. Jan to Dec Risk stratification and identification of immunogenetic and microbial markers of CD. Kugathasan S, PRO-KIIDS Consortium, Walters TD. Crohn s and Colitis Foundation of America Special Purpose Grant. ~$5,000,000 USD. Jul to Jun Risk stratification in newly diagnosed children with Crohn disease. Kugathasan S, Griffiths AM. Crohn s and Colitis Foundation of America. $225,000 USD to 2013 Role of ATG16L1 in Crohn disease. Jones NL. Crohn s and Colitis Foundation of Canada. $125,000 CAD /year. Jan to Dec Role of autophagy during H. pylori infection and disease. Jones NL. Canadian Institute of Health Research. $103,575 CAD /year. Jan to Mar Start-up Operating Fund. Gonska T. Research Institute and Department of Pediatrics, Hospital for Sick Children. $90,000 CAD. Aug to Mar Sweat rate sub study as part of the G551D Observational Study (GOAL). Gonska T. Cystic Fibrosis Foundation Therapeutics Inc./Seattle Children s Research Institute. $16,016 USD. Sep to Aug The gut microbiota in pediatric inflammatory bowel disease. Stintzi A, Mack D, Muise AM, Griffiths A, Walters T, Figelys, H. Canadian Institute of Health Research Operating Grant (MOP245176) $720,000 CAD. Oct to Sep The gut microbiota in pediatric inflammatory bowel disease. Stinzki A. Canadian Institutes of Health Research. $432,000 CAD to 2014 The gut microbiota in pediatric inflammatory bowel diseases. Stintzi A, Mack D, Figeys D, Griffiths AM, Hajibabaei M, Muise A, Walters TD. Canadian Institutes of Health Research Operating Grant. $150,000 CAD/year. Sep to Aug The role of Crohn s disease genes in intestinal failure and allograft rejection. Muise A, Avitzur Y. Transplant Center Grant $12,500 CAD to 2011 The role of NADPH oxidases in Crohn s disease. Brumell J, Muise AM. Canadian Institutes of Health Research Operating Grant (MOP97756). $771,358 CAD. Oct to Sept Tier 1 (renewal) Canada Research Chair. Sherman PM. Canada Research Chairs. $1,400,000 CAD. Jul to Jun Translational studies of the liver disease in autosomal recessive polycystic kidney disease. Rosenblum N, Kamath B. Liam s Light Foundation. $95,000 CAD/year. Dec to Dec Understanding the genes involved in inflammatory bowel disease. Muise AM. Ontario Ministry of Research and Innovation (ER ) - Early Researcher Award. $140,000 CAD. Mar to Dec the hospital for sick children department of paediatrics Annual Report

37 HAEMATOLOGY/ONCOLOGY overview Division Head Dr. James Whitlock Full-Time Faculty Dr. Oussama Abla Dr. Sarah Alexander Dr. Ute Bartels Dr. Sylvain Baruchel Dr. Victor Blanchette Dr. Eric Bouffet Dr. Leonardo Brandão Dr. Michaela Cada Dr. Manuel Carcao Dr. Helen Chan Dr. John Doyle Dr. Yigal Dror Dr. Maarten Egeler Dr. Adam Gassas Dr. Ron Grant Dr. Abha Gupta Dr. Johann Hitzler Dr. Annie Huang Dr. Meredith Irwin Dr. Walter Kahr Dr. Melanie Kirby Dr. David Malkin Dr. Ahmed Naqvi Dr. Paul Nathan Dr. Isaac Odame Dr. Angela Punnett Dr. Tal Schechter- Finkelstein Dr. Lillian Sung Dr. Uri Tabori Dr. Sheila Weitzman Dr. Suzan Williams Cross-Appointed/ Part-Time Dr. Stephen Comay Dr. Lee Ann Gallant Dr. Mark Greenberg Dr. David Hodgson Dr. Nicole Inch Dr. Normand Laperriere Dr. Barbara-Ann Millar Dr. Aubrey Maze Dr. Jill Solomon Research Fellows and Associates Dr. Muhammad Ali Dr. Kaleem Ashraf Dr. Muhammad Asim Dr. Amy Lee Chong Clinical Fellows Dr. Lesleigh Abbott Dr. Nawaf Al Anazi Dr. Ehssan Al-Faraj Dr. Nawaf AlKhayat Dr. Tahani Ali Dr. Jillian Baker Dr. Sylvia Cheng Dr. Avram Denburg Dr. Najwa Fayea Dr. Ashraf Fouda Dr. Moira Garraus Dr. Alisha Kassam Dr. Joerg Krueger Dr. Stephan Leucht Dr. Meera Rayar Dr. Michelle Reece-Mills Dr. Aisyah Rivai Dr. Thai Hoa Tran Dr. Ewurabena Simpson Dr. Anita Villani Dr. Stacey Marjerrison Dr. Kevin Weingarten Dr. Marta Wilejto Dr. Alexandra Zorzi Subspecialty Residents Dr. Brijesh Arora Dr. Laura Avila Dr. Huda El Faridi Dr. Satyendra Katewa Dr. Riten Kumar Dr. Eva Lee Dr. Mira Liebman Dr. Arif Manji Dr. Monia Marzouki Dr. Hasan Masmali Dr. Kamon Phuakpet Dr. Mattia Rizzi Dr. Christina Schindera Dr. Bibishahin Shamsian Research Fellows Dr. Doua Bakry Dr. Sumit Gupta Dr. Laura Rodriguez Dr. Furqan Shaikh Dr. Brent Williams Dr. Peggy Wong Dr. Antonella Zanette Dr. Nataliya Zhukova Administrative Staff Rhonda Adams Bibi Ali Ingrid Argiropoulos Barbara Black Michelle Bozzo Enda Brennan Sandra Carbone-Walker Denise Ferreira Connie Grillo Tiffany Jiminez Shanaz Karim Philippa McCaffrey Kathleen Meliton Irine Polyzogopoulos Vanessa Stephen Arlene Zaldivar The Division of Haematology/Oncology provides diagnostic services and specialist care for children < 18 years of age with cancer (paediatric oncology) and non-malignant blood disorders (paediatric hematology). The Division is the largest of its kind in Canada and among the largest such programs worldwide, with approximately 327 new cancer cases registered in the paediatric oncology program this year. The Division is the designated centre for haematopoietic stem cell transplantation for patients <18 years of age in the Province of Ontario; the majority of the approximate 105 transplants performed this year were for children with cancer, with others performed for children with benign hematologic disorders, severe immunodeficiency disorders or genetic diseases. The number of in-patient days for paediatric haematology/oncology/bmt cases this year was approximately 14,500, a slight decline due to the transition of some inpatient services to the day hospital setting, and the number of ambulatory care visits was stable at 22,500. In contrast, day hospital visits were significantly increased at 11,750. The largest Royal College of Physicians and Surgeons of Canada accredited training program for the specialty paediatric haematology/oncology in Canada. Reflecting the size and diversity of its clinical services, the Division is organized into six sections and three programs: the leukemia/ lymphoma section (Section Head, Dr. Johann Hitzler); the solid tumor section (interim Section Head, Dr. Meredith Irwin); the neuro-oncology (brain tumor) section (Section Head, Dr. Eric Bouffet); the blood and marrow transplantation section (Section Head, Dr. Maarten Egeler); the hematology section (Section Head, Dr. Yigal Dror); the long-term follow-up (Aftercare) program (Program Head, Dr. Paul Nathan); the new agents and innovative therapies program (Program Head, Dr. Sylvain Baruchel); and the cancer genetics program (Program Head, Dr. David Malkin). Dr. Sarah Alexander serves as the program s Clinical Director. The haematology section has large clinical and research programs in paediatric thrombosis and hemostasis, the paediatric hemoglobinopathies and the inherited and acquired paediatric marrow failure syndromes and myelodysplasias. The Division is an active member of the Children s Oncology Group (COG), the largest paediatric department of paediatrics Annual Report

38 cancer clinical trials group in North America (SickKids Principal Investigator, Dr. Ron Grant), and is committed to the improvement in outcomes and quality of life in children with cancer through participation in prospective clinical trials. The Division is a member of the COG Phase I Consortium (Principal Investigator, Dr. Sylvain Baruchel), and is an active participant in activities of the Societe Internationale d Oncologie Pediatrique (SIOP), the Histiocyte Society, New Approaches to Neuroblastoma Therapy (NANT), and the Therapeutic Advances in Childhood Leukemia (TACL). The Division, in collaboration with the Pediatric Oncology Group of Ontario (POGO), supports a network of pediatric oncology satellite centres in the Greater Toronto and surrounding areas. Satellite centres in sickle cell disease are established in Rouge Valley Hospital and the William Osler Health Centre. Major achievements in the past year included the appointment of Dr. Maarten Egeler as the second Garron Family Chair in Childhood Cancer Research and Director of the Stem Cell Transplantation program, and approval for construction and operation of an MIBG facility, offering an important new treatment modality for children with neuroblastoma, funded through a generous gift from the Garron family. The Division is the largest Royal College of Physicians and Surgeons of Canada accredited training program for the specialty paediatric haematology/oncology in Canada. The Division s Training Program Director is Dr. Michaela Cada. The Education Program in the Division also includes a very large international training program in paediatric hematology/oncology and offers subspecialty training opportunities in areas such as the paediatric leukemias/lymphomas, neuro-oncology, cancer genetics, blood and marrow transplantation and paediatric thrombosis and hemostasis. Research in the Division spans basic science to clinical care with a special focus on translational research. Approximately one-half of the Division s 30 full-time faculty have appointments as clinician scientists or clinician investigators in the Department of Paediatrics with parallel appointments in the Research Institute. Research in the Division is multidisciplinary, involving health-care professionals with expertise in a variety of areas (e.g. nursing, psychology, social work, pharmacy). The Division is committed to facilitating knowledge exchange through international partnerships with a particular focus on countries lacking the health-care resources available in Canada. Two such initiatives are currently underway in collaboration with SickKids International: the Global Sickle Cell Disease Network, under the leadership of Dr. Isaac Odame, and the Caribbean Collaboration in Paediatric Cancer and Blood Disorders, under the leadership of Dr. Victor Blanchette. The administrative team for the Division comprises Dr. Jim Whitlock (Division Head), Judy van Clieaf (Executive Director, Child Health Services) and Denise Ferreira (Supervisor, Business Operations). Honors and Awards Alexander, S: Alvin Zipursky Teaching Award, The Hospital for Sick Children, Blanchette, V: Distinguished Career Award, American Society of Pediatric Hematology/Oncology, Bouffet, E: Garron Family Chair in Childhood Cancer Research, The Hospital for Sick Children, Chan, H: Queen Elizabeth II Diamond Jubilee Medal, Government of Canada, Toronto, Egeler, M: Dutch Childhood Oncology Group Award, Utrecht, Egeler, M: Garron Family Chair in Childhood Cancer Research, Hospital for Sick Children, Gupta, A: Winner of Academic Half Day Teaching Award, Haematology/Oncology, Department of Paediatrics, The Hospital for Sick Children, Toronto, Gupta, A: Runner up for the Subspecialty Teaching Award, The Hospital for Sick Children, Toronto, Hitzler, J: Winner of the Division of Paediatric Haematology/ Oncology Academic Half Day Teaching Award, The Hospital for Sick Children, Toronto, Malkin, D: Schwarz-Reisman Visiting Scholar Award, Tel Aviv University, Malkin, D: POGO Chair in Childhood Cancer Control, University of Toronto, Malkin, D: Best Doctors America, Odame, I: Ghanaian-Canadian Award for Professional Excellence, Sung, L: Frank Oski Lectureship Award. American Society of Pediatric Hematology Oncology, New Orleans, Whitlock, J: Listed in America s Top Doctors for Cancer, Publications Abla O, Palmert MR. Reversal of LCH-related diabetes insipidus and reappearance of posterior pituitary bright spot with low-dose chemotherapy. Pediatric Blood and Cancer 2012: 59(1): pp Aledort L, Bullinger M, von Mackensen S, Wasserman J, Young NL, Globe D. Health related quality of life expert working group of the International Prophylaxis Study Group. Why should we care about quality of life in persons with haemophilia? Haemophilia 2012:18(3):e Alexander S, Nieder M, Zerr DM, Fisher BT, Dvorak CC, Sung L. Prevention of bacterial infection in pediatric oncology: What do we know, what can we learn? Pediatric Blood and Cancer 2012: 59(1): pp Alos N, Grant RM, Ramsay T, Halton J, Cummings EA, Miettunen PM, Abish S, Atkinson S, Barr R, Cabral DA, Cariney E, Couch R, Dix DB, Fernandez CV, Hay J, Israels S, Laverdière C, Lentle B, Lewis V, Matzinger M, Rodd C, Shenouda N, Stein R, Stephure D, Taback S, Wilson B, Williams K, Rauch F, Siminoski K, Ward LM. High incidence of vertebral fractures in children with acute lymphoblastic leukemia 12 months after the initiation of therapy. Journal of Clinical Oncology 2012: 30(22): pp Angelini P, Arora B, Kurkure P, Bouffet E, Punnett A. Commentary: Critical reflections on subspecialty fellowships in low-income countries. Academic Medicine 2012: 87: pp Angelini P, Hawkins C, Laperriere N, Bouffet E, Bartels U. Post mortem examinations in diffuse intrinsic pontine glioma: Challenges and chances. Journal of Neuro-Oncology 2011: 101: pp Angelini P, London WB, Cohn SL, Pearson AD, Matthay KK, Monclair T, Ambros PF, Shimada H, Leuschner I, Peuchmaur M, Irwin MS, Baruchel S. Characteristics and outcome of patients with ganglioneuroblastoma, nodular subtype: A report from the INRG project. European Journal of Cancer 2012: 48(8): pp Anninga JK, Gelderblom H, Fiocco M, Kroep JR, Taminiau AHM, Hogendoorn PCW, Egeler RM. Chemotherapeutic adjuvant treatment for osteosarcoma: Where do we stand? European Journal of Cancer 2011: 47 (16): pp Baker KS, Bhatia S, Bunin N, Nieder M, Dvorak CC, Sung L, Sanders JE, Kurtzberg J, Pulsipher MA. NCI, NHLBI first international consensus conference on late effects after pediatric hematopoietic cell transplantation: State of the science, future directions. Biology of Blood and Marrow Transplantation 2011: 17(10): pp Bartels U, Hawkins C, Vézina G, Kun L, Souweidane M, Bouffet E. Proceedings of the diffuse intrinsic pontine glioma (DIPG) Toronto Think Tank: Advancing basic and translational research and cooperation in DIPG. Journal of Clinical Oncology 2011: 105: pp Bartels U, Laperriere N, Bouffet E, Drake J. Intracystic therapies for cystic craniopharyngioma in childhood. Front Endocrinology (Lausanne) 2012: Mar 27 (Epub ahead of print). Baruchel S, Pappo A, Krailo M, Baker KS, Wu B, Villaluna D, Lee-Scott M, Adamson PC, Blaney SM. A phase 2 trial of trabectedin in children with recurrent rhabdomyosarcoma, Ewing sarcoma and non-rhabdomyosarcoma soft tissue sarcomas: A report from the Children s Oncology Group. European Journal of Cancer 2012: 48(8): pp Berghuis D, Schilham MW, Vos HI, Santos SJ, Kloess S, Buddingh EP, Egeler RM, Hogendoorn PCW, Lankester AC. Histone deacetylase inhibitors enhance expression of NKG2D ligands in Ewing sarcoma and sensitize for natural killer cellmediated cytolysis. Clinical Sarcoma Research 2012: 2 (1): pp the hospital for sick children department of paediatrics Annual Report

39 Biss TT, Avery PJ, Brandão LR, Chalmers EA, Williams MD, Grainger JD, Leathart JB, Hanley JP, Daly AK, Kamali F. VKORC1 and CYP2C9 genotype and patient characteristics explain a large proportion of the variability in warfarin dose requirement among children. Blood 2012: 119(3): pp Blanco E, Beyene J, Maloney AM, Almeida R, Ethier MC, Winick N, Alexander S, Sung L. Non-relapse mortality in pediatric acute lymphoblastic leukemia: A systematic review and meta-analysis. Leukemia and Lymphoma 2012: 52(5): pp Bouffet E, Hawkins CE, Balloura W, Taylor MD, Bartels U, Schoenhoff N, Tsangaris E, Huang A, Kulkarni A, Maboott DJ, Laperriere N, Tabori U. Survival benefit for pediatric patients with recurrent ependymoma treated with reirradiation. International Journal Radiation Oncology Biology Physics 2012: 18: pp Bouffet E, Jakacki R, Goldman S, Hargrave D, Hawkins C, Shroff M, Hukin J, Bartels U, Foreman N, Kellie S, Hilden J, Etzl M, Wilson B, Stephens D, Tabori U, Baruchel S. Phase II study of weekly vinblastine in recurrent or refractory pediatric low-grade glioma. Journal of Clinical Oncology 2012: 20(30): pp Brandão LR, Labarque V, Diab Y, Williams S, Manson DE. Pulmonary embolism in children. Seminars in Thrombosis and Hemostasis 2011: 37(7): pp Brandao LR, Segel GB. Question from the clinician: Risk of thrombophilia. Pediatric Review 2012: 33(7): pp Brandão LR, Simpson EA, Lau KK. Neonatal renal vein thrombosis. Seminars in Fetal and Neonatal Medicine (6): pp Breakey VR, Blanchette VS. Childhood immune thrombocytopenia: A changing therapeutic landscape. Seminars in Thrombosis and Hemostasis 2011: 37(1): pp Buddingh EP, Ruslan SEN, Berghuis D, Hogendoorn PCW, Egeler RM, Schilham MW, Lankester AC. Intact interferon signaling in peripheral blood leukocyte subsets of high-grade osteosarcoma patients. Cancer Immunology and Immunotherapy 2012: 61 (6): pp Bussel JB, Buchanan GR, Nugent DJ, Gnarra DJ, Bomgaars LR, Blanchette VS, Wang YM, Nie K, Jun S. A randomized, doubleblind study of romiplostim to determine its safety and efficacy in children with immune thrombocytopenia. Blood 2011: 118(1): pp Campo RA, Rowland JH, Irwin ML, Nathan PC, Gritz ER, Kinney AY. Cancer prevention after cancer: Changing the paradigm - A report from the American Society of Preventive Oncology. Cancer Epidemiology, Biomarkers and Prevention 2011: 20(10): pp Castelo-Branco P, Tabori U. Promises and challenges of exhausting pediatric neural cancer stem cells. Pediatric Research 2012: 71(4): pp Chen J, Boodhan S, Nanji M, Chang A, Sekharan S, Lavoratore S, Brandão LR, Skolnik JM, Dupuis LL. A reliable and safe method of collecting blood samples from implantable central venous catheters for determination of plasma gentamicin concentrations. Pharmacotherapy 2011:31 (8): pp Cheng HL, Holowka S, Moineddin R, Odame I. Liver iron overload assessment by T*2 magnetic resonance imaging in pediatric patients: An accuracy and reproducibility study. American Journal Hematology 2012: 87(4): pp Chiu, YE, Drolet BA, Blei F, Carcao MD, Fangusaro J, Kelly ME, Krol A, Lofgren S, Mancini AJ, Metry DW, Recht M, Silverman RA, Tom WL, Pope E. Variable response to propranolol treatment of kaposiform hemangioendothelioma, tufted angioma and Kasabach-Merritt phenomenon. Pediatric Blood and Cancer 2012: Feb 2 (Epub ahead of print). Chung J, Grant RI, Kaplan Dr. Irwin MS. Special AT-rich binding protein-2 (SATB2) differentially affects disease-causing p63 mutant proteins. Journal of Biological Chemistry 2011: 286(47): pp Cox K, Price V, Kahr WH. Inherited platelet disorders: A clinical approach to diagnosis and management. Expert Reviews of Hematology 2011: 4(4): pp Debaun MR, Sarnaik SA, Rodeghier MJ, Minniti CP, Howard TH, Iyer RV, Inusa B, Telfer PT, Kirby-Allen M, Quinn CT, Bernaudin F, Airewele G, Woods GM, Panepinto JA, Fuh B, Kwiatkowski JK, King AA, Rhodes MM, Thompson AA, Heiny ME, Redding-Lallinger RC, Kirkham FJ, Sabio H, Gonzalez CE, Saccente SL, Kalinyak KA, Strouse JJ, Fixler JM, Gordon MO, Miller JP, Ichord RN, Casella JF. Associated risk factors for silent cerebral infarcts in sickle cell anemia: Low baseline hemoglobin, gender and relative high systolic blood pressure. Blood 2012: 119(16): pp Denburg AE, Gupta S, Joffe S, Howard SC, Ribiero RC, Antillon FA, Vasquez R, Sung L. Pediatric oncology research in low income countries: Ethical concepts and challenges. Pediatric Blood and Cancer 2012: 58(4): pp Dimaras H, Kimani K, Dimba EAO, Gronsdahl P, White A, Chan HSL, Gallie BL. Retinoblastoma. Lancet 2012: 379(9824): Diorio C, Martino J, Boydell K, Ethier MC, Mayo C, Wing R, Teuffel O, Sung L, Tomlinson D. Parental perspectives on inpatient versus outpatient management of pediatric febrile neutropenia. Journal of Pediatric Oncology Nursing 2011: 28(6): pp Dror Y, Donadieu J, Koglmeier J, Dodge J, Toiviainen-Salo S, Makitie O, Kerr E, Zeidler C, Shimamura A, Shah N, Cipolli M, Kuijpers T, Durie P, Rommens J, Siderius L, Liu JM. Draft consensus guidelines for diagnosis and treatment of Shwachman-Diamond syndrome. Annual New York Academy of Science 2011: 1242: pp Dubois SG, Shusterman S, Reid JM, Ingle AM, Ahern CH, Baruchel S, Glade-Bender J, Ivy P, Adamson PC, Blaney SM. Tolerability and pharmacokinetic profile of a sunitinib powder formulation in pediatric patients with refractory solid tumors: A Children s Oncology Group study. Cancer Chemotherapy Pharmacology 2012: 69 (4): pp Durno CA, Aronson M, Tabori U, Malkin D, Gallinger S, Chan HSL. Oncologic surveillance for subjects with biallelic mismatch repair gene mutations: 10 year follow-up of a kindred. Pediatric Blood and Cancer 2012: 59(4): pp Dvorak CC, Fisher BT, Sung L, Steinback WJ, Nieder M, Alexander S, Zaoutis TE. Antifungal prophylaxis in pediatric hematology/oncology: New choices and new data. Pediatric Blood and Cancer 2012: 59(1): pp Edelstein K, D agostino N, Bernstein LJ, Nathan PC, Greenberg ML, Hodgson DC, Millar BA, Laperriere N, Spiegler BJ. Long-term neurocognitive outcomes in young adult survivors of childhood acute lymphoblastic leukemia. Journal of Pediatric Hematology/Oncology 2011: 33(6): pp Ellis MJ, Cheshier S, Sharma S, Armstrong D, Hawkins C, Bouffet E, Rutka JT, Taylor MD. Intracerebral malignant peripheral nerve sheath tumor in a child with neurofibromatosis type I and middle cerebral artery aneurysm treated with endovascular coil embolization. Journal of Neurosurgery 2011: 8: pp Engelen V, Detmar S, Koopman H, Stam M, Caron H, Hoogerbrugge P, Egeler RM, Kaspers G, Grootenhuis M. Reporting health-related quality of life scores to physicians during routine follow-up visits of pediatric oncology patients: Is it effective? Pediatrics Blood and Cancer 2012: 58 (5): pp Engelen V, van Zwieten M, Koopman H, Detmar S, Caron H, Brons P, Egeler RM, Kaspers G, Grootenhuis M. The influence of patient reported outcomes on the discussion of psychosocial issues in children with cancer. Pediatric Blood and Cancer 2012: 59 (1): pp Ethier MC, Alexander S, Abla O, Green G, Lam R, Sung L. Association between obesity at diagnosis and weight change during induction and survival in pediatric acute lymphoblastic leukemia. Leukemia Lymphoma: 2012: 53(9): pp Ethier MC, Blanco E, Lehrnbecher T, Sung L. Lack of clarity in the definition of treatment-related mortality: Pediatric acute leukemia and adult acute promyelocytic leukemia as examples. Blood 2011: 188(19): pp Ethier MC, Regier DA, Tomlinson D, Judd P, Doyle J, Gassas A, Naqvi A, Sung L. Perspectives toward oral mucositis prevention from parents and health care professionals in pediatric cancer. Support Care Cancer 2011: Sept 25 (Epub ahead of print). 76 the hospital for sick children department of paediatrics Annual Report

40 Feldman BM, Berger K, Bohn R, Carcao MD, Fischer K, Gringeri A, Hoots K, Mantovani L, Willan A, Schramm W. Hemophilia prophylaxis: How can we justify the costs? Hemophilia 2012: Apr 17 (Epub ahead of print). Finkelstein Y, Arceci R, Weitzman S. Pharmacokinetic and pharmacogenetic determinants and considerations in chemotherapy selection and dosing in infants. Expert Opinion in Drug Metabolic Toxicology 2012: 8(6): pp Fischer K, Collins P, Bjorkman S, Blanchette V, Oh M, Fritsch S, Schroth P, Spotts G, Ewenstein B. Trends in bleeding patterns during prophylaxis for severe haemophilia: Observation from a series of prospective clinical trials. Haemophilia 2011: 17(3): pp Fisher BT, Alexander S, Dvorak CC, Zaoutis TE, Zerr DM, Sung L. Epidemiology and potential preventative measures for viral infections in children with malignancy and those undergoing hematopoietic cell transplantation. Pediatric Blood and Cancer 2012: 59(1): pp Fisher MJ, Loguidice M, Gutmann DH, Listernick R, Ferner RE, Ullrich NJ, Packer RJ, Tabori U, Hoffman RO, Ardern-Holmes SL, Hummel TR, Hargrave Dr. Bouffet E, Charrow J, Bilaniuk LT, Balcer LJ, Liu GT. Visual outcomes in children with neurofibromatosis type 1-associated optic pathway glioma following chemotherapy: A multicenter retrospective analysis. Neuro Oncology 2012: 14(6): pp Fleming AJ, Hukin J, Rasseks SR, Fryer C, Kim J, Stemmer- Rechamimov A, Birks DK, Huang A, Yip S, Dunham C. Atypical teratoid rhabdoid tumours (ATRTs): The British Columbia Children s Hospital Experience, Brain Pathology 2011: Dec 20 (Epub ahead of print). French AE, Greenberg M, Irwin M, Nathan PC. Long-term hepatic outcomes in survivors of stage 4S and 4 neuroblastoma in infancy. Pediatric Blood and Cancer 2012: 58 (2): pp Furlong W, Rae C, Greenberg ML, Barr RD. Surveillance and survival among adolescents and young adults with cancer in Ontario, Canada. International Journal of Cancer 2012: Mar 7 (Epub ahead of print). Gamis AS, Alonzo TA, Gerbing RB, Hilden JM, Sorrell AD, Sharma M, Loew TW, Arceci RJ, Barnard D, Doyle J, Massey G, Perentesis J, Ravindranath Y, Taub J, Smith FO. Natural history of transient myeloproliferative disorder clinically diagnosed in Down syndrome neonates: A report from the Children s Oncology Group Study A2971. Blood 2011: 118 (26): pp Gassas A, Zaidman I, Schechter T, Doyle J. Beyond gastro intestinal graft-versus-host disease - A lesson to learn. Pediatric Transplant 2011: 15 (7): pp E Gerard LM, Xing K, Sherifi I, Granton J, Barth D, Abdelhaleem M, Bergeron C, Weitzman S, Cserti-Gazdewich C. Adult hemophagocytic lymphohistiocytosis with severe pulmonary hypertension and a novel perforin gene mutation. International Journal Hematology 2012: 95(4): pp Goh ES, Perez IC, Canales CP, Ruiz P, Agatep R, Yoon G, Chitayat D, Dror Y, Shago M, Goobie S, Sgro M, Walz K, Mendoza-Londono R. A definition of a critical genetic interval related to kidney abnormalities in the Potocki-Lupski syndrome. American Journal Medical Genetics 2012: 158A(7): pp Goldenberg NA, Brandão L, Journeycake J, Kahn S, Monagle P, Revel-vilk S, Sharathkumar A, Chan AK. Perinatal and Paediatric Haemostasis Subcommittee of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis, definition of post-thrombotic syndrome following lower extremity deep venous thrombosis and standardization of outcome measurement in pediatric clinical investigations. Journal of Thrombosis and Haemostasis 2012: 10(3): pp Gozali AE, Shane L, Gonzalez I, Gilles F, McComb JG, Krieger MD, Lavey RS, Shlien A, Villablanca JG, Erdreich- Epstein A, Dhall, G, Tabori U, Malkin D, Finlay JL. Choroid plexus tumors: Management, outcome and association with the Li-Fraumeni Syndrome: The Children s Hospital Los Angeles (CHLA) Experience, Pediatric Blood Cancer 2012: 58(6): pp Grace RF, Bennett CM, Ritchey AK, Jeng M, Thornburg CD, Lambert MP, Neier M, Recht M, Kumar M, Blanchette V, Klaassen RJ, Buchanan GR, Kurth MH, Nugent DG, Thompson AA, Stine K, Kalish LA, Neufeld EJ. Response to steroid predicts response to rituximab in pediatric chronic immune thrombocytopenia. Pediatric Blood and Cancer 2012: 58(2): pp Granek L, Nathan PC, Rosenberg-Yunger Z, D Agostino N, Amin L, Barr RD, Greenberg ML, Hodgson D, Boydell K, Klassen AF. Psychological factors impacting transition from pediatric to adult care by childhood cancer survivors. Journal of Cancer Survivorship 2012: 6(3): pp Groen W, van der Net J, Bos K, Abad A, Bergstrom BM, Blanchette VS, Feldman BM, Funk S, Helders P, Hilliard P, Manco-Johnson M, Petrini P, Zourikian N, Fischer K. Joint health and functional ability in children with haemophilia who receive intensive replacement therapy. Haemophilia 2011: 17(5): pp Grosse SD, Atrash HK, Odame I, Amendah D, Piel FB, Williams TN. The Jamaican historical experience of the impact of educational interventions on sickle cell disease child mortality. American Journal Preventative Medicine 2012: 41(6): pp e Grosse SD, Odame I, Atrash HK, Amendah DD, Piel FB, Williams TN. Sickle cell disease in Africa: A neglected cause of early childhood mortality. American Journal Preventative Medicine 2011: 41(6): pp S Gruenwald CE, Manlhiot C, Abadilla AA, Kwok J, Maxwell S, Holtby HM, Brandão LR, Chan AK, Crawford-Lean L, Foreman C, Caldarone CA, Van Arsdell GS, McCrindle BW. Heparin brand is associated with postsurgical outcomes in children undergoing cardiac surgery. Annals Thoracic Surgery 2012: 3: pp Gupta AA, Anderson JR, Pappo AS, Spunt SL, Dasgupta R, Indelicato DJ, Hawkins DS. Patterns of chemotherapy-induced toxicities in younger children and adolescents with rhabdomyosarcoma: A report from the Children s Oncology Group Soft Tissue Sarcoma Committee. Cancer 2012: 118 (4): pp Gupta S, Bonilla M, Valverde P, Fu L, Howard SC, Ribeiro RC, Sung L. Treatment related mortality in children with acute myeloid leukaemia in Central America: Incidence, timing and predictors. European Journal of Cancer 2012: 48(9): pp Hagen LE, Schechter T, Luk Y, Brodovitch A, Gassas A, Doyle JJ. High alpha-1 antitrypsin clearance predicts severity of gut graft-versus-host disease (GVHD) in children. Pediatric Transplant 2011: 15 (6): pp Halton J, Nagel K, Brandao LR, Silva M, Gibson P, Chan A, Blyth K, Hicks K, Parmar N, Paddock L, Willing S, Thabane L, Athale U. Do children with central venous line (CVL) dysfunction have increased risk of symptomatic thromboembolism compared to those without CVL-dysfunction, while on cancer therapy? BMC Cancer 2012: 12(1): pp 314. Hawkins CE, Bartels U, Bouffet E. Molecular genetic approaches and potential new therapeutic strategies for pediatric diffuse intrinsic pontine glioma. Journal of Clinical Oncology 2011: 29: pp Hay CR, DiMichele DM. The principal results of the International Immune Tolerance Study: A randomized dose comparison. Blood 2012: 119(6): Henderson TO, Oeffinger KC, Whitton J, Leisenring W, Neglia J, Meadows A, Crotty C, Rubin DT, Diller L, Inskip P, Smith SA, Stoval M, Constine LS, Hammond S, Armstrong GT, Robison LL, Nathan PC. Subsequent gastrointestinal malignancies in childhood cancer survivors: A cohort study. Annals of Internal Medicine 2012: 156: pp Heng Vong C, Bajard A, Thiesse P, Bouffet E, Seban H, Marec Berard P. Deep sedation in pediatric imaging: Efficacy and safety of intravenous chlorpromazine. Pediatric Radiology 2012: Jan 13 (Epub ahead of print). Hutso JR, Weitzman S, Schechter T, Arceci RJ, Kim RB, Finkelstein Y. Pharmacokinetic and pharmacogenetic determinants and considerations in chemotherapy selection and dosing in infants. Expert Opinion on Drug Metabolism and Toxicology 2012: 8 (6): pp Ibrahim GM, Fallah A, Shahideh M, Tabori U, Rutka JT. Primary Ewing s sarcoma affecting the central nervous system: A review and proposed prognostic considerations. Clinical Neuroscience 2012: 19(2): pp Ibrahim GM, Huang A, Halliday W, Dirks P, Malkin D, Baskin B, Shago M, Hawkins C. Cribriform neuroepithelial tumor: Novel clinicopathological, ultrastructural and cytogenetic findings. Acta Neuropathologica 2011: 122 (4): pp Inbal A, Oldenburg J, Carcao MD, Rosholm A, Tehranchi R, Nugent D. Recombinant Factor XIII, safe and novel treatment for hereditary Factor XIII deficiency. Blood 2012: 119(22): pp the hospital for sick children department of paediatrics Annual Report

41 Jakacki RI, Bouffet E, Adamson PC, Pollack IF, Ingle AM, Voss SD, Blaney SM. A phase 1 study of vinblastine in combination with carboplatin for children with low-grade gliomas: A Children s Oncology Group phase 1 consortium study. Journal of Neuro-Oncology 2011: 13: pp Janssens GO, Jansen MH, Lauwers SJ, Nowak PJ, Oldenburger FR, Bouffet E, Saran F, Kamphuis-van Ulzen K, van Lindert EJ, Schieving JH, Boterberg T, Kaspers GJ, Span PN, Kaanders JH, Gidding CE, Hargrave D. Hypofractionation vs conventional radiation therapy for newly diagnosed diffuse intrinsic pontine glioma: A matched-cohort analysis. International Journal of Radiation Oncology Biology Physics 2012: Jun 9 (Epub ahead of print). Jol-van der Zijde CM, Bredius RGM, Jansen-Hoogendijk AM, Raaijmakers S, Egeler RM, Lankester AC, van Tol MJD. IgG antibodies to ATG early after pediatric hematopoietic stem cell transplantation increase the risk of acute graft versus host disease. Bone Marrow Transplantation 2012: 47 (3): pp Jordan MA, Allen C, Weitzman S, Filipovich AH, McClain K. How I treat hemophagocytic lymphohistiocytosis. Blood 2011: 118(15): pp Joris M, Bierings MB, Egeler RM, Claas FHJ, van Rood JJ, Oudshoorn M. Access to allogeneic hematopoietic stem cell transplantation for patients with MDS or relapsed AML treated according to protocols of the Dutch Childhood Oncology Group. Bone Marrow Transplantation 2012: 47 (5): pp Kahr WH, Hinckley J, Li L, Schwertz H, Christensen H, Rowley JW, Pluthero FG, Urban D, Fabbro S, Nixon B, Gadzinski R, Storck M, Wang K, Ryu GY, Jobe SM, Schutte BC, Moseley J, Loughran NB, Parkinson J, Weyrich AS, Di Paola J. Mutations in NBEAL2, encoding a BEACH protein, cause gray platelet syndrome. Nature Genetics 2011: 43(8): pp Khuong-Quang DA, Buczkowicz P, Rakopoulos P, Liu XY, Fontebasso AM, Bouffet E, Bartels U, Albrecht S, Schwartzentruber J, Letourneau L, Bourgey M, Bourque G, Montpetit A, Bourret G, Lepage P, Fleming A, Lichter P, Kool M, von Deimling A, Sturm D, Korshunov A, Faury D, Jones DT, Majewski J, Pfister SM, Jabado N, Hawkins C. K27M mutation in histone H3.3 defines clinically and biologically distinct subgroups of pediatric diffuse intrinsic pontine gliomas. Acta Neuropathologica 2012: Jun 3 (Epub ahead of print). Kim YH, Faaij CMJM, van Halteren AGS, Schrama E, Dellemijn TAM, Scholler J, Egeler RM, Pavel S, Vyth-Dreese FA, van Tol MJD, Goulmy E, Spierings E. In situ detection of HYspecific T cells in acute graft-versus-host disease-affected male skin after gender mismatch stem cell transplantation. Biology of Blood and Marrow Transplantation 2012: 18(3): pp Klaassen R, Mathias S, Buchanan G, Bussel J, Deuson R, Young N, Collier A, Bomgaars L, Blanchette V. Effect of Romiplostim on child health-related quality of life and parental burden in immune thrombocytopenia (ITP). Pediatric Blood and Cancer 2012: 58(3): pp Klassen AF, Anthony S, Khan A, Sung L, Klaassen R. Identifying determinants of quality of life of children with cancer and childhood cancer survivors: A systematic review. Supportive Care in Cancer 2011: 19(9): pp Klassen AF, Dix D, Papsdorf M, Klaassen RJ, Yanofsky R, Sung L. Impact of caring for a child with cancer on single parents compared with parents from two-parent families. Pediatric Blood and Cancer 2012: 58(1): pp Klassen AF, Gulati S, Granek L, Rosenberg-Yunger ZR, Watt L, Sung L, Klaassen R, Dix D, Shaw NT. Understanding the health impact of caregiving: A qualitative study of immigrant parents and single parents of children with cancer. Quality of Life Research 2011: Nov 30 (Epub ahead of print). Klassen AF, Raina P, McIntosh C, Sung L, Klaassen RJ, O Donnell M, Yanofsky R, Dix D. Parents of children with cancer: Which factors explain differences in health-related quality of life. International Journal of Cancer 2011: 129(5): pp Koltin D, Sung L, Naqvi A, Urbach SL. Medication induced diabetes during induction in pediatric acute lymphoblastic leukemia: Prevalence, risk factors and characteristics. Support Care Cancer 2011: Nov 8 (Epub ahead of print). Kraemer BF, Campbell RA, Schwertz H, Cody MJ, Franks Z, Tolley ND, Kahr WH, Lindemann S, Seizer P, Yost CC, Zimmerman GA, Weyrich AS. Novel anti-bacterial activities of β-defensin 1 in human platelets: Suppression of pathogen growth and signaling of neutrophil extracellular trap formation. PLoS Pathogens 2011: Nov 10 (Epub ahead of print). Kumar S, Bayat Mokhtari R, Shiekh R, Wu B, Zhang L, Xu P, Man S, Oliveira ID, Yeger H, Kerbel RS, Baruchel S. Metronomic oral topotecan with pazopanib is an active antiangiogenic regimen in mouse models of aggressive pediatric solid tumor. Clinical Cancer Research 2011: 17(17): pp Kwiatkowski J, Kim H-Y, Thompson A, Quinn C, Mueller B, Odame I, Giardini P, Vichinsky E, Boudreax J, Cohen A, Porter J, Coates T, Olivieri N, Neufeld E for the Thalassemia Clinical Research Network. Chelation use and iron burden in North American and British thalassemia patients: A report from the Thalassemia Longitudinal Cohort. Blood 2012: 119 (12): pp Lafay-Cousin L, Hawkins C, Carret AS, Johnston D, Zelcer S, Wilson B, Jabado N, Scheinemann K, Eisenstat D, Fryer A, Mpofu C, Larouche V, Strother D, Bouffet E, Haung A. Central nervous system atypical teratoid rhabdoid tumours: The Canadian Paediatric Brain Tumour Consortium experience. European Journal of Cancer 2011: Oct 22 (Epub ahead of print). Lafay-Cousin L, Bouffet E. In response to Clinical features and management of carboplatin-related hypersensitivity reactions in pediatric low grade glioma. Supportive Care in Cancer 2011: Nov 11 (Epub ahead of print). 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42 McCrone L, Alexander, S, Karsli C, Taylor G, Amaral JG, Parra D, Temple M, John P, Connolly BL. US guided percutaneous needle biopsy of anterior mediastinal masses in children. Pediatric Radiology 2012: 42(1): pp Mendoza-Londono R, Chitayat D, Kahr WH, Hinek A, Blaser S, Dupuis L, Goh E, Badilla-Porras R, Howard A, Mittaz L, Superti-Furga A, Unger S, Nishimura G, Bonafe L. Extracellular matrix and platelet function in patients with musculocontractural Ehlers-Danlos syndrome caused by mutations in the CHST14 gene. American Journal of Medical Genetics A 2012: 158A(6): pp Naithani R, Ngan BY, Roifman C, Crump M, Baruchel S, Abla O. Thymic mucosa-associated lymphoid tissue lymphoma in an adolescent girl. Journal of Pediatric Hematology Oncology 2012: Mar 30 (Epub ahead of print). Nam RK, Zhang W, Siminovitch K, Shlien A, Kattan MW, Klotz LH, Trachtenberg J, Lu Y, Zhang J, Yu C, Toi A, Loblaw DA, Venkateswaran V, Stanimirovic A, Sugar L, Malkin D, Seth A, Narod SA. New variants at 10q26 and 15q21 are associated with aggressive prostate cancer in a genome-wide association study from a prostate cancer screening cohort. Cancer Biology and Therapy 2011: 12(11): pp Nathan PC, Schiffman J, Huang S, Landier W, Bhatia S, Kent D, Wright J, Oeffinger KC, Hudson MM. Childhood cancer survivorship educational resources in North American Pediatric Hematology/Oncology Fellowship Training Programs: A survey study. Pediatric Blood and Cancer 2011: 57(7): pp Nathan PC, Urbach SL. The metabolic cost of childhood ALL. Blood 2011: 117 (17): pp Nicholas DB, Chahauver A, Brownstone D, Hetherington R, McNeill T, Bouffet E. Evaluation of an online peer support network for fathers of a child with a brain tumor. Social Work Health Care 2012: 51(3): pp Noone D, Al-Matrafi J, Tinckam K, Zipfel PF, Herzenberg AM, Thorner PS, Pluthero FG, Kahr WH, Filler G, Hebert D, Harvey E, Licht C. 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Rapid, reliable and reproducible molecular sub-grouping of clinical medulloblastoma samples. Acta Neuropathology 2012: 123(4): pp Odame I, Kulkarni R, Ohene-Frempong K. Concerted global effort to combat sickle cell disease: The first global congress on sickle cell disease in Accra, Ghana. American Journal Preventative Medicine 2011: 41(6): pp S Pahl JH, Ruslan SE, Buddingh EP, Justo Santos S, Szuhai K, Serra M, Geldreblom H, Hogendoorn PC, Egeler RM, Schilham MW, Lankester AC. Anti-EGFR antibody cetuximab enhances the cytolytic activity of natural killer cells towards osteosarcoma. Clinical Cancer Research 2012: 18 (2): pp Parker TM, Bassal M, Klaassen R, Nikkel SM, Cada M, Johnston DL. A case of a young girl with myelodysplastic syndrome (MDS), dysmorphic features, short stature and developmental delay Is there a link? Journal of Clinical Case Reports 2012: 2(7): pp 1-2. Parsons SK, Phipps S, Sung L, Baker KS, Pulsipher MA, Ness KK. 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43 Schechter T, Kirton A, Laughlin S, Pontigon AM, Finkelstein Y, Macgregor D, Chan A, deveber G, Brandão LR. Safety of anticoagulants in children with arterial ischemic stroke. Blood 2012: 119(4): pp Schwartzentruber J, Korshunov A, Liu X-Y, Jones DTW, Pfaff E, Jacob K, Sturm D, Fontebasso AM, Quang DAK, Tönjes M, Hovestadt V, Albrecht S, Nantel A, Konermann C, Jäger N, Rausch T, Ryzhova M, Korbel J, Hielscher T, Hauser P, Garami M, Klekner A, Bognar L, Ebinger M, Schuhmann MU, Scheurlen W, Pekrun A, Frühwald M, Roggendorf W, Kramm C, Dürken M, Atkinson J, Lepage P, Montpetit A, Zakrzewska M, Zakrzewski K, Liberski PP, Dong Z, Siegel P, Kulozik AE, Zapatka M, Guha A, Malkin D, Felsberg J, Reifenberger G, von Deimling A, Ichimura K, Collins VP, Witt H, Milde T, Witt O, Zhang C, Castelo-Branco P, Lichter P, Faury D, Tabori U, Plass C, Majewski J, Pfister SM, Jabado N. Exome sequencing identifies frequent driver mutations in histone H3.3 and ATRX-DAXX in pediatric glioblastoma. Nature 2012: 482(7384): pp Sen S, Wang H, Nghiem CL, Zhou K, Yau J, Tailor C, Irwin M, Dror Y. The ribosome-related protein, SBDS, is critical for normal erythropoiesis. Blood 2011: 118(24): pp Shaikh F, Ngan BY, Alexander S, Grant R. Progressive transformation of germinal centers in children and adolescents: An intriguing cause of lymphadenopathy. Pediatric Blood Cancer 2012: June 15 (Epub ahead of print). Sharathkumar A, Carcao MD. Clinical advances in hemophilia management. Pediatric Blood and Cancer 2011: 57(6): pp Shuangshoti S, Shuangshoti S, Nuchprayoon I, Kanjanapongkul S, Marrano P, Irwin MS, Thorner PS. Natural course of low risk neuroblastoma. Pediatric Blood Cancer 2012: 58(5): pp Sivaprakasam P, Gupta AA, Greenberg ML, Capra M, Nathan PC. Survival and long-term outcomes in children with hepatoblastoma treated with continuous infusion of cisplatin and doxorubicin. Journal of Pediatric Hematology/ Oncology 2011: 33(6): pp Stinson JN, Sung L, Gupta A, White ME, Jibb LA, Dettmer E, Baker N. Disease self management needs of adolescents with cancer: Perspectives of adolescents with cancer and their parents and healthcare providers. Journal of Cancer Survivorship 2012: Apr 28 (Epub ahead of print). Sung L, Alibhai SM, Ethier MC, Teuffel O, Cheng S, Fisman D, Regier DA. Discrete choice experiment produced estimates of acceptable risk of therapeutic options in cancer patients with febrile neutropenia. Journal of Clinical Epidemiology 2012: 65(6): pp Sung L, Buxton A, Gamis A, Woods WG, Alonzo TA. Lifethreatening and fatal infections in children with acute myeloid leukemia: A report from the Children s Oncology Group. Journal of Pediatric Hematology and Oncology 2012: 34(1): pp e Sung L, Manji A, Beyene J, Dupuis LL, Alexander S, Phillips R, Lehrnbecher T. Fluoroquinolones in children with fever and neutropenia: A systematic review of prospective trials. Pediatric Infectious Diseases 2012: 31(5): pp Tabori U, Taylor M, Bouffet E, Malkin D, Hawkins C. Reply to Lindsey et al. Journal of Clinical Oncology 2011: 29: 12: 347. Terasaki M, Bouffet E, Maeda M, Sugita Y, Sawamura Y, Morioka M. Successful treatment of leptomeningeal gliomatosis of pilomyxoid astrocytoma after failed frontline chemotherapy. Neurologist 2012: 18: pp Teuffel O, Alibhai S, Cheng S, Ethier MC, Diorio C, Martino JR, Mayo C, Wing R, Sung L. Health-related quality of life anticipated with different management strategies for febrile neutropenia in adult cancer patients. British Journal Cancer 2011: 105(5): pp Teuffel O, Kuster SP, Hunger SP, Conter V, Hitzler J, Ethier MC, Shah P, Beyene J, Sung L. Dexamethasone versus prednisone for induction therapy in childhood acute lymphoblastic leukemia: A systematic review and meta-analysis. Leukemia 2011: 25(8): pp Teuffel O, Sung L. Advances in management of low-risk febrile neutropenia. Current Opinion in Pediatrics 2012: 24(1): pp Te Winkel M, Pieters R, Hop W, de Groot-Kruseman H, Lequin M, van der Sluis I, Bokkerink J, Leeuw J, Bruin M, Egeler RM, Veerman A, van den Heuvel-Eibrink M. A prospective study on incidence, risk factors and long-term outcome of osteonecrosis in pediatric acute lymphoblastic leukemia. Journal of Clinical Oncology 2011: 29 (31): pp Tomlinson D, Bartels U, Gammon J, Hinds PS, Volpe J, Bouffet E, Regier DA, Baruchel S, Greenberg M, Barrera M, Llewellyn- Thomas H, Sung L. Chemotherapy versus supportive care alone in pediatric palliative care for cancer: Comparing the preferences of parents and health professionals. Canadian Medical Association Journal 2011: 183(17): pp Tomlinson D, Hendershot E, Bartels U, Maloney AM, Armstrong C, Wrathall G, Sung L. Concordance between couples reporting their child s quality of life and their decision making in pediatric oncology palliative care. Journal of Pediatric Oncology Nursing 2011: 28: pp Tomlinson D, Mermel LA, Ethier MC, Matlow A, Gillmeister B, Sung L. Defining bloodstream infections due to central venous catheters in cancer patients: A systematic review. Clinical Infectious Disease 2011: 53(7): pp Traubici J, Somers GR, Ling S, Pearl R, Nathan PC. Pleuropulmonary blastoma in a patient with autosomal recessive polycystic kidney disease. Pediatric Radiology 2011: 41(11): pp Trottestam H, Horne AC, Arico M, Egeler RM, Filipovich AH, Gadner H, Imashuku S, Ladisch S, Webb D, Janka G, Henter JI for the Histiocyte Society. Chemo-immunotherapy for hemophagocytic lymphohistiocytosis: Long-term results of the HLH-94 treatment protocol. Blood 2011: 118 (17): pp Tsimicalis A, Stevens B, Ungar WJ, Greenberg M, McKeever P, Agha M, Guerriere D, Barr R, Naqvi A, Moineddin R. Determining the costs of families support networks following a child s cancer diagnosis. Cancer Nursing 2012: May 4 (Epub ahead of print). Urbach SL, Guger S, Nathan PC. Long-term survivors of cancer in childhood and adolescence. Current Pediatric Reviews 2012: 8 (2): pp Urbach S, Laperriere N, Bartels U. Endocrine complications in children treated for medulloblastoma or ependymoma using radiation therapy outcomes in the computed tomography planning era. European Oncology and Haematology 2011: 7: pp van den Akker M, Angelini P, Taylor G, Chami R, Gerstle JT, Gupta A. Malignant pancreatic tumours in children: A single institution series. Journal of Pediatric Surgery 2012: 47 (4): pp Van Walraven SM, Ball LM, Koopman HM, Switzer GE, Ropes-de Jong CM, de Jong A, Bredius RG, Egeler RM. Managing a dual role-experiences and coping strategies of parents donating haploidentical G-CSF mobilized peripheral blood stem cells to their children. Psychology 2012: 21 (2): pp Villani A, Malkin D, Tabori U. Syndromes predisposing to pediatric central nervous system tumors: Lessons learned and new promise. Current Neurology and Neuroscience Reports 2012: 12(2): pp Vincent J, van Gelder M, Egeler RM, Bos GMJ. Haplo-identieke stamceltransplantaties: Een nieuwe strategie. Nederlands Tijdschrift voor Hematologie 2011: 8 (6): pp Walker E, Zhang C, Castelo-Branco P, Hawkins C, Wilson W, Zhukova N, Alon N, Novokmet A, Baskin B, Ray P, Knobbe C, Dirks P, Taylor MD, Croul S, Malkin D, Tabori U. Clinical and biological implications of monoallelic expression in benign and malignant brain tumors. Cancer Research 2012: 72(3): pp Wang Y, Roche O, Xu C, Moriyama EH, Heir P, Chung J, Roos RC, Chen E, Finak G, Milosevic M, Wilson BC, The BT, Park M, Irwin MS, Ohh M. Hypoxia promotes ligandindependent EGFR signaling via HIF-mediated upregualtion of caveolin. Proceedings of the National Academy of Sciences 2012: 109(13): pp Wasserman J, Zambetti G, Malkin D. Towards an understanding of the role of p53 in adrenocortical carcinogenesis. Molecular and Cellular Endocrinology 2012: 351(1): pp Watt L, Dix D, Gulati S, Sung L, Klaassen RJ, Shaw NT, Klassen AF. Family-centered care: A qualitative study of Chinese and South Asian immigrant parents experiences of care in pediatric oncology. Child: Care, Health and Development 2011: Nov 9 (Epub ahead of print). 84 the hospital for sick children department of paediatrics Annual Report

44 Webb B, Barrera M, Beyene J, Carcao MD, Daneman D, Elliott I, Gong GWK, Halperin I, Lord S, Melville H, Narayanan UG, Ota S, Solomon M, Sung L, Young NL, Zachos M, Feldman BM. Determinants of quality of life in children with chronic somatic disease: Pilot data from the GapS questionnaire. Quality of Life Research 2012: Mar 30 (Epub ahead of print). Webert KE, Rivard GE, Teitel J, Carcao MD, Lilllicrap D, St-Louis J, Walker IR. Low prevalence of inhibitor antibodies in the Canadian haemophilia population. Haemophilia 2012: 18(3): pp e Weitzman S. Approach to hemophagocytic lymphohistiocytosis syndromes. Hematology American Society Hematology Education Program 2011: 2011: pp Williams B, Meyn MS, Hitzler J. Transient leukemia in newborns without Down syndrome- Diagnostic and management challenges. Journal of Pediatric Hematology/ Oncology 2011: 33 (6): pp e Witt H, Mack SC, Ryzhova M, Bender S, Sill M, Isserlin R, Benner A, Hielscher T, Milde T, Remke M, Jones DT, Northcott PA, Garzia L, Bertrand KC, Wittmann A, Yao Y, Roberts SS, Massimi L, Van Meter T, Weiss WA, Gupta N, Grajkowska W, Lach B, Cho YJ, von Deimling A, Kulozik AE, Witt O, Bader GD, Hawkins CE, Tabori U, Guha A, Rutka JT, Lichter P, Korshunov A, Taylor MD, Pfister SM. Delineation of two clinically and molecularly distinct subgroups of posterior fossa ependymoma. Cancer Cell 2011: 20(2): pp Wu X, Northcott, Dubuc A, Dupuy AJ, Shih DJ, Witt H, Croul S, Bouffet E, Fults DW, Eberhart CG, Garzia L, Van Meter T, Zagzag D, Jabado N, Schwartzentruber J, Majewski J, Scheetz TE, Pfister SM, Korshunov A, Li XN, Scherer SW, Cho YJ, Akagi K, MacDonald TJ, Koster J, McCabe MG, Sarver AL, Collins VP, Weiss WA, Largaespada DA, Collier LS, Taylor MD. Clonal selection drives genetic divergence of metastatic medulloblastoma. Nature 2012: 482(7386): pp Young G, Auerswald G, Jimenez-Yuste V, Konkle BA, Lambert T, Morfini M, Santagostino E, Blanchette V. When should prophylaxis therapy in inhibitor patients be considered? Haemophilia 2011:17(5): pp e849-e857. Young NL, St-Louis J, Burke T, Hershon L, Blanchette V. Cross-cultural validation of the CHO-KLAT and HAEMO-QoL-A in Canadian French. Haemophilia 2012: 18(3): pp Yu A, Stephens D, Feldman BM, Parkin PP, Kahr WH, Brandão LR, Shouldice M, Levin AV. The role of prothrombotic factors in the ocular manifestations of abusive and non-abusive head trauma: A feasibility study. Child Abuse Neglect 2012: 36(4): pp Zorzi AP, Grant R, Gupta AA, Hodgson DC, Nathan PC. Cranial nerve palsies in childhood parameningeal rhabdomyosarcoma. Pediatric Blood Cancer 2012: Mar 7 (Epub ahead of print). Books and Chapters Arico M, Weitzman S. The histiocytoses. In: Cancer in Children Clinical Management, Sixth Edition. (Stevens MCG, Caron HN, Biondi A, eds.). Oxford University Press, UK Bouffet E, Bartels U: The use of steroids in patients with DIPG. In: Understanding the Journey: A Parent s Guide to DIPG (Hoffman, Ruth I, ed.) American Childhood Cancer Organization, Kensington, MD Brandão L, Howard S, Gow K, Rajasekaran S, Tamburro R. Hematology and oncology in critical illness. In: Pediatric Critical Care Study Guide (Lucking SE, Maffei FA, Tamburro RF, Thomas NJ, eds.) Springer London, UK Dror Y. Inherited bone marrow failure syndromes: Genetic complexity monogenic disorders. In: Genetic Disorders. InTech Open Access Publisher, Croatia Egeler RM, Arceci RJ, Henter JI. Histiocytoses In: Rudolph s Pediatrics, 22nd Edition (Rudolph CD, Rudolph AM, Lister GE, First LR, Gershon AA, eds.). McGraw-Hill Publishers, New York NY 2011: pp Handgretinger R, Egeler RM. Allogeneic stem cell transplantation and immunotherapy. In: Cancer in Children, Clinical Management, Sixth Edition (Stevens MCG, Caron HC, Biondi A, eds.). Oxford University Press, New York Hawkins C, Bouffet E, Bartels U. DIPG and tissue donation. In: Understanding the Journey: A Parent s Guide to DIPG (Hoffman, Ruth I, eds.) American Childhood Cancer Organization, Kensington, MD Huang A, Bartels U, Bouffet E. Spinal cord tumors. In: Clinical Pediatric Oncology and Hematology, Scientific Principles and Clinical Practice (Estlin, Gilbertson, Wynn, eds.). Wiley-Blackwell Publishing, Toronto Jhanke K, Shenkier T, Abla O. Rare variants of primary CNS lymphoma: Pediatric primary CNS lymphoma. In: Lymphoma and Leukemia of the Nervous System. (Batchelor, DeAngelis, eds.). Springer Publishing, New York Nathan PC, Janzen L, Henderson T. Late effects after treatment for hematological malignancies during childhood, adolescence or young adulthood. In: Hematologic Malignancies in Childhood, Adolescents and Young Adults (Cairo M, Perkins S, eds.). World Scientific Publishing, UK Nessim C, Ferguson P, Gupta A, Catton C, Gladdy R. Retroperitoneal and extremity soft tissue sarcomas. In: University of Toronto Surgical Oncology Manual (Culkier M, Gillis A, Escallon J, Wright F, eds.). University of Toronto, Toronto Punnett A. Organ and tissue donation by pediatric brain tumor patients. In: Understanding the Journey, A Parent s Guide to DIPG (Hoffman R, Swisher L, eds.). American Childhood Cancer Organization, Kensington, MD Rodriguez L, Punnett A. Post-transplant lymphoproliferative disorders following solid organ transplantation. In: Organ Donation and Transplantation- Public Policy and Clinical Perspectives. InTech, New York Sokolik C, Gupta A, Ling SC. Hepatocellular carcinoma in children. In: Primary Liver Cancer: Surveillance, Diagnosis and Treatment (Reau N, Poordad F, eds.). Springer Science, New York Spence T, Ngugen J, Bouffet E, Huang A. MicroRNAs in brain tumors. In: MicroRNA in Cancer Translational Research (Cho, William CS, ed.). Springer, Dordrecht Heidelberg London New York Sung L. Initial management of low-risk pediatric fever and neutropenia: Efficacy and safety, costs, quality-of-life considerations and preferences. In: ASCO 2012 Educational Book (Govindan R, ed.). American Society of Clinical Oncology, Alexandria, VA Tomlinson D, Sung L: Mucositis and the development of a new instrument for the measurement of oral mucositis in children. In: Perspectives on Cancer Care (Fawcett JN, McQueen A, eds.). Blackwell Publishing Ltd, Toronto Truong TH, Weitzman S, Arceci RJ. Non-Hodgkin lymphoma of childhood. In: Neoplastic Diseases of the Blood, Fifth edition (Wiernik PH, Goldman JM, Dutcher JP, Kyle RA, eds.). Cambridge University Press, New York Whitlock JA, Horton TM: Design of early-phase trials. In: New Agents for the Treatment of Acute Lymphoblastic Leukaemia (Saha V, Kearns P, eds.). Springer Publishing, NY, New York Grants and Funding A feasibility trial of individualized homeopathic treatment of fatigue in children receiving chemotherapy. Sung L, Brule D. Lotte and John Hecht Foundation. $69,530 CAD to 2013 A multi-centre, open-label, single-arm, and multiple dosing trial on efficacy and safety of monthly recombinant factor XIII (rfxiii) in subjects with congenital factor XIII deficiency - Safety extension trial. Carcao M, Blanchette V. NovoNordisk. $12,647 CAD/pt to present A multicentre, randomized, open-label phase II trial evaluating deferasirox compared with deferoxamine in patients with cardiac iron overload due to chronic blood transfusions. Maseruka H, Jehl V, Lawniczek R, Odame I. Novartis. $23,345 CAD/pt to 2012 A phase I study of Tie2 peptide inhibitor in recurrent solid tumour. Baruchel S. COG and AMGEN. $52,000 CAD to 2014 A phase II, open label, multi-center, single-dose pharmacokinetics, and multiple dose study of the safety, efficacy and tolerability of FBS0701 in a pediatric population with transfusional iron overload. Kirby M, Williams S, Odame I. Ferrokin Biosciences. $14,220/patient to 2012 A phase II study of pazopanib in recurrent solid tumour. Baruchel S. COG and GSK. $62,000 CAD to the hospital for sick children department of paediatrics Annual Report

45 A program of Paediatric Pharmacogenomics Medicine (PPM) : GeneMed to evaluate existing evidence and produce new knowledge on genetic testing for prevention of drug side effects in children. Ito S, Hitzler J. Ontario Ministry of Health Drug Innovation Fund. $589,697 CAD to 2015 A prospective non-interventional study to describe health-related quality of life of youth and young adults with moderate or severe hemophilia A using recombinant factor VIII (Helixate FS). St. Louis J, Young N, Blanchette V. CSL Behring. $296,388 CAD to 2013 A randomized controlled multicenter non-inferiority trial of twice daily low dose dexamethasone versus high dose dexamethasone for symptom control in children undergoing cranial or craniospinal radiation after complete or near complete resection of a brain tumour. Bartels U. C17 Research Network. $120,000 CAD to 2011 A randomized, open-label, multi-dose study of HQK-1001 in subjects with sickle cell disease. Kirby M, Odame I. Haemaquest Inc. $14,512/patient to 2012 A randomized study of IVIG vs IVIG with high dose methylprednisolone in rapidly augmenting platelet counts in childhood ITP. Carcao M, Blanchette V. Grifols. $145,065 CAD to 2012 A three-month prospective open label study of therapy with Fragmin (Dalteparin Sodium Injection) in children with malignancies and venous thromboembolism. Brandão LR, Williams S. Eisai Inc. $10,000/pt. Apr 2010 to Mar 2014 A-LONG: An open-label, multicenter evaluation of the safety, pharmacokinetics, and efficacy of recombinant, long-acting coagulation factor VIIIFc fusion (rfviiifc) in the prevention and treatment of bleeding in previously treated subjects with severe hemophilia A. Doria A, Blanchette V, Mclimont M, Brandão L, Carcao M, Vilaça P, Carneiro J, D Amico E. Biogen Idec. $19,996 CAD. Oct to Nov Acute megakaryocytic leukemia in Down syndrome. Hitzler J. Canadian Cancer Society Research Institute. $142,000 CAD to 2013 An international network to determine the origins and improve survival for children with mismatch repair genes mutations affected by malignant brain tumors. Tabori U, Bakry D, Hawkins C, Taylor M, Durno C, Aaronson M, Galinger S, Qaddoumi I, Bouffet E, Malkin D. B.r.a.i.n.child. $68,700 CAD to 2013 Antimicrobial prophylaxis: An assessment of preferences in pediatric patients with cancer, parents of children with cancer and healthcare professionals. Sung L, Diorio C. University of Toronto Comprehensive Research Experience for Medical Students. $7,500 CAD to 2012 Antithrombin enhancement may improve anticoagulation efficiency in infants undergoing cardiopulmonary bypass for cardiac surgery. Gruenwald C, Chan A, Van Arsdell GS, McCrindle BW, Holtby H, Brandão LB, Manlhiot C. Heart and Stroke Foundation of Ontario/Baxter Healthcare Corporation. $395,518 CAD. Jun 2010 to July 2012 Applying biomarkers to long-term effects in child and adolescent cancer treatment (ABLE Team). Schultz. CIHR ABLE Team Grant. $4,076,000 CAD to 2015 Assessing the efficacy of a psychosocial intervention program for siblings of children with cancer. Barrera M, Nathan P, Chung J, Schulte F, Strother D, Bond M. C17 Research Network. $197,684 CAD. Jul 2011 to Jun 2013 Attitudes and preferences toward infection prophylaxis in pediatric oncology. Sung L, Alexander S, Regier D, Boydell K, Ethier MC. Pediatric Oncology Group of Ontario. $13,942 CAD to 2012 B-LONG: An open-label, multicenter evaluation of the safety, pharmacokinetics, and efficacy of recombinant, long-acting coagulation Factor IX Fc fusion (rfixfc) in the prevention and treatment of bleeding in previously treated subjects with severe hemophilia B. Carcao M. Biogen-Idec. $20,315 US/pt to 2012 Biological and clinical impact of chromothripsis and early TP53 mutations on carcinogenesis in Li-Fraumeni syndrome. Canadian Cancer Research Society. $200,000 CAD to 2014 Bone marrow failure-causing alleles in Canada and genotypephenotype correlation. Dror Y, Cada M, Odame I, Yanofsky R, Steele M, J, Dower N, Fernandez C, Silva M, Champagne J, Jardine L, Abish S, Klaassen R, Ali K, Michon B, Brossard J, Goodyear L, Wu J, Sung L, Beyene J. C17 Research Network. $200,000 CAD to 2014 Canadian hemophilia dose escalation primary prophylaxis (CHPS-3) observation extension study. Feldman BM, Blanchette V, Rivard GE, Israels S, Chan A, Lillicrap D, Willan A, Einarson T, Babyn P, Doria A, Wintler R. Bayer HealthCare Canada. $583,671 CAD to 2012 Child Health Research Institute (Infrastructure for new Research Building). Rossant J, Malkin D. Canada Foundation for Innovation. $91,000,000 CAD to 2013 Children s Oncology Group Chair s Grant. Adamson P, Whitlock JA. National Childhood Cancer Foundation. $13,264 CAD. Mar to Feb Clinical and biological implications of monoallelic gene expression in pediatric malignant brain tumors. Tabori U, Taylor M. Canadian Institute of Health Research. $573,543 CAD to 2016 Clinical assessment of thrombosis in children after heart surgery: The CATCH Study. Manlhiot C, Brandão LR, Gruenwald C, Chan AK, Schwartz S, Holtby H, Sivarajan B, Van Arsdell GS, Caldarone C, Mertens L, Mital S, McCrindle BW. Canadian Institute of Heart Research. $1,514,330 CAD. Jul to Jun Clinical Research Training Institute (CRTI) Summer Workshop for the American Society of Hematology. Sung L, Miller E, Clayton C, Basso J, Lombardi K. National Institutes of Health National Heart, Lung and Blood Institute. $31,350 USD to 2012 Comparing the burden of illness of hemophilia in the developing and the developed world: The Sao Paulo-Toronto hemophilia study. Feldman B, Blanchette V, Hilliard P, Young N, Carneiro J, Villaça P. Baxter Healthcare Corporation. $219, US. Oct to Sep Correcting the defect in blood cell formation from Shwachman- Diamond syndrome induced pluripotent stem cells. Dror Y. Butterfly Guild, Seattle Children s Hospital - Research Foundation. $30,000 USD to 2013 Cross-cultural validation of the CHO-KLAT (Canadian Hemophilia Outcomes-Kids Life Assessment Tool). Blanchette V, Young N, McCusker P. Bayer HealthCare Canada. $260,000 CAD to 2012 Cyclosporin pharmacokinetics study and area under the curve (AUC) relationship to graft-versus-host disease (GvHD) in children post bone marrow transplantation. Dupuis L, Schechter- Finkelstein T, Sung L, Pharm D, Fernandez C, Schutze K, Gassas A, Doyle J. C17 Research Network. $199,840 CAD to 2012 Development and evaluation of an internet-based program to promote self-management and transitional care for teens with hemophilia. Breakey V, Stinson J, Blanchette V. Canadian Hemophilia Society, Care Unit Cure Research Program. $75,000 CAD to 2012 Development and testing of a multidimensional electronic pain diary for youths with cancer. Stinson J, Nathan PC, Maloney AM, Gerstle T, Alman B, Hopyan S, Yanofsky R, Portwine C, Johnston D, Strahlendorf C. C17 Research Network. $199,929 CAD. Jul 2010 to Jun 2012 Development and usability testing of an online self-management and transitional care program for youth with cancer. Stinson J, Dettmer E, Dick B, Dupuis F, Gupta A, Laverdiere C, LeMay S, Sung L. Canadian Institutes of Health Research Disease Management Program. $200,000 CAD to 2012 Development and usability of electronic children s international mucositis evaluation scale (e-chimes) for children with cancer. Sung L, Tomlinson D. Garron Family Cancer Centre. $49,905 CAD to 2012 Development of a pediatric cancer-specific symptom assessment screening tool. Sung L, Dupuis LL. Pediatric Oncology Group of Ontario. $14,970 CAD to 2013 Development of international guidelines for the management of pediatric febrile neutropenia. Sung L. Canadian Institutes of Health Research Meeting, Planning and Dissemination Grant: Cancer Research. $12,500 CAD Discovery and characterization of C19MC a novel oncogenic mirna locus in malignant brain tumors. Huang A. Canadian Institute of Health Research. $520,000 CAD to the hospital for sick children department of paediatrics Annual Report

46 DNA copy number variation in Li-Fraumeni Syndrome. Malkin D. Canadian Institutes for Health Research. $784,000 CAD to 2013 Establishing and validating a Shwachman-Diamond syndrome induced pluripotent stem cell model. Dror Y. Donor Directed Research Funds. $30,000 CAD to 2012 Establishment of an international diffuse intrinsic pontine glioma (DIPG) registry. Fouladi M, Van Vuurden GD, Warren K, Savage S, Hawkins C, Bouffet E, Bartels U, Hargrave D, Grill J, Jones C, Baker S, Wesseling P, Jacques T, Varlet P, Vezina G, Young Poussaint T, Leach J, Jones B, Saunders D. Cure Starts Now. $120, to 2013 Ex-vivo expanded hematopoietic progenitors for AML supportive care. Delaney AM, Sung L, Meshinchi S. St. Baldrick s Foundation. $250,000 CAD to 2013 Examining the relationship between white matter integrity and the speed of neuronal processing in children with brain tumours. Mabbott D, Tabori U, Bouffet E. Canadian Institute of Health Research. $640,915 CAD to 2017 Exome sequencing to identify novel genes causing inherited bone marrow failure. Dror Y and 17 Canadian co-investigators. Canadian Pediatric Genetic Disorder Sequencing Consortium. $45,000 CAD to 2012 External validation of ultrasound as a diagnostic tool for assessment of hemophilic arthropathy of knees and ankles. Doria A, Blanchette VS, Carneiro J, D Amico EA, Ribeiro Villaça P, Rodrigues MB, Castro de Ozelo M, de Paula EV, Antunes S, Fernandes A, Moineddin R, Hilliard P. Baxter Healthcare Corporation. $ 203,741 USD. Oct to Sep Functional oncogenomics for the discovery of cancer drivers and unique subclasses (FOCUS). Khokha R, Irwin MS. Ontario Research Fund. $2,182,180 CAD to 2013 Genetic influences on the pharmacokinetics of Factor VIII. Lillicrap D, Blanchette V, Carcao M, Rivard G. Baxter-Canadian Hemophilia Epidemiological Research Program (B-CHERP). $75,000 CAD to 2013 Genome-wide profiling of pediatric diffuse intrinsic pontine glioma. Hawkins C, Bartels U, Stavropoulos J, Bouffet E. National Brain Tumor Society. $198, to 2011 Hematopoietic stem cell transplantation in inherited bone marrow failure syndromes. Cada M, Dror Y. Paediatric Research and Clinical Summer Program: Hospital for Sick Children, Department of Paediatrics and Division of Haematology/ Oncology. $5000 CAD. Apr 2012 Identification of prognostic factors and therapeutic targets in childhood CNS atypical teratoid rhabdoid tumours (ATRT). Lafay-Cousin L, Bouffet E, Hawkins C, Chan J, Huang A. C17 Childhood Cancer and Blood Disorders Research Network. $200,000 CAD to 2013 Identifying of novel inherited bone marrow failure syndromes and genes. Dror Y. March of Dimes. $330,000 CAD to 2012 Improving transition to follow-up care in childhood cancer survivors: development of a questionnaire to measure transition readiness. Barr R, Klassen A, Nathan PC, Greenberg M, Boydell K, D Agostino N, Dettmer E. Hamilton Academic Health Sciences Organization. $133,701 CAD. Apr 2009 to Mar 2012 Integrative genomics for health research Phase II. Scherer S, Malkin D and 8 other co-pis. Canada Foundation for Innovation. $19,989,710 CAD to 2013 International rare histiocytic disorders registry-irhdr. Abla O. Histiocytosis Association of America. $20,000 USD. Dec 2011 In vivo anti-tumor and anti-metastatic activity and functional imaging of Palifosfamide in preclinical neuroblastoma models. Ziopharm/C17. $125,000 CAD to 2013 In vivo anti-tumor and anti-metastatic activity of administration of Abraxane and low-dose chemotherapy in preclinical neuroblastoma and sarcoma mouse model. Baruchel S, Zhang L. Celgene Inc. $107,000 CAD to 2014 Is pre-morbid development vulnerability a risk factor for acute neurotoxicity of adverse neurobehavioral outcomes after treatment for pediatric acute lymphoblastic leukemia? Janzen L, Hitzler J. Canadian Cancer Society/Pediatric Oncology Group of Ontario. $120,864 CAD to 2012 Longitudinal assessment of peripheral neuropathy and motor function in children treated for average risk acute lymphoblastic leukemia. Kadan-Lottick N, Ness K, Hockenberry M, Sung L. Biomarker, Imaging and Quality of Life Studies Funding Program. $1,633, to 2016 Molecular determinants of Li-Fraumeni Syndrome-associated cancers. Malkin D. Canadian Institutes for Health Research. $750,150 CAD to 2015 Molecular targeted therapies of childhood choroid plexus carcinoma. Ellison D, Guy K, Malkin D, Gilbertson R. Department of Defense. $1,750,000 US to 2013 National study of prophylaxis in adolescents and young adults with inherited severe bleeding disorders. Carcao MD, Blanchette VB, Chan A, Teitel J, Lillicrap D, Wu J. Bayer/Canadian Blood Services/Hema-Quebec Partnership Fund. $165,899 CAD to 2012 Neuroblastoma (MIBG) and acute leukemia phase I/II trials programs at the Hospital for Sick Children. Whitlock J, Baruchel S, Irwin M, Hitzler J, Punnett A. Alex s Lemonade Stand Infrastructure Grant. $77,760 CAD to 2013 Neurocognitive-phenome, genome, epigenome and nutriome in childhood leukemia survivors: N-PhenoGENICS. Ito S, Guger S, Hitzler J, O Connor D, Schachar R, Spiegler B, Weksberg R, Carleton B. Canadian Institutes of Health Research. $356,676 CAD. Oct 2011 to Sep 2016 Novel approaches to the prediction, diagnosis and treatment of cardiac late effects in survivors of childhood cancer. Nathan P, Mertens L, Mital S, Kantor P, Greenberg M, Dillenburg R, Liu P, McManus B, Sandor G, Phillips M, Dube M-P, Mandel K, Scherer S, Hodgson D, Manlhiot C, Rosenberg H, Zelcer S, Lougheed J. Canadian Institutes for Health Research. $2,797,748 CAD. Dec 2011 to Dec 2016 Novel imaging techniques for assessment of early cartilage and soft tissue changes in hemophilic ankles. Doria A, Fenster A, Sussman M, Blanchette V. Canadian Hemophilia Society. $149,971 CAD to 2013 NUMED Kids. Targeted radio therapy for treatment of neuroblastoma. Baruchel S, Zhang L. CJ Memorial Fund and The Garron Family Cancer Centre. $96,000 CAD to One retinoblastoma world A global proposal for saving children with retinoblastoma. Gallie B, Chan H. Anna-Liisa and Graham Farquharson Fast Foundation. $125,000 CAD to 2014 One retinoblastoma world A global proposal for saving children with retinoblastoma. Gallie B, Chan H. TUYF Charitable Trust. $1,000, to 2014 Paradigm TM 5: An open-label, single-arm, multinational, non-controlled, confirmatory trial investigating safety, efficacy and pharmacokinetics of NNC (N9-GP) in prophylaxis and treatment of breakthrough bleeding episodes in previously treated children with haemophilia B (12 years of age and younger at inclusion), with extension phase. Carcao M. NovoNordisk. $20,315 US/pt to 2015 Parent and provider perspectives of investigational interventions for fertility preservation in pre-pubertal boys with cancer: Measuring preferences and exploring the factors influencing decisions. Gupta A. Creative Professional Activities Grant - The Hospital for Sick Children. $9,909 CAD. May 2012 to May 2013 Patterns and predictors of health care utilization by adult survivors of childhood cancer: A Pediatric Oncology Group of Ontario and Institute for Clinical Evaluative Sciences population database linkage study. Nathan PC, Greenberg M, Hodgson D, Pole J, Agha M, Guttmann A, Sutradahr R. Canadian Institutes of Health Research. $427,052 CAD. Nov 2009 to Oct 2012 Pediatric Cancer Outcomes Initiative. Greenberg M, POGO, et al. Canadian Cancer Society. $1,000,000 CAD. Apr 2010 to Mar 2013 Pediatric Heart Network The Hospital for Sick Children. McCrindle B, Schwartz S, Mital S, Benson L, Brandao LR, Caldarone C, Chan AK, Gruenwald C, Holtby H, Manlhiot C, Mertens L, Russell J, Sivarajan B, Van Arsdell GS, Sananes R. National Institute of Health. $7,518,965 USD to 2016 Pharmacological predictors of successful cyclosporine acute graft-versus-host disease prophylaxis in children undergoing hematopoietic stem cell transplant. Schechter T, Dupuis LL, Doyle J, Sung L, Seto W, Gassas A. C17. $199,840 CAD to 2012 Pharmacologic reversal of ventricular remodeling in childhood cancer survivors at risk for congestive heart failure (PReVent-CHF). Armenian S, Nathan P, et al. The St. Baldrick s Foundation. $262,088 USD. Jul 2011 to Jun the hospital for sick children department of paediatrics Annual Report

47 Pharmacologic reversal of ventricular remodeling in childhood cancer survivors at risk for congestive heart failure (PReVent- CHF). Armenian S, Nathan P, et al. The Leukemia and Lymphoma Society. $540,054 USD. Oct 2011 to Mar 2014 Phase II study of high dose chemotherapy with cyclosporin for vision-threatening intraocular retinoblastoma. Chan HSL, Gallie BL. Terry Fox Research Institute. $75,908 CAD to 2012 Phase-specific and lifetime medical costs of care for childhood cancer in British Columbia and Ontario. McBride M, Krahn M, De Oliveira C, Greenberg M, Nathan P, Peacock S, Rogers P. Canadian Institutes of Health Research. $533,712 CAD. Dec 2011 to Nov 2014 Physical activity to modify sequelae and QOL in childhood ALL: A nursing trial. Cox C, Nathan PC, Bhatia S, Wasilewski K, Welk G, Ness K, Srivastava DK, Robison L, Howard S, Kaste S. National Institutes of Health/ National Cancer Institute. $4,544,841 USD. Aug 2009 to May 2014 Pilot study of once-a-day prophylaxis for youth and young adults with severe hemophilia. Feldman B, Rivard G. Bayer - Canadian Blood Services - Hema-Quebec Partnership Fund. $151,422 CAD to 2012 Post-marketing observational study on children treated with Exjade. Kirby-Allen M, Odame I. Novartis. $20, to 2011 Predicting late effects of individual cancer survivors. Yasui Y, Greenberg M, Mcbride M, Nathan P. Canadian Institutes of Health Research. $73,430 CAD to 2012 Predicting late effects of individual cancer survivors: A pilot methodological study. Yasui Y, Greenberg M, McBride M, Nathan PC. Canadian Institutes of Health Research. $109,049 CAD. Jul 2010 to Jan 2012 Prediction and prevention of glioma recurrence by targeting telomere dependent self-renewal capacity of tumor initiating cells. Tabori U. Canadian Institutes of Health Research. $919,270 CAD to 2016 Pregnancy complications, perinatal outcomes and use of health care resources in adult survivors of childhood cancer. Nathan PC. Ontario Ministry of Research and Innovation Early Researcher Award. $100,000 CAD. Mar 2010 to Mar 2013 Professional skills development: A program to develop non medical expert CanMEDS roles for Pediatric Hematology- Oncology fellows. Wilejto M, DeSouza C, Punnett A. C17 Education Grant Competition. $3032 CAD to 2012 Prospective analysis of a standardized treatment and follow-up protocol for pediatric non-medullary thyroid cancer. Wasserman J, Gupta A, Hamilton J. Rare Disease Foundation. $3,500 CAD Prospective surveillance of resistance in colonizing bacteria from stool or peri-rectal swab cultures in bacterial isolates from sterile site cultures in pediatric patients receiving levofloxacin for AL or undergoing SickKidsT. Fisher B, Alexander S, Guar A, Zaoutis T, Sung L. Biomarker, Imaging and Quality of Life Studies Funding Program. $913, to 2015 Randomized study of first TIME Immunotolerance Induction in patients with severe type A haemophilia with inhibitor at high risk of failure: Comparison of induction of immune tolerance with FVIII concentrates with or without Von Willebrand factor. Carcao M. City of Hope Medical Centre. 1000/pt to present Rodin: Research of determinants of inhibitor development among previously untreated patients with haemophilia. Carcao M, Blanchette V. University Medical Center Utrecht. 250/ patient to present Secondary prevention among childhood cancer survivors: A quantitative approach to facilitate the effective use of screening. Hodgson D, Cotton C, Hoch J, Nathan PC, Pole J, Sivaloganathan S, Yu E. Pediatric Oncology Group of Ontario. $115,140 CAD. Apr 2011 to Mar 2013 SickKids MIBG and phase I leukemia programs. Whitlock JA. Alex s Lemonade Stand: Foundation for Childhood Cancer. $233,280 CAD. Jul to Jun Silent Infarct Transfusion (SIT) Trial in Patients with Sickle Cell Disease. Kirby M. National Heart, Lung and Blood Institute of NIH. $1,900/patient to 2012 Stratifying and targeting pediatric medulloblastoma through genomics. Malkin D, Taylor M, Marra C. Genome Canada. $9,724,200 CAD to 2014 Stratifying and targeting pediatric medulloblastoma through genomics. Taylor M, Marra M, Malkin D, Marra C, Mabbott D, Hawkins C, Bouffet E, Rutka J, Chan J, Jaboado N, Pfister S, Bader G, Jae C, Scoott P, Clifford S. Canadian Cancer Research Alliance. $9,955,746. Jul 2011 to Jun 2014 Studies of alpha granule formation in human megakaryocytes and platelets. Kahr WHA. Canadian Institutes of Health Research Canada. $689,721 CAD. Apr 2010 to Mar 2015 Studying the blood cell defects in Shwachman-Diamond syndrome using an induced pluripotent stem cell disease model. Dror Y. Butterfly Guild Seattle Children s Hospital and Research Foundation. $30,000 USD to 2012 Targeting the integrin-linked kinase (ILK) in rhabdomyosarcoma. Malkin D. Canadian Institutes for Health Research. $381,270 CAD to 2014 Targeting thrombospondin-1 in medulloblastoma. Huang A, Zadeh G. Canadian Institute of Health Research. $762,000 CAD to 2017 TCD ith Transfusions Changing to Hydroxyurea (TWiTCH): A Phase III randomized clinical trial to compare standard therapy (erythrocyte transfusions) with alternative therapy (hydroxyurea) for the maintenance of lowered TCD velocities in pediatric subjects with sickle cell anemia and abnormal pre-treatment TCD velocities. Kirby M, Williams S, Odame I. National Heart, Lung, and Blood Institute (Amount under negotiation) to 2014 Terry Fox New Investigator award - Exhaustion of tumor initiating cells by targeting their self-renewal capacity with telomerase inhibition. Tabori U. The Terry Fox Foundation. $450,000 CAD to 2013 The Canadian Pediatric Cancer Genome Consortium: Translating next-generation sequencing technologies into improved therapies for high-risk childhood cancer. Sorensen P, Malkin D, Taylor M, Huang A, Jabado N, Hawkins C, Sinnet D, Fernandez C. CIHR-Genome Canada. $2,700,000 CAD to 2012 The role of dysfunctional central venous catheters as a predictor of thrombotic events and/or infections in pediatric cancer patients. Athale U, Brandão LR, Halton J, Gibson P, Silva M. C17 Paediatric Haematology/Oncology Centres in Canada. $150,000 CAD. Jul to Jun The utility of serum specific lung biomarkers as indicators of bronchiolitis obliterans in children receiving allogeneic hematopoietic stem cell transplantation. Gassas A, Sung L, Doyle JJ, Finkelstein-Schechter T, Dell S, Subbarao P, Palaniyar N. Paediatric Oncology Group of Ontario. $14,995 CAD to 2012 The Von Willebrand Disease (VWD) international prophylaxis (VIP) study. Carcao M. Rho Inc. $2,500 US/pt to present Treatment choices for children with typical acute immune thrombocytopenic purpura: Development of a decision aid. Beck C, Parkin P, Birken C, Blanchette V, Boydell K, Llewellyn-Thomas H. Dean s Fund New Staff Competition, University of Toronto. $10,000 CAD to 2011 Tumour-associated macrophages as predictors of survival in paediatric hodgkins lymphoma. Gupta S, Punnett A, Chung C. Physician Services Inc. $13, to 2012 Understanding body image, dating relationships and sexuality in adolescents with cancer. Stinson J, Gupta A, Marrera B, Greenberg M. Pediatric Oncology Group of Ontario Seed Funding Grant. $14,963 CAD Up regulation of Epstein-Barr virus activity by plasmodium falciparum: Determinants of host control. Allen U, Weitzman S. Canadian Institutes of Health Research. $700,000 CAD to 2014 Using the zebrafish as a pre-clinical animal tumour model for drug discovery and the evaluation of anti-leukemia agents. Dellaire GP, Berman J, Chiu P, Ross C, Whitlock J. Canadian Institutes of Health Research $400,000 CAD to 2015 VWD plasma and platelets: Functional characterization of quantitative and qualitative VWF mutations. Kahr WHA, James P, Robinson S. Canadian Hemophilia Society $150,000 CAD. Apr 2011 to Mar What factors do children with cancer and childhood cancer survivors say are important to understanding their quality of life? A qualitative study. Klaasen A, Anthony S, Dix D, Klaassen R, Sung L. C17 Children s Cancer and Blood Disorders $156,093 CAD to the hospital for sick children department of paediatrics Annual Report

48 IMMUNOLOGY AND ALLERGY overview Division Head Dr. Eyal Grunebaum Full-Time Staff Physicians Dr. Adelle Atkinson Dr. Chaim Roifman Dr. Julia Upton Clinical Assistants Dr. Vy Kim Part-Time Staff Physicians Dr. Maria Asper Dr. Stephen Feanny Dr. David Hummel Dr. Sasson Lavi Clinical Fellows Dr. Nufar Marcus Dr. Luis Murguia Subspeciality Residents Dr. Amy French Dr. Joyce Ching Dr. Rae Koffman Dr. Reem Mohammed Dr. Caroline McLaughlin Dr. Anastasios Papadopoulos Dr. Audrey Segal Immunology and Allergy Nurse Anna Kasprzak, RN Brenda Reid, CNS Senior Scientist-Emeritus Amos Cohen, PhD Research Associate Nigel Sharfe, PhD Research Technologist Lorand Cimpeon Tom Grunberger Ariana Karanxha Weixian Min Murugesapillai Mylvaganam Medical Lab Technologis Harjit Dadi, PhD Andrea Newell Administrative Staff Sandra Mendonca Carrie Smith Canadian Centre of PID and JMF Centres Manager Linda Pires Dr. Grunebaum was appointed Head of the Division of Immunology and Allergy in May 2012, replacing Dr. Chaim M. Roifman who led the division for more than 20 years. During this period, the Division had become a world-leading centre for the diagnosis, understanding and management of primary immune deficiencies as well as some of the complex allergic conditions that affect children. The SickKids PID Program is the largest of its kind in Canada and is considered to be one of the largest in the world. The Primary Immunodeficiency (PID) Program in the Division of Immunology and Allergy at The Hospital for Sick Children is the largest of its kind in Canada and is considered to be one of the largest in the world. Medical services are provided to more than 2000 outpatients, referred from physicians and hospitals locally, nationally and internationally. The number of referred patients has steadily increased by 30 per cent over the last decade. This year the division expanded its consultation and management services for children and adults with primary immune deficiencies to 10 clinics per month. Some clinics focus on caring for patients with complex immune abnormalities and genetic defects, while other clinics are dedicated to the short- and long- term management of patients following bone marrow transplantation or gene therapy for severe inherited immune deficiencies. The Division is also responsible for the Immunology Isolation Unit, where on average 5-10 patients with severe immune defects receive haematopoietic stem cell transplantation every year. The innovative efforts and experience of our division in transplantation for patients with PID was recently summarized (Grunebaum and Roifman. Hematology/Oncology Clinics of North America). In members of the Division made major contributions to the advancement of the diagnosis and management of PID. We provided the first definition of combined immune deficiency (Roifman et al., JACI 2012), which is crucial for further understanding and managing patients with varying severities of inherited immune defects (Modell et al., Immunological research 2011). In addition, Dr. Roifman has led a revolutionary Canadian neonatal screening initiative for the early diagnosis of severe combined immunodeficiency, a program that will clearly save lives of many infants. Indeed, we have shown that early diagnosis and proper supportive management of department of paediatrics Annual Report

49 patients with SCID lead to improved outcome (Barron et al., J. Peds 2011 and Nicholas et al., Clin Immuno 2011) while delay in diagnosis is associated with devastating consequences (Newell et al., JACI 2011). The Hypogammaglobulinemia Program at SickKids is recognized globally for its innovative approach and long-term follow-up of over 200 patients. We transformed the therapy of these patients from multiple hospital-based admissions, to visiting the short-stay unit and subsequently receiving treatment in their community. The proficiency of our team coupled with convenient care at a medical centre close to home, provides our patients with the personalized treatment they require. To further enhance this program, implementation of a home therapy program is underway. Funding by the Canadian Immunodeficiency Society (CI Society) has permitted the completion of a provincial patient survey to assess such a model here in Ontario. Final analysis is underway and a clinical trial is under development. As a leader in immunoglobulin replacement therapy, Dr. Roifman acted as the lead investigator in multiple international clinical trials (Borte M, et al., Clin Exp Immunol 2011). Currently members of the division are involved in an international study assessing the safety and efficacy of a new brand of immunoglobulin (IVIg-SN-TM) and next year we plan to study a novel concept of immunoglobulin administration combined with hyaluronidase. The Allergy Program in our Division is a referral-based clinic for the diagnosis and management of children with complex allergic diseases. These conditions include multiple or difficultto-manage drug allergies, latex allergy, anaphylaxis, food challenges, urticaria and asthma. More than 1,000 patients with complex allergic diseases are seen each year in the Division s clinics and similar numbers of patients are referred to the Division s community affiliated clinics. The allergy program also offers inpatient consultation for allergic diseases, with specific expertise in adverse reactions to drugs and biologics in medically complex children, including those suffering from cancer or receiving blood and organ transplants. For children with complex medical conditions suffering also from adverse drug reactions, the DART (Drug, Adverse Reaction and Toxicology Clinic) clinic provides targeted diagnostic and management strategies, while collecting important data about this population. This clinic has demonstrated significant clinical impact in this vulnerable population. Importantly, with the increasing frequency of influenza epidemics, the allergy team at The Hospital for Sick Children demonstrated that most egg-allergic children can safely receive influenza vaccines, even when they suffer from severe co-morbidities (Upton et al., Allergy Asthma Clin Immunol, 2012). Additionally, with the growing prevalence of life threatening food allergy in our community, the Division is establishing a program dedicated to food allergy and is in the process of recruiting a leader in this field. Education remains a major focus for every member of the Division. In September 2011, Dr. Grunebaum was appointed as Director of The Pediatric Immunology and Allergy Training program - Royal College stream, while Dr. Roifman continues to head the Advanced Immunology Fellowship Program. Importantly, the training program has had a flawless certification record by the Royal College of Physician and Surgeons of Canada since Our program provides exceptional training in clinical immunology and allergy as well as research and laboratory immunology. By preparing outstanding academic and non-academic specialists in immunology and allergy, we continue to attract brilliant candidates from Canada and across the world. We underwent an internal review of the program and achieved full marks and we are now preparing for the Royal College accreditation expected in To accommodate these additional The Division had become a world-leading centre for the diagnosis, understanding and management of primary immune deficiencies. national and international fellows, The CI Society-Talecris (now Grifols) Fellowship Program has supported our Division for over a decade. These trainees have the opportunity to participate in a flexible program which can be predominantly research oriented. Our commitment to education far exceeds these training programs. As the Chair of the Jeffrey Modell Centers Network, Dr. Chaim Roifman established The Robert A. Good/Jeffrey Modell Fellowship in Immunodeficiency and Transplantation. This international program began in 2009 and provides matching funds for several immunology fellowship positions each year globally. In addition the Jeffrey Modell Canadian Immunodeficiency Network implemented PID video conference rounds. The rounds are accredited by the Royal College and held quarterly. Hosted by SickKids, the seminars link immunologists and trainees across Canada to discuss complex cases. Participation is accessible through telehealth video conferencing and through webcasting, with an average of 100 participants and 12 national sites and five observing sites. Dr. Roifman research team has recently completed studies exploring the role of Lyp phosphatase, (a molecule that the lab had previously originally cloned and sequenced) in the normal immune system, autoimmunity and immunodeficiency. The studies by Dr. Roifman s group illustrated subcellular localization of and its possible novel role in the nucleus and have defined the role of a lyp genetic variant in chronic mucocutaneous candidiasis (JACI, 2011). Moreover, the research linked a genetic variant of TLR3 with a subgroup of patients with this disorder (Nahum et al., Autoimmun Rev. 2012). In several international collaborative studies, Dr. Roifman also helped identify Tartarate-resistant acid phosphatise mutations in Roifman-Costa syndrome (Briggs et al., Nat Genet. 2011), mutations in the IL-10R gene as a cause for very-early-onset ulcerative colitis (Moran et al., Inflamm Bowel Dis. 2012) and various genetic defects in patients with inherited bone marrow failure syndromes (Tsangaris E, et al. J Med Genet. 2011). Dr. Roifman also led a multicentre study defining the best treatment for CD3δ deficiency (Marcus et al., JACI, 2011) and a study which characterized failure to thrive in patients with severe combined immunodeficiency (Barron et al., J Pediatrics, 2011). With such life altering advancements in the care and quality of life of patients, Dr. Chaim Roifman, continues to remain a member of the IUIS committee for primary immunodeficiency (previously WHO) which leads the field of PID (Al-Herz W, et al. Frontiers in Immunology 2011). He was also invited to write a chapter on chronic mucocutaneous candidiasis in the prestigious UpToDate series (Roifman. UpToDate, 2011). Under his guidance, The Jeffrey Modell Research Laboratory for the Diagnosis of PID (Canadian Centre for PID) continues to thrive. Dr. Eyal Grunebaum s laboratory further advanced the understanding of the mechanisms and treatment for inherited purine defects, which are amongst the most common causes for severe immune deficiency in children. Following the identification of a severe lung abnormality unique to patients with adenosine deaminase (ADA) deficiency (Grunebaum et al., JACI 2012), Dr. Grunebaum s lab has begun elucidating some of the mechanisms for these abnormalities as well as the optimal management options. Dr. Grunebaum s group provided some alarming information about outcome of patients who received gene therapy for adenosine deaminase (ADA) deficiency. The incomplete immune reconstitution and the persistent abnormalities in purine metabolisms following gene therapy seemed to predispose patients to tumour development and could also contribute to increased long-term susceptibility to infections and autoimmunity in these patients (Grunebaum et al., JACI, 2011). In contrast, Dr. Grunebaum s lab, with a grant that they received in 2011 from the Canadian Immunodeficiency Society, were able to develop an ADA protein replacement therapy that could prevent the early lung and immune abnormalities in ADA-deficient mice. Moreover, with the support of the Fred Rosen/Jeffrey Modell/NIH research grant, Dr. Grunebaum s team showed that delivering normal PNP protein into thymocytes could prevent the apoptosis of these cells in purine nucleoside phosphorylase (PNP) deficiency and corrected the immune abnormalities associated with this disease (Papinazath et al., JACI, 2011). Importantly, early initiation of this treatment also prevented the development of neurological abnormalities associated with PNP deficiency (Mansouri et al., Neurobiology of Dis 2011). The ADA and PNP novel enzyme replacement treatments options for purine defects developed by Dr. Grunebaum s team are attracting increasing international recognition, with invitations for presentations at prestigious international conferences such as the JMF First World Immunology Conference held at Rockefeller University, NY, and The Editors Choice in the Journal of Allergy and Clinical Immunology. Dr. Adelle Atkinson is in her seventh year as Director of Postgraduate Medical Education in the Department of Paediatrics. She continues to excel as an outstanding educator, and the training program continues to attract the brightest stars. In recognition of her achievements, Dr. Atkinson received the Sarita Verma award for mentorship and advocacy in the spring of Over the last year she has given invited presentations at international conferences in the area of postgraduate medical education and faculty development including topics such as residency selection, professionalism, clinical teaching and feedback. Dr. Atkinson was also involved in Undergraduate Medical Education Accreditation in 2012, and is preparing the Department of Paediatrics Postgraduate medical education for the 2013 Royal college accreditation. In the last year Dr. Atkinson has also led a Department of Paediatrics initiative to attract and retain paediatric trainees who would be our future clinician scientists. In addition to the extensive educational commitments, Dr. Atkinson runs a novel clinic studying drug allergies in medically complex patients, providing an important clinical service to this tertiary care population. Brenda Reid is Clinical Nurse Specialist in the Division. She has been instrumental in providing leadership to nurses across the country in the management of primary immunodeficient patients. Through the CI Society, she formed a network of nurses across the country and has developed a nursing section for their website. Additionally, she has contributed enormously to the research and education generated by our division. 96 the hospital for sick children department of paediatrics Annual Report

50 Publications Al-Herz W, Bousfiha A, Cassanova JL, Chapel H, Conley ME, Cunningham-Rundles C, Etzioni A, Fischer A, Franco JL, Geha RS, Hammarstrom L, Nonoyama S, Notarangelo LD, Ochs HD, Puck JM, Roifman CM, Seger R, Tang M. Primary immunodeficiency diseases: An update from the International Union of Immunological Societies Primary Immunodeficiency Classification Committee. Frontiers in Immunology 2011: E Pub. Grunebaum E, Cutz E, Roifman CM. Pulmonary alveolar proteinosis in patients with adenosine deaminase deficiency. Journal of Allergy and Clinical Immunology 2012: 129: pp Mansouri A, Min W, Cole CJ, Josselyn SA, Henderson JT, vaneede M, Henkelman RM, Ackerley C, Grunebaum E, Roifman CM. Cerebellar abnormalities in purine nucleoside phosphorylase deficient mice. Neurobiology Diseases 2012: 47(2): pp Epub April 1. PubMed PMID: Marcus N, Takada H, Law J, Cowan MJ, Gil J, Regueiro JR, Plaza Lopez de Sabando D, Lopez-Granados E, Dalal J, Friedrich W, Manfred H, Hanson IC, Grunebaum E, Shearer WT, Roifman CM. Hematopoietic stem cell transplantation for CD3δ deficiency. Journal of Allergy and Clinical Immunology 2011: 128(5): pp Modell V, Gee B, Lewis DB, Orange JS, Roifman CM, Routes JM, Sorenson RU, Notarangelo LD, Modell F. Global study of primary immunodeficiency diseases (PI). Diagnosis, treatment and economic impact: An updated report from the Jeffrey Modell Foundation. Immunologic Research 2011: 51(1): pp Moran CJ, Guo C-H, Kugathasan S, Klein C, Wolters VM, Walters T, NEOPICS, Bandsma RH, Mouzaki M, Langer JC, Cutz E, Benseler SM, Roifman CM, Silverberg MS, Griffiths AM, Snapper SB, Muise AM. IL-10R polymorphisms associate with very early-onset ulcerative colitis. GUT 2012: E Pub. Nahum A, Dadi H, Bates A, Roifman CM. The biological significance of TLR3 variant, L412F, in conferring susceptibility to cutaneous candidiasis, CMV and autoimmunity. Autoimmunity Reviews 2011: Epub. Papinazath T, Min W, Suntharalingam S, Cohen A, Roifman CM, Grunebaum E. The role of purine nucleoside phosphorylase in thymocyte development. Journal of Allergy and Clinical Immunology 2011: 128(4): pp (chosen by Editors as issue s Feature Manuscript). Roifman CM, Somech R, Kavadas F, Pires L, Nahum A, Dalal I, Grunebaum E. Defining combined immunodeficiency. Journal of Allergy and Clinical Immunology 2012: Epub Jun 2. pubmed PMID: Upton J, Hummel D, Kasprzak A, Atkinson A. No systemic reactions to influenza vaccination in egg-sensitized tertiary-care pediatric patients: A review of our protocol. Allergy, Asthma and Clinical Immunology 2012: 8: pp 2. Books and Chapters French A, Atkinson AR. Anaphylaxis Code Card The Hospital for Sick Children, Grants and Funding An open label, single arm, historically controlled, prospective, multicenter Phase III study to evaluate the safety, efficacy and pharmacokinetics of immune globulin intravenous IVIG-SN-T. Roifman CM, Grunebaum E, Vy K, Reid B, Kasprzak A. Atlantic Research Group - Greencross study. $100, to 2012 CI Society Talecris Treatment and Bone Marrow Transplant Centre for primary immunodeficiency. Roifman CM. Canadian Immunodeficiency Society. $150, to 2012 Contribution of T cell dysregulation to the development of food allergy. Development of an animal model. Grunebaum E. Sickkids Foundation. $15, to 2012 Determinanants of the microbiome in IBD by the Harvard/Broad Institute - Toronto IBD Consortium. Snapper, Scott, Notarangelo, Geha, Muise et al. Crohn s and Colitis Foundation America with support from the Helmsley Foundations. $500, to 2014 Development of therapeutic inhibitors for treatment of multiple myeloma. Roifman CM. Multiple Myeloma Research Foundation (MMRF). $200, to 2012 Epstein-Barr virus in health and disease in children and youth: A prospective study of markers of virus-host interaction and genetic variation. Allen U, Beyenne J, Grunebaum E, Mazzulli T, Read S, Robinson J, Tellier R, Weitzman S, Zielenska M. CIHR. $398, to 2012 Home immunoglobulin therapy. Roifman CM. Canadian Immunodeficiency Society. $430, to 2011 Immune and non-immune abnormalities associated with PNP. Roifman CM, Grunebaum E. Jeffrey Modell Centres Network National Institute of Health. $250, to Improving the management of patients with severe inherited immune deficiencies. Grunebaum E. Canadian immunodeficiency Society (CI Society). $40, to 2012 Immune and non-immune abnormalities associated with purine nucleoside phosphorylase deficiency. Grunebaum E, Roifman CM. Jeffrey Modell Foundation and the National Institute of Health and the National Institute of Allergy and Infectious Diseases. $250, to Optimizing hematopoietic stem cell transplantation - The cord advantage. Grunebaum E. Elizabeth Rose Herman Endowment Fund. $29, to 2011 PNP deficiency - A multicenter study. Grunebaum E. Jeffrey Modell Foundation $60, to the hospital for sick children department of paediatrics Annual Report

51 INFECTIOUS DISEASES overview Division Head Dr. Upton Allen Full-Time Dr. Ari Bitnun Dr. Shaun Morris Dr. Michelle Science Dr. Dat Tran Dr. Anu Wadhwa (Training Programme Director) Dr. Valerie Waters Part-Time Dr. Stan Read Dr. Ian Kitai Dr. Jonathan Gubbay Cross-Appointees Dr. Anne Matlow Dr. Anna Banerji Research Fellows and Associates Dr. Sandra Asner Dr. Vicky Fera Dr. Michael Hawkes Dr. Nisha Thampi Clinical Fellows Dr. Rabee Adwan Dr. Hilal Al-Hashami Dr. Ahmed Al-Jarie Dr. Ohoud Al-Yabes Dr. Aparna Chakravarty Dr. Faisal Kordy Subspecialty Residents Dr. Sarah Khan Dr. Kevin Schwartz Administrative Staff Linda Martin Brenda Bishop Erin Manych Louisa Matela Honorary Status Dr. Elaine Wang Dr. Jason Brophy The Division of Infectious Diseases is a leader in the academic practice of infectious diseases nationally and internationally. The Division has active inpatient as well as ambulatory consultant services that include teams that focus on general infectious diseases and infectious diseases in immunocompromised infants, children and youth. We are a resource for issues that relate to infectious disease outbreaks, including new and emerging infections, paediatric infection control and the specialized areas of HIV, congenital infections, management of the infectious complications of organ transplantation, and the ever-increasing problem of multi-drug resistant tuberculosis. Division members have leading roles in national and international epidemiological, clinical, research advisory and related committees (e.g., the Infectious Diseases Society of America, the Canadian Paediatric Society, Health Canada, the American Society for Transplantation, the International Pediatric Transplant Association, the Canadian AIDS Research Group and the Government of the Bahamas). We are a resource for issues that relate to infectious disease outbreaks Our research initiatives are structured around themes that include host susceptibility and related factors and innovative antimicrobial and associated therapies. Specific research initiatives include multicentered paediatric HIV studies that relate to maternal-to-child transmission and studies on new antiretrovirals, vaccine studies and research into the mechanisms of host susceptibility to viral infections. These infections include EBV-related post-transplant lymphoproliferative disorder (PTLD), influenza illness, infections in patients with cystic fibrosis, HIV, and encephalitis. Multicentred trials related to the diagnosis and prevention of infections in transplant patients are ongoing, as well as participation in national surveillance studies (IMPACT) and national collaborative paediatric studies through the Paediatric Investigators Collaborative Network on Infections in Canada (PICNIC). department of paediatrics Annual Report

52 The Division continued to have a leadership role in structuring the approach to the management of major emerging and resurgent infectious disease threats. In this regard, the Division has been actively involved in influenza pandemic preparedness efforts at SickKids as well as provincially and nationally. Indeed, members of the Division had pivotal local, provincial, national and international leadership roles in the H1N1 pandemic of We have one of the largest and most well-established paediatric infectious diseases training programmes internationally. Representatives from our Division continue to play a key role in activities of the Royal College of Physicians and Surgeons of Canada, and in many areas of undergraduate, graduate, postgraduate and continuing medical education activities. Faculty members continue to hold editorships, visiting professorships and related academic honours nationally and internationally. Honors and Awards Matlow, A: Winner of Colin R Woolf Award for Excellence in Course Coordination, collaborator in curriculum development and coordination. Continuing Education, Faculty of Medicine, Centre for Patient Safety, University of Toronto, Toronto Ontario Canada (Continuing Education, 2012) Patient Safety and Quality Improvement Certificate Course. 2011/07 to 2012/06 Publications Achonu C, Rosella L, Gubbay JB, Deeks S, Rebbapragada A, Mazzulli T, Willison D, Foisy J, McGeer A, Johnson I, Lafreniere M, Johnson C, Willmore J, Yue C, Crowcroft NS. Seroprevalence of pandemic influenza H1N1 in Ontario from January May Public Library of Science One 2011: 6 (11):e Epub 2011 Nov 14. Al-Dabbagh M, Lapprha K, Pattar R, Inrig K, Sauve L, Schaaf SH, Marais BJ, Kitai I, Kollmann RR. Drug-resistant tuberculosis: A review of pediatric treatment guidelines. Pediatric Infectious Diseases Journal 2011: 30: pp Allen U, Green M. The menace of CMV disease after small bowel transplantation: Bearer of bad news! Pediatric Transplantation 2012: 16 (6): pp Allen U, Michaels MG. Fungal prophylaxis after pediatric organ transplantation: To be or not to be that is the question. Pediatric Transplantation 2011: 15 (5): pp Allen U, Preiksaitis J. Epstein-Barr virus and post-transplant lymphoproliferative disorder. Guidelines for publication. American Journal of Transplantation 2012: 12 (S3) online April Amin R, Waters V. Antibiotic treatment for stenotrophomonas maltophilia in people with cystic fibrosis. Cochrane Database of Systematic Reviews (online) 2012: 16: 5: CD Asner S, Peci A, Marchand-Austin A, Winter AL, Olsha R, Kristjanson E, Low DE, Gubbay JB. Respiratory infections in institutions from late stage of the first and second waves of pandemic (H1N1) 2009, Ontario, Canada. Influenza and Other Respiratory Viruses 2012: Feb 21. doi: /j x. (Epub ahead of print). Asner S, Waters V, Solomon M, Yau Y, Richardson S, Grasemann H, Gharabaghi F, Tran D. Role of respiratory viruses in pulmonary exacerbations in children with cystic fibrosis. Journal of Cystic Fibrosis 2012: May 10. (Epub ahead of print) Baluch A, Humar A, Egli A, Gubbay J, Lisboa L, Wilson L, Kumar D. Long term immune responses to pandemic influenza A/H1N1 infection in solid organ transplant recipients. Public Library of Science One 2011: 6 (12):e Epub 2011 Dec 14. Bastien N, Gubbay JB, Richardson D, Sleeman K, Gubareva L, Li Y. Detection of an influenza B strain with reduced susceptibility to neuraminidase inhibitor drugs. Journal of Clinical Microbiology 2011: 49 (11): pp Epub 2011 Sep 7. Coleman BL, Kuster SP, Gubbay J, Scheifele D, Li Y, Low D, Crowcroft N, Mazzulli T, Shi L, Halperin SA, Law B, McGeer A. Immunogenicity of a half-dose of adjuvanted 2009 pandemic H1N1 influenza vaccine in adults: A prospective cohort study. European Journal of Clinical Microbiology and Infectious Disease 2012: 31 (4) pp Epub 2011 Jul 28. Eshaghi A, Duvvuri VR, Lai R, Nadarajah JT, Li A, Patel SN, Low DE, Gubbay JB. Genetic variability of human respiratory syncytial virus A strains circulating in Ontario: A novel genotype with a 72 nucleotide G gene duplication. Public Library of Science One 2012: 7 (3): e doi: /journal.pone Eshaghi A, Patel SN, Sarabia A, Higgins RR, Savchenko A, Stojios PJ, Li Y, Bastien N, Alexander DC, Low DE, Gubbay JB. Multidrug-resistant pandemic (H1N1) 2009 infection in immunocompetent child. Emerging Infectious Diseases 2011:17 (8): pp Etchells E, Koo M, Daneman N, McDonald A, Baker M, Matlow A, Krahn M, Mittmann N. Comparative economic analyses of patient safety improvement strategies in acute care: A systematic review. British Medical Journal: Quality and Safety 2012: 21 (6): pp Ethier MC, Science M, Beyene J, Briel M, Lehrnbecher T, Sung L. Mould-active compared to fluconazole prophylaxis to prevent invasive fungal diseases in cancer patients receiving chemotherapy or haematopoietic stem-cell transplantation: A systematic review and meta-analysis of randomized controlled trials. British Journal of Cancer 2012:106 (10): pp Forbes JC, Alimenti AM, Singer J, Brophy JC, Bitnun A, Samson LM, Money DM, Lee TCK, Lapointe ND, Read SE, the Canadian Pediatric AIDS Research Group (CPARG). (Allen U and Read S are members). A national review of vertical HIV transmission. AIDS 2012: 26 (6): pp Frieling ML, Williams A, Al Shareef T, Kala G, Teh JC, Langlois V, Allen U, Hebert D, Robinson LA. Novel influenza (H1N1) infection in pediatric renal transplant recipients: A single centre experience. Pediatric Transplantation 2012: 16 (2): pp Greenaway C, Sandoe A, Vissandjee B, Kitai I, Gruner D, Wobeser W, Pottie K, Ueffing E, Menzies D, Schwartzman K, Canadian Collaboration for Immigrant Health. Tuberculosis: Evidence review for newly arriving immigrants and refugees. Canadian Medical Association Journal 2011: 183 (12) pp E939-E951. DOI /cmaj Gubbay JB, Al-Rezqi, Hawkes M, Williams L, Richardson S, Matlow A. The role of torovirus in nosocomial viral gastroenteritis at a large tertiary pediatric centre. Canadian Journal of Infectious Diseases and Medical Microbiology 2012: 23(2) pp Hsiao M, Morris SK, Bassani DG, Montgomery AL, Thakur JS, Jha P. Factors associated with physician agreement on verbal autopsy over injury deaths in India. Public Library of Science One 2012: 7 (1) e (Epub 2012 Jan 176). Hui CP, MacDonald NE, Canadian Paediatric Society, Infectious Diseases and Immunization Committee (Allen U is a committee member). Use of influenza vaccines in children with an egg allergy. Paediatrics and Child Health 2011: 16 (8): pp Kakalia S, Sochett E B, Stephens D, Assor E, Read S, Bitnun A. Vitamin D supplementation and CD4 count in children infected with human immunodeficiency virus. Journal of Pediatrics 2011: 159 (6): pp Kellner JD, The Canadian Paediatric Society, Infectious Diseases and Immunization Committee (Allen U is a committee member). Update on the success of the pneumococcal conjugate vaccine. Paediatrics and Child Health 2011: 16 (4): pp Kumar D, Blumberg EA, Danziger-Isakov L, Kotton CN, Halasa NB, Ison MG, Avery RK, Green M, Allen U, Edwards KA, Miller G, Michaels MG, AST Infectious Disease Community Practice. Influenza vaccination in the organ transplant recipient: Review and summary recommendations. American Journal of Transplantation 2011: 11(10): pp Kuster SP, Katz KC, Blair J, Downey J, Drews SJ, Finkelstein S, Fowler R, Green K, Gubbay J, Hassan K, Lapinsky SE, Mazzulli T, McRitchie D, Pataki J, Plevneshi A, Powis J, Rose D, Sarabia A, Simone C, McGeer A. When should a diagnosis of influenza be considered in adults requiring intensive care unit admission. Results of population-based active surveillance in Toronto. Critical Care 2011: 15:R Kuster SP, Tuite AR, Kwong JC, McGeer A, Fisman DN, Toronto Invasive Bacterial Disease Network Investigators (Kitai I is a member). Evaluation of co-seasonality of influenza and invasive pneumococcal disease: Results from prospective surveillance. Public Library of Science Medicine 2011: (6): pp e the hospital for sick children department of paediatrics Annual Report

53 Le Saux N, Bettinger J, Halperin S, Vaudry W, Scheifele D, Canadian Immunization Monitoring Program (Tran D is a member of the program). Hospital acquired rotavirus infections: Burden in Canadian paediatric hospital. Journal of Infection Prevention (4): pp Moore DL, Bortolussi R, Canadian Pediatric Society, Infectious Diseases and Immunization Committee (Allen U is a committee member). Home intravenous therapy: Accessibility for Canadian children and youth. Paediatrics and Child Health 2011: 16 (2): pp Robinson JL, Salvadori MI, Canadian Pediatric Society, Infectious Diseases and Immunization Committee (Allen U is a committee member). Management of community-associated methicillin-resistant staphylococcus aureus skin abscesses in children. Paediatrics and Child Health 2011: 16 (2): pp Books and Chapters Allen U. Clinical infectious disease problems in pediatric transplant patients. In: 2012 Annual Update for ID Staff and Trainees. Hospital for Sick Children, Le Saux N, Canadian Paediatric Society, Infectious Diseases and Immunization Committee (Allen U is a committee member). Biologic response modifiers to decrease inflammation: Focus on infection risks. Paediatrics and Child Health 2012:17 (3): pp Morris SK, Bassani DG, Awasthi S, Kumar R, Shet A, Suraweera W, Jha P. Diarrhea, pneumonia, and infectious disease mortality in children aged 5 to 14 years in India. Public Library of Science One 2011: 6 (5): e Salvadori MI, Bortolussi R, Canadian Pediatric Society, Infectious Diseases and Immunization Committee (Allen U is a committee member). Meningococcal vaccines in Canada: An update. Paediatrics and Child Health 2011: 16 (8): pp Renwick R, Salter R, King S, Read S. Children with HIV and their families. In: Developmental Disabilities in Ontario (2011),Third edition. Ontario Association on Developmental Disabilities. Le Saux N, Robinson JL, Canadian Paediatric Society, Infectious Diseases and Immunization Committee (Allen U is a committee member). Pneumonia in healthy Canadian children and youth: Practice points for management. Paediatrics and Child Health 2011: 16 (7): pp MacDonald NE, Bortolussi R, Canadian Paediatric Society, Immunization and Infectious Diseases Committee (Allen U is a committee member). A harmonized immunization schedule for Canada: A call to action. Paediatrics and Child Health 2011:16 (1): pp Mark S, Murphy KE, Read S, Bitnun A, Yudin MH. HIV mother-to child transmission, mode of delivery and duration of rupture of membranes: Experience in the current era. Infectious Diseases in Obstetrics and Gynecology 2012: Article ID Matlow A, Forgie S, Pelude L, Embree J, Gravel D, Langley JM, Saux NL, Moore D, Mounchili A, Mulvey M, Shurgold J, Simor AE, Thomas E, Vayalumkal J, The Canadian Nosocomial Infection Surveillance Program. National surveillance of methicillin-resistant staphylococcus among hospitalized pediatric patients in Canadian acute care facilities, Pediatric Infectious Disease Journal 2012: 31(8):pp Mitchell I, Paes BA, Li A, Lanctot K, CARESS Investigators (Allen U is a member). CARESS: The Canadian Registry of palivizumab. Pediatric Infectious Disease Journal 2011: 30 (8): pp Montgomery AL, Morris SK, Bassani DG, Kumar R, Jotkar R, Jha P. Factors associated with physician agreement and coding of cause of death using verbal autopsies for 1130 maternal deaths in India. Public Library of Science One (3): pp e DOI: /journal.pone Morris SK, Science M, Subbarao P, Parkin P, Yau Y, Allen U, Tran D. A retrospective cross- sectional study of risk factors and clinical spectrum of children admitted to hospital with pandemic H1N1 influenza as compared to influenza A. British Medical Journal Open 2012: (2) : e doi: /bmjopen : pp 1-8. Paquette SG, Banner D, Zhao Z, Fang Y, Huang SSH, Leon AJ, Ng DCK, Almansa R, Martin-Leoches Ramirez P, Socias L, Loza A, Blanco J, Sansonetti P, Rello J, Andaluz D, Shum B, Rubino S, de Lejarazu RO, Tran D, Delogu G, Fadda G, Krajden S, Rubin BB, Bermejo-Martin JF, Kelvin AA, Kelvin DJ. Interleukin-6 is a potential biomarker for severe pandemic H1N1 influenza A infection. Public Library of Science One 7 (6): pp e DOI: /journal.pone Patel SN, McGeer A, Melano R, Tyrell GJ, Green K, Pillai D, Low DE, Canadian Bacterial Surveillance Network (Kitai I is a member). Steptococcus pneumonia to fluoroquinolones in Canada. Antimicrobial Agents Chemotherapy 2011: 55 (8): pp Pottie K, Greenaway C, Feightner J, Canadian Collaboration for Immigrant and Refugee Health Committee (Kitai I is a member). Evidence-based clinical guidelines for immigrants and refugees. Canadian Medical Association Journal 2011:183 (12): pp E DOI: /CMAJ Robichaud P, Saari M, Burnham E, Omar S, Wray RD, Baker R, Matlow AG. The value of a quality improvement project in promoting interprofessional collaboration. Journal of Interprofessional Care 2012: 26(2): pp Robinson JL, Canadian Pediatric Society, Infectious Diseases and Immunization Committee (Allen U is a committee member). Preventing respiratory syncytial virus infections. Paediatrics and Child Health 2011: 16 (8): pp Salvadori MI, Canadian Pediatric Society, Infectious Diseases and Immunization Committee (Allen U is a committee member). Influenza vaccine recommendations for children and youth for the 2011/2012 season. Paediatrics and Child Health 2011: 16 (9): pp Salvadori MI, Canadian Pediatric Society, Infectious Diseases and Immunization Committee (Allen U is a committee member). Preventing varicella: Recommendations for routine two-dose varicella immunization in children. Paediatrics and Child Health 2011: 16(7): pp Science M, Johnstone J, Roth D, Guyatt G, Loeb M. Zinc for the treatment of the common cold: A systemic review and meta-analysis of randomized controlled trials. Canadian Medical Association Journal 2012: 184(10):E Epub 2012 May 7. Thampi N, Stephens D, Rea E, Kitai I. Unexplained deterioration during antituberculous therapy in children and adolescents: Clinical presentation and risk factors. Pediatric Infectious Disease Journal 2012: 31(2): pp Warren T, Yau Y, Ratjen F, Tullis E, Waters V. Serum galactomannan in cystic fibrosis patients colonized with aspergillus species. Medical Mycology 2012: April 23 (Epub ahead of print). Waters V, Atenafu EG, Salazar JG, Lu A, Yau Y, Matukas L, Tullis E, Ratjen F. Chronic stenotrophomonas maltophilia infection and exacerbation outcomes in cystic fibrosis. Journal of Cystic Fibrosis 2012: 11 (1): pp Waters V. New treatment for emerging CF pathogens other than pseudomonas. Current Pharmaceutical Design 2012: 18 (5): pp Science M. Guide to vaccinations in pediatric IBD patients on anti-tnf agents and immunodulators. In: Patient and Physician handout prepared by the GI and the Infectious Diseases Services at the Hospital for Sick Children, Grants and Funding Assessing the breadth and depth of cystic fibrosis-associated polymicrobial respiratory tract infections. Guttman DS, Cowen LE, Davidson AR, Hwang DM, O Doherty KC, Parkinson J, Tullis DE, Waters VJ, Yau YC, Maughan H. Canadian Institutes of Health Research (CIHR) CMF Emerging Team Grant: Canadian Microbiome Initiative. Institute of Infection and Immunity. $2,447,425. Cystic Fibrosis Canada. $50,000 CAD to 2015 Bone and renal outcomes in HIV-exposed, uninfected infants with perinatal exposure to Tenofovir. Brophy J, Samson L, Bitnun A, Read S, Feber J, Matzinger M, Alos N, Lamarre V, Alimenti A. Canadian HIV Trials Network (CTN). $50,000 CAD. July 2010 to July 2012 Care and research to optimize HIV-positive women s health during preconception, pregnancy and motherhood. Interdisciplinary HIV Pregnancy Research Group. Loutfy MR, Serghides L, Yudin MH, Bilsborrow P, Doolittle-Romas M, Ion A, Katherine W, Leader A, Linklater G, Maggi JD, Margolese SL, Masinde Khatundi I, Maxwell JD, McGee F, Muchenje M, O Brien-Teengs D, Peltier DJ, Ringlein M, Said T, Sandre R, Sharp M, Tharao Wangari E, Angel JB, Bayoumi AM, Bitnun SA, Brophy JC, Calzavara L, Carvalhal A, Greene S, Gruslin AM, Hamilton GS, Laporte C, Lye SJ, Murphy KE, Newmeyer TS, Raboud JM, Rachlis AR, Ralph E D, Rosenthal KL, Shapiro HM, Shynlova O, Silverman M, Smaill FM, Smith ML, Walmsley SL, Wobeser WL. Canadian Institutes of Health Research. (CIHR) Emerging Team Grant Maternal Health: From Pre-conception to the Empty Nest. $900,000 CAD. Apr 2011 to Apr the hospital for sick children department of paediatrics Annual Report

54 Does complicated pneumonia lead to chronic lung disease? Cohen E, Weinstein M, Mahant S, Dell S, Traubici J, Wadhwa A. Pediatric Consultants Partnership, Hospital for Sick Children, University of Toronto. $9900 CAD to Jul 2011 Effect of omega-3 fatty acid supplementation on hypertriglyceridemia in HIV-infected children taking highly active antiretroviral therapy (HAART): A randomized cross-over study. Bitnun A, Read SE, Brophy J. Abbott Laboratories. $40,500 CAD. Sep 2008 to Sep 2011 Epstein-Barr virus in health and disease in children and youth: A prospective study of markers in virus host interaction and genetic variation. Allen U, Beyene J, Mazzulli, Robinson J, Read S, Grunebaum El, Zielenska M, Tellier R, Weitzman S. Canadian Institutes of Health Research (CIHR). $398,968 CAD. Jan 2008 to Mar 2012 Exploring the experiences of paediatric residents with the hidden curriculum using narrative reflections. Matlow A, Atkinson A, Martiminanakis T. Pediatric Consultants, Hospital for Sick Children, Education Grant. $4,750 CAD to 2013 IHPREG pilot study: Feasibility of the Ontario HIV-preconception cohort study. Loutfy M, Angel J, Carvalhal A, Murphy K, Gough K, Bayoumi A,, Walmsley S, Smaill F, Rachlis A, Gysler M, Bitnun A, Margolese S, Raboud J, Yudin M, Brophy J, Masinde K, Rosenthal K, Shapiro H, Librach C, Silverman M, Sandre R, Leader A, Ralph E, Powis J, Laziri A, Ringlein, Vijayanathan, Tharao W, Burtch, Margolese S, Masinde K. Canadian Institutes for Health Research (CIHR). $96,075 CAD. Oct 2010 to Oct 2011 Immunogenicity of arepanrix (AS03-adjuvanted H1N1 pandemic influenza vaccine) in HIV-infected children. Brophy J, Samson L, Read S, Lamarre V, Ward B, Bitnun A. Canadian Foundation for HIV/AIDS Research (CANFAR). $25,000 CAD. Oct 2010 to Oct 2013 Improving residents handoff in teaching hospitals: Understanding implementation and effectiveness of a handoff bundle. Coffey M, Matlow A. Physicians Services Incorporated Foundation (PSI). $154,000 CAD Interferon gamma release assays in immunocompromised children: A pilot study. Kitai I, Read S, Bitnun A, Malloy P. Pediatric Consultants, Hospital for Sick Children. $9,620 CAD. Sep 2010 to Sep 2012 Maternal, newborn, and child health (MNCH) coalition knowledge management initiative. Zlotkin S, Barwick M. Collaborators: Roth D, Bassani D, Bhutta Z, Wong J, Stein J, Hayford K, Arnold W, Morris S. Care Canada, Plan Canada, Save the Children Canada, World Vision Canada. $1,000,000 CAD. Jun 2012 to Feb 2015 Molecular aging and HIV co-morbidities in women and children. Cote H, Bitnun A, Money DM, Pick N, Soudeyns H. Canadian Institutes of Health Research (CIHR) Team Grant. $8,750 CAD to 2013 Nevirapine dosing in neonates for prophylaxis of mother-tochild-transmission (MTCT) of HIV infection. Bitnun SA, Lau E, Colantonio D, Seto W, Read SE, Brophy J, Samson L. Canadian Foundation for HIV/AIDS Research (CANFAR). $20,528 CAD. Oct 2011 to Oct 2013 PanCanadian study to describe the epidemiology of adverse events in children hospitalized in Canada. Matlow A, Baker R, Flintoft V. Canadian Patient Safety Institute. $120,000 CAD. Canadian Academic Hospitals. $500,000 CAD to 2012 PHAC/CIHR Influenza Research Network. Halperin SA, Bortolussi RA, Collet J-P, De Serres G, Johnson MM, Kwong JC, McNeil SA, Scheifele DW, Ward BJ and the PCIRN Investigators Group (Allen U is a co-investigator) PHAC Infectious Disease and Emergency Preparedness Branch. $13,474,459 CAD to 2013 Pilot study of genetic determinants of severe influenza in children. Tran D, Paterson A, Beyene J, Richardson S, Schuh S. University of Toronto: Dean s Fund New Staff Grant. $10,000 CAD to 2012 Preclinical characterization of multimetric ribozymes as potential preventive and therapeutic agents for HIV infection. Joshi S, Read SE. Canadian Institutes for Health Research (CIHR). $236,234 CAD to 2012 Predicting the risk of infection in children receiving chemotherapy for acute myeloid leukemia. Sung L, Allen U, Beyene J. National Cancer Institute of Canada (NCIC). $817,963. CAD. Apr 2008 to Mar 2012 Predictors of virdans group streptococcal bacteremia in pediatric acute myeloid leukemia. Science M. CIHR Master`s Award, Frederick Banting and Charles Best Graduate Scholarship. Canadian Institutes of Health Research (CIHR). $17,500 CAD to 2011 Primary EBV infection in the immunosupressed setting of organ transplantation: Determinants of virus-host interaction. Allen U, Beyene J, Dipchand A, Grunebaum E, Hebert D, Husain D, Mazzulli T, Ng V, Read S, Robinson J, Tellier R, Weitzman S. Canadian Institutes of Health Research (CIHR). $509,125 CAD. Apr 2010 to Sep 2014 Randomized double blind controlled trial of the use of a biofilm antimicrobial susceptibility assay to guide antibiotic therapy in chronic pseudomonas aeruginosa infected cystic fibrosis patients. Waters V. Innovotech Inc. and National Research Council Industrial Research Assistance Program (NRC-IRAP). $313,603 CAD to 2012 Severe influenza in infants, children and youths: Role of clinical and epidemiologic risk factors. Tran D, Halperin S, Bettinger JA, Dery P, Gubbay JB, Ipp MM, Le Saux NM, Loeb MB, McGeer AJ, Moore DL, O Connor DL, Richardson SE, Salvadori MI, Sauve LJ, Schuh S, Vaudry WL. Canadian Institutes of Health Research (CIHR): Operating Grant. $776,835 CAD to 2016 The Canadian National Transplant Research Program: Increasing donation and improving transplantation. West LJ, Caufield TA, Hebert M-J, Humar A, Keshavjee S, Knoll G, Levings MK, Mital S, Roy DC, Schultz KR, Allen U. Canadian Institutes of Health Research (CIHR). $25,000 CAD The mechanism and immunological correlates of severe respiratory illness following 2009 H1N1 pandemic influenza virus infection. Sharma S, Haeyfar SM, Mansour, Hancock REW, Sahai BM, Tran D, Alexander M, Gubbay JB, Halperin Scot A, Lin F, Loeb MB, McGeer AJ, Planiyar N, Patterson AD. Canadian Institutes of Health Research (CIHR) Catalyst Grant: Pandemic Outbreak Research Response. Institute of Circulatory and Respiratory Health. $200,000 CAD, Pandemic. $100,000 CAD. The psychosocial experiences and needs of HIV-positive women during their first year of motherhood. Greene S, Loutfy M, Carvalhal A, Bitnun A, Brophy J, Kapiriri L, Margolese S, Masinde K, Murphy K, Raboud J, Salter R, Samson L, Smaill F, Yudin M. Ontario HIV Trials Network (OHTN). $450,000 CAD to 2013 The role of clonal pseudomonas aeruginosa in cystic fibrosis patients in Canada. Waters V. Team Grant, Cystic Fibrosis Canada. $170,000 CAD to 2014 Trainee perceptions of the educational value of clinical cases on an inpatient ward: An exploration of patient and trainee-level predictors. Cohen E, Mahant S, Wadhwa A, Bismilla Z. Pediatric Consultants Education Research Grant, Hospital for Sick Children, University of Toronto. $5000 CAD to the hospital for sick children department of paediatrics Annual Report

55 NEONATOLOGY overview Division Head Dr. Shoo Lee Full-Time Dr. Jacques Belik Dr. Jonathan Hellmann Dr. Andrew James Dr. Robert Jankov Dr. Kyong-Soon Lee Dr. Linh Ly Dr. Patrick McNamara Dr. Gerda Meijler Dr. Aideen Moore Dr. Martin Offringa Dr. Boriana Parvez Dr. Keith Tanswell Dr. Christopher Tomlinson Dr. Hilary Whyte Cross-Appointees The Hospital for Sick Children Dr. Bonnie Stevens Mount Sinai Hospital Dr. Olufunmi Akindele Dr. Yenge Diambomba Dr. Afif El Khuffash Dr. Emer Finan Dr. Ann Jefferies Dr. Edmond Kelly Dr. Adel Mohamed Dr. Karel O Brien Dr. Prakesh Shah Dr. Vibhuti Shah Dr. Sharon Unger Dr. Constance Williams Dr. Jennifer Young Dr. Kin Fan Young Tai North York General Hospital Dr. Maggie Shu St. Joseph s Hospital Dr. Dilip Mehta St. Michael s Hospital Dr. Tony Barozzino Dr. Douglas Campbell Dr. Michael Sgro Dr. Ethel Ying Sunnybrook Health Sciences Centre Dr. Elizabeth Asztalos Dr. Paige Church Dr. Michael Dunn Dr. Eugene Ng Dr. Brian Simmons Dr. Martin Skidmore Dr. Adrian Ziino Research Fellows and Associates Evelynia Ciolko Peyman Ghorbani Kiran Gosal Wenlan Huang Mobin Jamal Rishikesan Kamaleswaran Enomoto Masahiro Megan Melland-Smith Yi Quan Emily Xu Dr. Yanping Zhu Clinical Fellows Dr. Nizar Abazid Dr. Mutih Abu Awwad Dr. Suhair Al-Shanteer Dr. Mai Al-Qubaisi Dr. Rudaina Banihani Dr. Sladjana Bulatovic Dr. Marina Castro Dr. Poorva Deshpande Dr. Colleen Doherty Dr. Marta Fontanin Dr. Danielle Freeman Dr. Habib Haddad Dr. Hamid Hakak Dr. Mingyan Hei Dr. Cecilia Herbozo Dr. Tetsuya Isayama Dr. Faiza Khurshid Dr. Jillian Lewis Dr. Jia Li Dr. Patrocinio Mateo-Rodriguez Dr. Amir Elmekkawi Mohamed Dr. Woei Bing Poon Dr. Sheryle Rogerson Dr. Asaph Rolnitsky Dr. Pankaj Sakhuja Dr. Jyotsna Shah Dr. Mohamed Tagin Dr. Jessie Van Dyk Dr. Qing (Shirly) Xia Dr. Lin Yuan Dr. Meijian Zhao The three level III NICUs continue to experience excessively high occupancy rates and difficulties with retro-transfers to community hospitals, despite the additional level II beds provided by the province to community hospitals. The NICU at Sunnybrook Health Sciences Centre opened eight additional NICU beds. Mount Sinai is building a new NICU which is scheduled to open in early SickKids has commissioned an architectural firm to begin planning for rebuilding the NICU. The Ontario Human Donor Milk Bank was funded by the Ontario government and construction is in progress at Mount Sinai Hospital. The Ontario Human Donor Milk Bank was funded by the Ontario government and construction is in progress at Mount Sinai Hospital. The bank is a partnership of three hospitals Mount Sinai Hospital, Hospital for Sick Children and Sunnybrook Health Sciences Centre. It will begin providing milk in April 2012, and will provide human donor milk to all eligible babies in Toronto during the first year, and then to all eligible babies in Ontario during the second year. A major donor has provided funding of $1.2M for building the milk bank. The newborn follow-up clinics at Mount Sinai Hospital, Sunnybrook Health Sciences Centre and The Hospital for Sick Children utilize a common protocol and are developing a shared database, to facilitate monitoring of outcomes and research. The Division of Neonatology welcomed Dr. Boriana Parvez to the Faculty at the Hospital for Sick Children and Dr. Martin Offringa to the Faculty at Mount Sinai Hospital. Dr. Gerda Meijler briefly joined the Faculty at Mount Sinai Hospital but then decided to return to the Netherlands. The Division of Neonatology wishes to thank Dr. Adrian Ziino for his service and significant contributions at Sunnybrook Health Sciences Centre. department of paediatrics Annual Report

56 Subspecialty Residents Dr. Dalal Abdelgadir Dr. Julie Emberley Dr. Enas El Gouhary Dr. Maksim Kirtsman Dr. Amit Mukerji Dr. Julia Panczuk Dr. James Edward Pugh Dr. Wissam Shalish Dr. Navneet Sharma Dr. Jennifer Twiss Dr. Payam Vali Dr. Dany Weisz Adjunct Dr. Xiaoping Luo Honorary Faculty Dr. Max Perlman Dr. Paul Swyer Dr. Arne Ohlsson Administrative Staff Ana Ciasca Sonia Dos Santos Violeta Dukic Jewel Randolph Nithya Ravi Linde Weedman The Division s Neonatal-Perinatal Fellowship Training Program will undergo external review in The Division thanks Dr. Martin Skidmore for his leadership and significant contributions to the program over the past 10 years and welcomes Dr. Emer Finan as the new Fellowship Program Director. The Clinician-Scientist Scholar Training Program, under the direction of Dr. Robert Jankov, expanded with the recruitment of Dr. Sandra Leibel, who has enrolled in the PhD program at the University of Toronto in Lung Physiology. The Division congratulates Dr. Afif El-Khuffash and Dr. Connie Williams for completing the program and obtaining staff appointments at Dublin and McMaster University respectively. The Division continues to be well supported by major funding agencies for its various foci at the three sites, including lung biology, epidemiology, outcomes-based research and clinical trials. This year we had 146 publications, and received grant funding of $119,938, /08 08/09 Publications ( ) Honors and Awards Barozzino, T: Award for Excellence in Clinical Teaching, Undergraduate and Postgraduate Teaching in Pediatrics, Education Achievement Day, St. Michael s Hospital, Toronto, Barozzino, T: Director of Community Outreach and Ambulatory Services, Department of Pediatrics, St. Michael s Hospital, Toronto, Campbell, D: Winner of Marvin Gerstein Award as chosen by the Pediatric Residents of University of Toronto for outstanding teaching. El-Khuffash, A: Winner of Best Neonatal Platform Presentation at the 11th Annual Neonatal and Maternal Fetal Medicine Research Day, Toronto, April Finan, E: Program Director, Neonatal-Perinatal Medicine Fellowship Program, University of Toronto. Hellmann, J: The Institute of Medical Science (IMS) Course Director Award, University of Toronto, McNamara, P: Best oral presentation for neonatal trainee, Neonatal-Perinatal Research Day Weisz D et al. Non-invasive Cardiac Output Monitoring in Neonates Using Bioreactance: A Comparison with Echocardiography. McNamara, P: PAS Travel Award 2012 (D Weisz). Non-invasive Cardiac Output Monitoring in Neonates Using Bioreactance: A Comparison with Echocardiography. McNamara, P: Recipient of Canadian Paediatric Society Emerging Leader in Neonatology Award, Canada, May Meijler, G: Assistant Professor of Paediatrics, University of Toronto, Moore, A: Deputy Division Head of Neonatology, The Hospital for Sick Children and University of Toronto, January O Brien, K: Award for Excellence in Innovation - The Teach Learn Care program team - Karen McGibbon Award of Excellence 2011, Mount Sinai Hospital, Toronto, Offringa, M: Professor of Pediatrics, University of Toronto, /10 10/11 11/12 07/08 08/09 09/10 10/11 11/12 106% increase since 2008 Grants ( ) Jain, A: Winner of 2011 Exceptional Trainee Award in program of Physiology and Experimental Medicine, Research Institute, Hospital for Sick Children, Toronto, Canada. Jamal, B: Winner of Basic Science Senior Trainee Award, Department of Paediatrics Annual Research Day, University of Toronto, June Lee, S: Appointed Professor, Dalla Lana School of Public Health, University of Toronto, December Lee, KS: Nominee, President s Award, Hospital for Sick Children, June Lee, S: Appointed Scientific Director, Institute of Human Development, Child and Youth Health, Canadian Institutes of Health Research, January Offringa, M: Program Head, Child Health Evaluative Sciences (CHES), Research Institute, The Hospital for Sick Children, Panczuk, J: 2012 Postgraduate Medical Research Award, University of Toronto, Perinatal-Neonatal Medicine Research Project (Unger S, supervisor), Toronto, Parvez, B: Assistant Professor of Pediatrics, University of Toronto, Parvez, B: Clinical Director, Neonatal Intensive Care Unit, The Hospital for Sick Children, November Pugh E: Winner of SickKids Foundation Student Humanitarian Award, June Sgro, M: Chief of Pediatrics, Department of Pediatrics, St. Michael s Hospital, Toronto, % increase since 2008 Lee, S: Researcher of the Month, Canadians for Health Research, February Shah, P: Emerging Leader in Neonatology Award from the Canadian Pediatric Society, Canada, May department of paediatrics Annual Report

57 Shah, P: Karen McGibbon Award of Excellence for Interprofessional Team Participation by the Improving Quality Team (Co-Leader), Mount Sinai Hospital, Toronto, Shah, V: Canadian Pain Society s Pain Awareness Award for our HELPinKIDS website and video, Whyte, H: Acting Clinical Director, NICU, The Hospital for Sick Children, April Williams, C: Canadian Institutes of Health Research Doctoral Award Frederick Banting and Charles Best Canada Graduate Scholarship, Williams, C: Canadian Institutes of Health Research Douglas Kinsella Doctoral Award for Research in Bioethics, Young, J: Associate Professor, Department of Paediatrics, University of Toronto, Publications Agha M, Glazier RH, Moineddin R, Moore AM, Guttmann A. Socioeconomic status and prevalence of congenital heart defect: Does universal access to health care eliminate the gap? Birth Defects Research, Part A, Clinical and Molecular Teratology 2011: 91 (12): pp Alfaraj MA, Shah PS, Bohn D, Panatazi S, O Brien K, Chiu PP, Gaiteiro R, Ryan G. Congenital diaphragmatic hernia: Lung/ head ratio and lung volume for prediction of outcome. American Journal of Obstetrics and Gynecology 2011: 205 (1): pp 43.e1-8. Appendino JP, McNamara PJ, Keyzers M, Stephens D, Hahn C. The impact of amplitude-integrated electroencephalography on neonatal intensive care unit practice. Canadian Journal of Neurological Sciences 2012: 39 (3): pp Asztalos E. Antenatal corticosteroids: A risk factor for the development of chronic disease. Journal of Nutrition and Metabolism 2012: Epub 2012 Mar 5.PMID: Azarpazhooh A, Lawrence H, Limeback H, Shah PS. Xylitol for preventing acute otitis media in children up to 12 years of age. Cochrane Database of Systematic Reviews 2011: 11:CD Baird R, Eason G, Safavi A, Puligandla P, Laberge JM, Skarsgard ED, Canadian Pediatric Surgery Network. Institutional practice and outcome variation in the management of congenital diaphragmatic hernia and gastroschisis in Canada: A report from the Canadian Pediatric Surgery Network. Journal of Pediatric Surgery 2011: 46: pp Barrett R, Heddle N, Kirpalani H, Moore RG, Arnold E, Shah PS, Staines A. Someone else s blood : Directed blood donation for neonatal transfusion and parental perceptions of risk. Journal of Risk Research 2011: 14 (7): pp Basil K, Lee SK, Barrington K, Harrison A, da Silva O, Shah P. The changing epidemiology of preterm twins and triplets admitted to NICUs in Canada, American Journal of Perinatology 2011: DOI: /s Beck J, Reilly M, Grasselli G, Qui H, Campoccia F, Slutsky A, Dunn MS, Sinderby C. Characterization of neural breathing pattern in spontaneously breathing preterm infants. Pediatric Research 2011: 70 (6): pp Aug: Epub. Belik J, McIntyre BAS, Pan J, Grasemann H, Vasquez-Vivar J. Pulmonary hypertension in the newborn GTP-cyclohydrolase I-deficient mouse. Free Radical Biology and Medicine 2011: 51: pp Binenbaum G, Ying GS, Quinn GE, Dreisetti S, Karp K, Roberts RS, Kirpalani H, for the Premature Infants in Need of Transfusion Study Group (including Asztalos E). A clinical prediction model to stratify retinopathy of prematurity risk using postnatal weight gain. Pediatrics 2011: 127 (3): pp e607-e614. Boyajian T, Shah PS, Murphy KE. HIV positive pregnant women receiving HAART: A matched cohort study of pregnancy outcomes. Journal of Obstetricians and Gynecologists of Canada 2012: 34 (2): pp Brignardello-Petersen R, Carrasco-Labra A, Araya I, Yanine N, Beyene J, Shah PS. Is adjuvant laser therapy effective for preventing pain, swelling, and trismus after surgical removal of impacted mandibular third molars? A systematic review and meta-analysis. Journal of Oral and Maxillofacial Surgery 2012: 70 (8): pp Epub 2012 Mar 6. Brindle ME, Brar M, Skarsgard ED, Canadian Pediatric Surgery Network (CAPSNet). Patch repair is an independent predictor of morbidity and mortality in congenital diaphragmatic hernia. Pediatric Surgery International 2011: 27 (9): pp Cately C, McGregor C, James A. A framework for multidimensional real-time data analysis: A case study for the detection of apnoea of prematurity. Journal of Computational Models and Algorithms in Medicine 2011: 2 (1): pp Chahal J, Marks PH, MacDonald PB, Shah PS, Theodoropoulos J, Ravi B, Whelan DB. Anatomic Bankart repair compared with nonoperative treatment and/or arthroscopic lavage for first-time traumatic shoulder dislocation: A systematic review and metaanalysis. Arthroscopy 2012: 28 (4): pp Epub 2012 Feb 14. Chierichetti M, Engelberts D, EL-Khuffash A, Babyn P, Post M, Kavanagh BP. Continuous negative abdominal distension recruits atelectatic lungs. Critical Care Medicine 2012: 40 (6): pp Chirinian N, Uji A, Isayama T, Shah V. Neonatal care in a Japanese NICU: Notes based on a site visit. Neonatal Network 2012: 31 (2): pp Church PT. The canary s warning: Lessons from patients with spina bifida. Journal of Population Therapeutics and Clinical Pharmacology 2011: 18 (3): pp e503-e504. Church PT, Luther M, Asztalos E. The perfect storm: The high prevalence low severity outcomes of the preterm survivor. Current Pediatric Reviews 2012: 8 (2): pp Cronin CMG, Baker GR, Lee SK, Ohlsson A, McMillan D, Seshia MMK, Canadian Neonatal Network EPIQ Study Group. Reflections on knowledge translation in Canadian NICUs using the EPIQ method. Healthcare Quarterly Special Issue October 2011: 14: pp Crowther CA, Aghajafari F, Askie LM, Asztalos EV, Brocklehurst P, Bubner TK, Doyle LW, Dutta S, Garite TJ, Guinn DA, Hallman M, Hannah ME, Hardy P, Maurel K, Mazumder P, McEvoy C, Middleton PF, Murphy KE, Peltoniemi OM, Peters D, Sullivan L, Thom EA, Voysey M, Wapner RJ, Yelland L, Zhang S. Repeat prenatal corticosteroids prior to preterm birth: A systematic review and individual participant data meta-analysis for the PRECISE study group(prenatal repeat corticosteroid international IPD study group: Assessing the effects using the best level of evidence)-study protocol. Systematic Reviews 2012: 1: 12.PMID: Curran V, Hollett A, Casimiro L, McCarthy P, Banfield V, Hall P, Lackie K, Oandasan I, Simmons B, Wagner S. Development and validation of an interprofessional collaborator assessment rubric (ICAR). Journal of Interprofessional Care 2011: 25 (5): pp de Bruïne FT, van den Berg-Huysmans A, Leijser LM, Rijken M, Steggerda SJ, van der Grond J, van Wezel-Meijler G. Clinical implications of MRI findings in the white matter in very preterm infants: A two year follow-up study. Radiology 2011: Oct 26. (Epub ahead of print). de Haan TR, Bijleveld YA, van de Lee JH, Groenendaal F, van den Broek MP, Rademaker CM, van Straaten HL, van Weissenbruch MM, Vermeulen JR, Dijk PH, Dudink J, Rijken M, van Heijst A, Dijkman KP, Gavilanes D, van Kaam AH, Offringa M, Mathot RA. Pharmacokinetics and pharmacodynamics of medication in asphyxiated newborns during controlled hypothermia. The PharmaCool multicenter study. BMC Pediatrics 2012: 12(1): pp Apr 19 (Epub ahead of print). de Ru MH, Boelens JJ, Das AM, Jones SA, van der Lee JH, Mahlaoui N, Mengel E, Offringa M, O Meara A, Parini R, Rovelli A, Sykora KW, Valayannopoulos V, Vellodi A, Wynn RF, Wijburg FA. Enzyme replacement therapy and/or hematopoietic stem cell transplantation at diagnosis in patients with mucopolysaccharidosis type I: Results of a European consensus procedure. Orphanet Journal of Rare Diseases 2011: 6: pp 55. de Winter JP, Joosten KFM, Dorrius MD, van Hasselt PM, IJland MM, Verkade HJ, Offringa M. Een nieuwe richtlijn voor vitamine K toediening aan voldragen pasgeborenen in Nederland. Nederlands Tijdschrift voor Geneeskunde 2011: 155: A the hospital for sick children department of paediatrics Annual Report

58 Demauro SB, Roberts RS, Davis P, Alvaro R, Bairam A, Schmidt B, for Caffeine for Apnea of Prematurity Trial Group (including Asztalos E). Impact of delivery room resuscitation on outcomes up to 18 months in very low birth weight infants. Journal of Pediatrics 2011: 159 (3): pp Diamond IR, desilva N, Tomlinson G, Pencharz P, Feldman BM, Moore AM, Ling SC, Wales PW. The role of parenteral lipids in the development of advanced intestinal failure associated liver disease in infants - A multiple variable analysis. Journal of Parenteral and Enteral Nutrition 2011: 35 (5): pp Diamond IR, Feldman BM, Pencharz P, Moore AM, Ling SC, Wales PW. Novel lipid based approaches to pediatric intestinal failure associated liver disease. Archives of Pediatric and Adolescent Medicine 2012: 166 (5): pp Dunn MS, Jefferies AL, Canadian Paediatric Society, Fetus and Newborn Committee. Recommendations for neonatal surfactant therapy - Addendum. Paediatrics and Child Health 2012: 17 (3): pp Dunn M, Kaempf J, deklerk A, deklerk R, Reilly M, Howard D, Ferrelli K, O Conor J, Soll R, for the VON DRM Study Group. Randomized trial comparing 3 approaches to the initial respiratory management of preterm neonates. Pediatrics 2011: 128 (5): pp e Egyepong J, Jain A, Chow P, Godambe S. Parenteral nutrition Ascitis with acute renal failure as a complication from umbilical venous catheter in an extremely low birth weight infant. BMJ Case Reports 2011 (doi: /bcr ). EL-Khuffash A, Jain A, McNamara PJ, Ng E, Lee SK. The use of targeted neonatal echocardiography to guide placement of peripherally inserted central catheters in neonates: An assessment of practice. American Journal of Perinatology 2012: 29: pp DOI: / EL-Khuffash A, Mertens L. Early treatment of a patent ductus arteriosus. Do we know what we are doing? Pediatric Critical Care Medicine 2012: 13 (3): pp El-Khufash A, Unger S. The concept of milk kinship in Islam: Issues raised when offering preterm infants of Muslim families donor human milk. Journal of Human Lactation 2012: 28 (2): pp Feb 6; Epub. El-Naggar W, McNamara PJ. Delivery room resuscitation of preterm infants in Canada: Current practice and neonatologists views. Journal of Perinatology 2011: 32 (7): pp Sep 22. doi: /jp (Epub ahead of print). Epelman M, Daneman A, Halliday W, Whyte H, Blaser S. Abnormal corpus callosum in neonates after hypoxic ischemic injury. Pediatric Radiology 2012: 42 (3): pp Ewaschuk J, Unger S, Harvey S, O Connor D, Field C. Effect of pasteurization on immune components of milk: Implications for feeding preterm infants. Applied Physiology, Nutrition, and Metabolism 2011: 36: pp Fernandes RM, van der Lee JH, Offringa M. Data monitoring committees, interim analysis and early termination in paediatric trials. Acta Paediatrica 2011: doi: /j x. (Epub ahead of print). Finan E, Bismilla Z, Campbell C, LeBlanc V, Jefferies A, Whyte H. Improved procedural performance following a simulation training session may not be transferable to the clinical environment. Journal of Perinatology 2011: Sep 29. doi: /jp Finan E, Bismilla Z, Whyte HE, LeBlancV, McNamara PJ. High-fidelity simulator technology may not be superior to traditional low-fidelity equipment for neonatal resuscitation training. Journal of Perinatology 2012: 32 (4): pp Oct 27. doi: /jp (Epub ahead of print). Gilbert NL, Fell DB, Joseph KS, Liu S, León JA, Sauve R, for the Fetal and Infant Health Study Group of the Canadian Perinatal Surveillance System. Temporal trends in sudden infant death syndrome in Canada from 1991 to 2005: Contribution of changes in cause of death assignment practices and in maternal and infant characteristics. Paediatrics and Perinatal Epidemiology 2012: Jan 9 (Epub ahead of print) doi: /j x Harel Z, Gilbert C, Wald R, Bell C, Juurlink D, Perl J, Beyene J, Shah PS. The effect of combination therapy with aliskiren and blockers of the renin angiotensin system on hyperkalemia and acute kidney injury: A systematic review and meta-analyses. British Medical Journal 2012: 344: pp e42. Hartling L, Wittmeier KD, Caldwell PH, van der Lee JH, Klassen TP, Craig JC, Offringa M for the StaR Child Health group. StaR Child Health: Developing evidence-based guidance for the design, conduct, and reporting of pediatric trials. Clinical Pharmacology and Therapeutics 2011: 90 (5): pp doi: /clpt Epub 2011 Oct 12. Hellmann J, Williams C, Ives-Baine L, Shah PS. Withdrawal of artificial nutrition and hydration in the neonatal intensive care unit: Parental perspectives. Archives of Disease of Childhood. Fetal Neonatal Edition 2012 (Epub ahead of print March 23). Hui D, Morrison LJ, Windrim R, Lausman AY, Hawryluck L, Dorian P, Lapinsky SE, Halpern SH, Campbell DM, Hawkins P, Wax R, Carvalho JCA, Kainty KN, Maxwell C, Jeejeebhoy FM. A novel implementation strategy for the 2010 Guidelines for the Management of Cardiac Arrest in Pregnancy. Journal of Obstetrics and Gynaecology Canada 2011: 33 (8): pp Hui D, Okun N, Murphy K, Kingdom J, Uleryk E, Shah PS. Combinations of maternal serum markers to predict adverse pregnancy outcome: A systematic review. Journal of Obstetricians and Gynecologists of Canada 2012: 34 (2): pp Jacobs SE, Stewart M, McNamara PJ, Wright I, Kirpalani H, Darlow B, Inder TE, Doyle LW, Morley CJ for the ICE Collaboration. The ICE randomised trial of whole body cooling for hypoxic-ischaemic encephalopathy (HIE). Archives of Pediatric and Adolescent Medicine 2011: 165 (8): pp Jain A, McNamara PJ, Ng E, EL-Khuffash A. The use of targeted neonatal echocardiography to confirm placement of peripherally inserted central catheters in neonates: An assessment of practice. American Journal of Perinatology 2012: 29 (2): pp Nov 21. (Epub ahead of print; PMID: ) Jain A, Sahni M, El-Khuffash A, Khadawardi E, Sehgal A, McNamara PJ. Use of targeted neonatal echocardiography to prevent postoperative cardiorespiratory instability after PDA ligation. Journal of Pediatrics 2012: 160 (4): pp e Nov 1. (Epub ahead of print; PMID: ) Jamal M, Tanswell AK. Understanding the role of reactive oxygen species in alveolar formation of neonatal rat lungs. Canadian Respiratory Journal 2012: 19: pp e46. Jansen LA, Lin Y, McNab YC, Skarsgard ED and the Canadian Pediatric Surgery Network. Pre-closure fluid resuscitation influences outcome in gastrochisis. Journal of Perinatology 2011: DOI: /s Jefferies A, Fetus and Newborn Committee and Psychosocial Paediatrics Committee, Canadian Paediatic Society. Selective serotonin reuptake inhibitors in pregnancy and infant outcomes. Paediatrics and Child Health 2011: 16: pp 562. Jefferies A, Fetus and Newborn Committee, Canadian Paediatric Society. Kangaroo care for the preterm infant and family. Paediatrics and Child Health 2012: 17: pp Jefferies A, Shah V. Clinicians prefer simple educational tools for implementing practice change. Medical Teacher 2011: 33 (11): pp e Jerkic M, Kabir MG, Davies A, Yu LX, McIntyre BAS, Husain NW, Enomoto M, Sotov V, Husain M, Henkelman M, Belik J, Letarte M. Pulmonary hypertension in adult Alk1 heterozygous mice due to oxidative stress. Cardiovascular Research 2011: 92: pp Joseph KS, Liu S, Rouleau J, Lisonkova S, Hutcheon J, Sauve R, Allen AC, Kramer MS for the Fetal and Infant Health Study Group of the Canadian Perinatal Surveillance System (including Shah P). Influence of definition-based versus pragmatic birth registration on international comparisons of perinatal and infant mortality: A population-based retrospective study. British Medical Journal 2012: pp 344: e746. doi: /bmj.e the hospital for sick children department of paediatrics Annual Report

59 Juarez B, Santos I, McDonald M, Hayden J, Shah PS, Ross H. Factors associated with the development of cardiac allograft vasculopathy: A systematic review of observational studies. Clinical Transplantation 2011: Dec 13. doi: /j x. (Epub ahead of print). Kamholz KL, Dukhovny D, Kirpalani H, Whyte RK, Roberts RS, Wang N, Mao W, Zupancic JA, for the Premature Infants in Need of Transfusion Study Group (including Asztalos E). Economic evaluation alongside the Premature Infants in Need of Transfusion randomized controlled trial. Archives of Disease in Childhood. Fetal and Neonatal Edition 2011: Jul 6 (Epub ahead of print). Kanungo J, James A, Lodha A, McMillan D, Faucher D, Lee SK, Shah PS and the Canadian Neonatal Network. Advanced maternal age and the outcomes of preterm infants: A social paradox? Obstetrics and Gynecology 2011: 118 (4): pp Kemper EM, Merkus M, Wierenga PC, van Rijn PC, van der Werff D, Lie-A-Huen L, Offringa M. Towards evidence-based pharmacotherapy in children. Pediatric Anesthesia 2011: 21 (3): pp Kingdom J, Walker M, Proctor L, Keating S, Shah PS, McLeod A, Johannes K, Windrim R, Dodd J. Unfractionated heparin for second trimester placental insufficiency. A pilot randomized trial. Journal of Thrombosis and Haemostasis 2011: 9 (8): pp Kok JH, Offringa M. Bevacizumab voorkomt retinopathie van de prematuur. Nederlands Tijdschrift Voor Geneeskunde 2011: 155: pp A3987. Kuster SP, Shah PS, Coleman BL, Lam PP, Tong A, Wormsbecker A, McGeer A. Incidence of influenza in healthy adults and health care workers: A systematic review. PLoS One 2011: 6 (10): pp e Ladhani N, Shah PS, Murphy K on behalf of Knowledge Synthesis Group on Determinants of Preterm/LBW Births. Prenatal amphetamine exposure and birth outcomes: A systematic review and meta-analyses. American Journal of Obstetrics and Gynecology 2011: 205 (3): 219. pp e1-7. Lee SK, Aziz K, Singhal N, Cronin G, Canadian Neonatal Network. The EPIQ evidence reviews - Practical tools for an integrated approach to knowledge translation. Paediatrics and Child Health 2011: 16: pp Lee W, Donner EJ, Nossin-Manor R, Whyte HE, Sled JG, Taylor MJ. Visual functional MRI of premature infants. Developmental Medicine and Child Neurology 2012: 54 (8): pp Levendoglu-Tygal O, Alexander V, Parvez B, Lagamma EF. Hemophagocytic lymphohistiocytosis associated with necrotizing enterocolitis in premature newborns. Pediatric Blood and Cancer 2012: Feb 7 doi: /pbc (Epub ahead of print). Leviton A, Kuban KC, Allred EN, Fichorova RN, O Shea TM, Paneth N, ELGAN Study Investigators (including Church PT). Early postnatal blood concentrations of inflammation-related proteins and microcephaly two years later in infants born before the 28th post-menstrual week. Early Human Development 2011: 87: pp Lisonkova S, Sheps SB, Janssen PA, Lee SK, Dalghren L, MacNab Y. Birth outcomes among older mothers in rural versus urban areas: A residence-based approach. Journal of Rural Health 2011: 27: pp Magee LA, Abalos E, von Dadelszen P, Sibai B, Easterling T, Walkinshaw S, for the CHIPS Study Group (including Asztalos E). How to manage hypertension in pregnancy effectively. British Journal of Clinical Pharmacology 2011: 72 (3): pp Mertens L (on behalf of TnECHO writing group). Neonatal echocardiography Different approaches on different continents. Reply to a Letter by Evans and Kluckow. Journal of the American Society of Echocardiography 2011: Dec 15. (Epub ahead of print). Mertens L, Seri I, Marek J, Arlettaz R, Barker P, McNamara P, Moon-Grady AJ, Coon PD, Noori S, Simpson J, Lai WW. Targeted neonatal echocardiography in the Neonatal Intensive Care Unit: Practice guidelines and recommendations for Training Writing Group of the American Society of Echocardiography (ASE) in collaboration with the European Association of Echocardiography (EAE) and the Association for European Pediatric Cardiologists (AEPC). Journal of the American Society of Echocardiography 2011: 24 (10): pp Mertens L, Seri I, Marek J, Arlettaz R, Barker P, McNamara P, Moon-Grady AJ, Coon PD, Noori S, Simpson J, Lai WW. Targeted neonatal echocardiography in the Neonatal Intensive Care Unit: Practice guidelines and recommendations for Training Writing group of the American Society of Echocardiography (ASE) in collaboration with the European Association of Echocardiography (EAE) and the Association for European Pediatric Cardiologists (AEPC). European Journal of Echocardiography 2011: 12 (10): pp Mirea L, Sankaran K, Seshia MMK, Ohlsson A, Allen AC, Aziz K, Lee SK, Shah PS, and the Canadian Neonatal Network. Treatment of patent ductus arteriosus and neonatal mortality/ morbidities: Adjustment for treatment selection bias. Journal of Pediatrics 2012: Jun 13 (Epub ahead of print). Mitchell I, Paes BA, Li A, Lanctot KL, CARES investigators. CARESS: The Canadian registry of palivizumab. Pediatric Infectious Disease 2011: 30 (8): pp Mohamed A, Shah PS. Transfusion associated necrotizing enterocolitis: A meta-analysis of observational data. Pediatrics 2012: 129 (3): pp Morag I, Eppelman M, Daneman A, Moore A, Shah PS. Long term outcomes of neonatal portal venous thrombosis. Journal of Paediatrics and Child Health 2011: 47 (6): pp Murphy KE, Hannah ME, Willan AR, Ohlsson A, Kelly EN, Matthews SG, Saigal S, Asztalos E, Ross S, Delisle MF, Tomat L, Amankwah K, Guselle P, Gafni A, Lee SK, Armson BA, for the MACS Collaborative Group. Maternal side-effects after multiple courses of antenatal corticosteroids (MACS): The three-month follow-up of women in the randomized controlled trial of MACS for preterm birth study. Journal of Obstetrics and Gynaecology Canada 2011: 33 (9): pp Murphy KE, Willan AR, Hannah ME, Ohlsson A, Kelly EN, Matthews SG, Saigal S, Asztalos E, Ross S, Delisle MF, Amankwah K, Guselle P, Gafni A, Lee SK, Armson BA, Multiple Courses of Antenatal Corticosteroids for Preterm Birth Study Collaborative Group. Effect of antenatal corticosteroids on fetal growth and gestational age at birth. Obstetrics and Gynaecology 2012: 119: pp Nash A, Dunn M, Asztalos E, Corey M, Mulvihill-Jory B, O Connor DL. Pattern of growth of very low birth weight infants, assessed using the WHO Growth Standards, is associated with neurodevelopment. Applied Physiology, Nutrition, and Metabolism 2011: 36 (4): pp Epub 2011 Aug 19. Nasr A, Langer J, Canadian Pediatric Surgery Network. Influence of location of delivery on outcome in neonates with congenital diaphragmatic hernia. Journal of Pediatric Surgery 2011: 46: pp Nasui O, Nathanael G, Miller E, Belik J, Crawley A, Weiss R, Detzler G, Zhong A, Moineddin R, Doria A. Responsiveness of BOLD MRI to short-term temperature changes in rabbit knees with inflammatory arthritis. Rheumatology: Current Research 2012: S2: 003. Nousin-Manor R, Chung A, Whyte H, Shroff M, Taylor M, Sled J. Deep gray matter maturation in very preterm neonates: Regional variations and pathology-related age dependent changes in magnetization transfer ratio. Radiology 2012: 263 (2): pp O Connor DL, Weishuhn K, Rovet J, Vaughan J, Mirabella G, Jefferies A, Campbell DM, Asztalos E, Feldman M, Whyte H, Westall C on behalf of the Post-discharge Feeding Study Group. Visual development of human milk-fed preterm infants provided with extra energy and nutrients after hospital discharge. Journal of Parenteral and Enteral Nutrition 2012: 36: pp : Dec13. DOI / O Brien K, Campbell C, Brown L, Wenger L, Shah V. Infant flow biphasic nasal continuous positive airway pressure (BP- NCPAP) vs. infant flow NCPAP for the facilitation of extubation in infants 1,250 grams: A randomized controlled trial. BMC Pediatrics 2012: 12: pp 43. O Shea TM, Allred EN, Kuban KC, Hirtz D, Specter B, Durfee S, Paneth N, Leviton A and ELGAN Study Investigators (including Church PT). Intraventricular hemorrhage and developmental outcomes at 24 months of age in extremely preterm infants. Journal of Child Neurology 2012: 27: pp the hospital for sick children department of paediatrics Annual Report

60 O Shea TM, Allred EN, Kuban KC, Dammann O, Paneth N, Fichorova R, Hirtz D, Leviton A, ELGAN Study Investigators (including Church PT). Elevated concentrations of inflammationrelated proteins in postnatal blood predict severe developmental delay at 2 years in extremely preterm infants. Journal of Pediatrics 2012: 160: pp Offringa M. Nieuwe normaalwaarden hartfrequentie en ademhalingsfrequentie bij kinderen. Nederlands Tijdschrift Voor Geneeskunde 2011: 155: A3975. Offringa M, Newton R. Prophylactic drug management for febrile seizures in children. Cochrane Database of Systemic Reviews 2012: Apr 18:4:CD PubMed PMID: Offringa M, van der Lee H. Small sample approach, and statistical and epidemiological aspects. Handbook of Experimental Pharmacology 2011: 205: pp Okwundu CI, Okoromah CAN, Shah PS. Prophylactic phototherapy for preventing jaundice in preterm very low birth weight infants. Cochrane Database of Systematic Reviews 2012: Jan 18:1:CD Onland W, de Laat MW, Mol BW, Offringa M. Effects of antenatal corticosteroids given prior to 26 weeks gestation: A systematic review of randomized controlled trials. American Journal of Perinatology 2011: 28 (1): pp Onland W, Offringa M, Cools F, De Jaegere AP, Rademaker K, Blom H, Cavatorta E, Debeer A, Dijk PH, van Heijst AF, Kramer BW, Kroon AA, Mohns T, van Straaten HL, Te Pas AB, Theyskens C, van Weissenbruch MM, van Kaam AH. Systemic hydrocortisone to prevent bronchopulmonary dysplasia in preterm infants (the SToP-BPD study): A multicenter randomized placebo controlled trial. BMC Pediatrics 2011: 11: 102. Onland W, Offringa M, van Kaam A. Late ( 7 days) inhalation corticosteroids to reduce bronchopulmonary dysplasia in preterm infants. Cochrane Database of Systemic Reviews 2012: Apr 18:4:CD PubMed PMID: Peliowski-Davidovich A, Canadian Paediatric Society Fetus and Newborn Committee (including Whyte H). Hypothermia for newborns with hypoxic ischemic encephalopathy. Paediatrics and Child Health 2012: 17 (1): pp Peliowski A, Canadian Paediatric Society Fetus and Newborn Committee (including Whyte H). Inhaled nitric oxide use in newborns. Paediatrics and Child Health 2012: 17 (2): pp Peng G, Ivanovska J, Kantores C, Van Vliet T, Jain A, Engelberts D, Kavanagh BP, Enomoto M, Belik J, McNamara PJ, Jankov RP. Sustained hypercapnia reverses chronic hypoxic pulmonary hypertension by inhibiting Rho-kinase activity. American Journal of Physiology: Heart and Circulatory Physiology 2012: 302 (12): pp H2599-H2611. Pound C, Unger S. The baby friendly initiative. Pediatrics and Child Health 2012: 17 (6): pp Qiu X, Lodha A, Shah PS, Sankaran K, Seshia MM, Yee W, Jefferies A, Lee SK, the Canadian Neonatal Network. Outcomes of small for gestational age preterm infants in Canada. American Journal of Perinatology 2012: 29 (2): pp Epub 2011 Nov 30. Rosier FMF, van Wezel-Meijler G, Kaschula ROC, Wranz PAB, Odendaal HJ, de Vries JIP. Placental histology related to fetal brain sonography in fetuses at risk for preterm birth. Archives of Disease of Childhood. Fetal Neonatal Edition 2011: 96: pp F Safavi A, Skarsgard ED, Butterworth S, Canadian Pediatric Surgery Network. Bowel-defect disproportion in gastroschisis. Does the need to extend the fascial defect predict outcome? Pediatric Surgery International 2012: Feb 14. Safavi A, Synnes AR, O Brien K, Chiang M, Skarsgard ED, Chiu PP, Canadian Pediatric Surgery Network. Multi-institutional follow-up of patients with congenital diaphragmatic hernia reveals severe disability and variations in practice. Journal of Pediatric Surgery 2012: 47 (5): pp Sananes R, Manlhiot C, Kelly E, Hornberger LK, Williams WG, MacGregor D, Buncic R, McCrindle BW. Neurodevelopmental outcomes following open heart surgery before 3 months of age. Annals of Thoracic Surgery 2012: 93 (5): pp Sakhuja P, Whyte H, Finan E. Accuracy of the rule: Neonatal endotracheal intubation. Journal of Nepal Paediatric Society 2012: 32 (1): pp 93. Saunders NR, Hellmann J, Farine D. Cerebral palsy and assisted conception. Journal of Obstetrics and Gynaecology Canada 2011: 33 (10): pp Schmidt B, Anderson PJ, Doyle LW, Dewey D, Grunau RE, Asztalos EV, Davis PG, Tin W, Moddemann D, Solimano A, Ohlsson A, Barrington KJ, Roberts RS, for the Caffeine for Apnea of Prematurity (CAP) Trial Investigators. Survival without disability to age 5 years after neonatal caffeine therapy for apnea of prematurity. Journal of the American Medical Association 2012: 307 (3): pp Schmidt B, Roberts RS, Davis P, Doyle LW, Steering Committee on the Caffeine for Apnea of Prematurity (CAP) Trial (including Asztalos E). Archimedes: Does caffeine treatment for apnoea of prematurity improve neurodevelopmental outcome in later life? Archives of Disease in Childhood 2011: 96 (8): pp 784. Schmidt B, Seshia M, Shankaran S, Mildenhall L, Tyson J, Lui K, Fok T, Roberts R, for Trial of Indomethacin Prophylaxis in Preterms (including Asztalos E). Effects of prophylactic indomethacin in extremely low-birth-weight infants with and without adequate exposure to antenatal corticoids. Archives of Pediatrics and Adolescent Medicine 2011: 165 (7): pp Schrey S, Kelly EN, Langer JC, Davies GA, Windrim R, Seaward PG, Ryan G. Fetal thoraco-amniotic shunting for large macrocystic congenital cystic adenomatoid malformations (CCAM) of the lung. Ultrasound in Obstetrics and Gynecology 2012: 39 (5): pp : Jan 5. Doi: /uog (Epub ahead of print). Schulz S, Boeker M, Spackman K, James A, Cocos C. Scalable representations of diseases in biomedical ontologies. Journal of Biomedical Semantics 2011: 17 (2) Suppl 2: S6. Scott JA, North M L, Rafii M, Huang H, Pencharz P, Subbarao P, Belik J, Grasemann H. Asymmetric dimethylarginine contributes to the airways hyperresponsiveness in asthma. American Journal of Respiratory Critical Care Medicine 2011: 184: pp Sehgal A, Francis J, James A, McNamara PJ. Staging the ductus arteriosus facilitates identification of neonates at increased risk of respiratory morbidity. Journal of Neonatal-Perinatal Medicine 2011: 4 (1): pp Sehgal A, McNamara PJ. The ductus arteriosus. A refined approach? Seminars in Perinatology 2012: 36 (2): pp Sehgal AS, McNamara PJ. Coronary artery hypo-perfusion is associated with impaired diastolic dysfunction in preterm infants after patent ductus arteriosus ligation. Journal of Thoracic and Cardiovascular Surgery 2012: 143 (6): pp Sehgal AS, McNamara PJ. Indomethacin administration for ductal closure in preterm infants causes decline in coronary perfusion. Neonatology 2011: 101 (1): pp (Epub ahead of print) 2011 Jul 26. Sehgal AS, Osborn D, McNamara PJ. Cardiovascular support in preterm infants: A survey of practices in Australia and New Zealand. Journal of Pediatrics and Child Health 2012: 48 (4): pp : Nov 16. doi: /j x. (Epub ahead of print). Seitz D, Shah PS, Herrmann N, Beyene J, Siddiqui N. General anesthesia and risk of Alzheimer s disease: A systematic review and meta-analysis. BMC Geriatrics 2011: 11 (1): pp 83. Sewing C, Kantores C, Ivanovska J, Lee A, Masood A, Jain A, McNamara PJ, Tanswell AK, Jankov RP. Therapeutic hypercapnia prevents bleomycin-induced pulmonary hypertension in neonatal rats by limiting macrophage-derived tumor necrosis factor-α. American Journal of Physiology 2012: 303: pp L75-L87. Sgro M, Shah PS, Campbell D, Tenuta A, Shivananda S, Lee SK and The Canadian Neonatal Network. Early-onset neonatal sepsis: Rate and organism pattern between 2003 and Journal of Perinatology 2011:31 (12): pp Shah PS, Balkhair T, Ohlsson A, Beyene J, Scott F, Frick C on behalf of Knowledge Synthesis Group on LBW/Preterm Birth. Intention to become pregnant and low birth weight and preterm birth: A systematic review. Maternal and Child Health Journal 2011: 15: pp Shah PS, Ohlsson A. Sildenafil for pulmonary hypertension in neonates. Cochrane Database of Systemic Reviews 2011: (8):CD the hospital for sick children department of paediatrics Annual Report

61 Shah PS, Sankaran K, Aziz K, Allen AC, Seshia M, Ohlsson A, Lee SK, Canadian Neonatal Network, Andrews W, Barrington K, Yee W, Bullied B, Canning R, Cronin G, Dow K, Dunn M, Harrison A, James A, Kalapesi Z, Kovacs L, da Silva O, McMillan DD, Shah P, Ojah C, Peliowski A, Aziz K, Piedboeuf B, Riley P, Faucher D, Rouvinez-Bouali N, Sankaran K, Seshia M, Shivananda S, Cieslak Z, Synnes A, Walti N. Outcomes of preterm infants <29 weeks gestation over 10-year period in Canada: A cause for concern? Journal of Perinatology 2012: 32 (2): pp Shah PS, Ye XY, Synnes A, Rouvinez-Bouali N, Yee W, Lee SK, Canadian Neonatal Network. Prediction of survival without morbidity for infants born at under 33 weeks gestational age: A user friendly graphical tool. Archives of Disease of Childhood. Fetal Neonatal Edition 2012: 97: pp F110-F doi: /archdischild-2 of Shah PS. Meta-analysis of neurodevelopmental outcome after room air versus 100% oxygen resuscitation: Generating more questions than answers? Neonatology 2012: 102 (2): pp Epub 2012 Jun 6. Shah PS, Yoon W, Kalapesi Z, Bassil K, Dunn M, Lee SK, and the Canadian Neonatal Network. Summer and infections in neonatal intensive care units: Seasonal variations in health care-associated infection in neonates in Canada. Archives of Disease in Childhood. Fetal and Neonatal Edition 2012: May 3 (Epub ahead of print). Shah V, Chirinian N, Lee S, EPIQ Evidence Review Group. Does the use of glycerin laxatives decrease feeding intolerance in preterm infants? Pediatrics and Child Health 2011: 16: pp e68-e70. Shah V, Jefferies A. Commentary on the article: Johnston CC, Campbell-Yeo M, Filion F. Paternal vs. maternal kangaroo care for procedural pain in preterm neonates: A randomized crossover trial. Archives of Pediatric and Adolescent Medicine 2011: 165 (9): pp Shah V, Jefferies A. Preterm infants receiving heel lance procedures have slightly lower pain scores and quicker time to return to baseline heart rate when held in kangaroo care by the mother than by the father. Evidence-Based Medicine 2012 Mar 7 (Epub ahead of print). Shah VS, Ohlsson A, Halliday HL, Dunn M. Early administration of inhaled corticosteroids for preventing chronic lung disease in ventilated very low birth weight preterm neonates. Cochrane Database of Systemic Reviews 2012 May: pp CD Shivananda S, Ahlawalia L, Kluckow M, Luc J, Jankov RP, McNamara PJ. Variation in the management of persistent pulmonary hypertension of newborn (PPHN): A survey of physicians in Canada, Australia and New Zealand. American Journal of Perinatology 2012: 29 (7): pp Steggerda SJ, de Bruïne FT, Smits-Wintjens VEHJ, Walther FJ, van Wezel-Meijler G. Ultrasound detection of posterior fossa abnormalities in full-term neonates. Early Human Development 2011: Sep 14 (Epub ahead of print). Stevens B, Riahi S, Cardoso R, Ballantyne M, Yamada J, Beyene J, Breau L, Camfield C, Finley GA, Franck L, Gibbins S, Howlett A, McGrath PJ, McKeever P, O Brien K, Ohlsson A. The influence of context on pain practices in the NICU: Perceptions of health care professionals. Qualitative Health Research 2011: 21 (6): pp Stevens BJ, Abbott LK, Yamada J, Harrison D, Stinson J, Taddio A, Barwick M, Latimer M, Scott SD, Rashotte J, Campbell F, Finley GA, CIHR Team in Children s Pain. Epidemiology and management of painful procedures in children in Canadian hospitals. Canadian Medical Association Journal 2011: 183 (7): pp E Teuffel O, Kuster SP, Hunger SP, Conter V, Hitzler J, Ethier MC, Shah PS, Beyene J, Sung L. Dexamethasone versus prednisone for induction therapy in childhood acute lymphoblastic leukemia: A systematic review and meta-analysis. Leukemia 2011: 25 (8): pp Tromp WF, Schoenmaker NJ, van der Lee JH, Offringa M, Groothoff JW, et al. Lessons learned from efforts to improve the quality of care in children with end-stage renal disease in the Netherlands and Belgium. Archives of Diseases in Childhood 2011: 96 (12): pp Tromp WF, Schoenmaker NJ, van der Lee JH, Adams B, Bouts AH, Collard L, Cransberg K, Van Damme-Lombaerts R, Godefroid N, van Hoeck K, Koster-Kamphuis L, Lilien MR, Raes A, Offringa M, Groothoff JW. Important differences in management policies for children with end-stage renal disease in the Netherlands and Belgium - Report from the RICH-Q study. Nephrology, Dialysis, Transplantation 2011: Oct 4 (Epub ahead of print). van Wezel-Meijler G, Leijser LM, Wiggers-de Bruïne FT, Steggerda SJ, van der Grond J, Walther FJ. Diffuse hyperechogenicity of the basal ganglia and thalami in preterm infants: A physiological finding? Radiology 2011: 258: pp van Wezel-Meijler G, Wiggers-de Bruïne FT, Steggerda SJ, van den Berg-Huysmans AA, Zijlemaker S, Leijser LM, van der Grond J. Ultrasound detection of white matter injury in very preterm neonates: Practical implications. Developmental Medicine and Child Neurology 2011: 53 (suppl 4): pp Weisz DE, Jain A, McNamara PJ, EL-Khuffash A. Non-invasive cardiac output monitoring in neonates using Bioreactance: A comparison with echocardiography. Neonatology 2012: 102 (1): pp Wiggers-de Bruïne FT, van Wezel-Meijler G, van den Berg- Huysmans AA, Leijser LM, van Steenis A, van Buchem MA, van der Grond J. Tractography of developing white matter in preterm born infants. European Radiology 2011: 21: pp Wong J, Dow K, Shah PS, Andrews W, Lee SK, Canadian Neonatal Network. Percutaneously placed central venous catheter related blood stream infections in Canadian NICUs: Impact on outcomes and variations. American Journal of Perinatology 2012: May 7 (Epub ahead of print). Yee W, Scotland J, Pham Y, Finch R, for the Canadian Neonatal Network EPIQ Study Group. Does primary continuous positive airway pressure (CPAP) reduce the need for intubation for assisted mechanical ventilation and surfactant use in VLBW infants? Paediatrics and Child Health 2011: 16: pp Yee W, Soraisham A, Shah V, Aziz K, Yoon WJ, Lee SK, Canadian Neonatal Network. Incidence and timing of presentation of necrotizing enterocolitis in preterm infants within NICUs across Canada. Pediatrics 2012: 129: pp e298-e304 Books and Chapters Hellmann J. Commentary: Bereaved parents perspectives of the autopsy examination of their child. In: 2011 Year Book of Neonatal and Perinatal Medicine (Fanaroff AA, Benitz WE, Donn SM, Neu J, Papile L-A, Polin RA, Van Marter LJ, eds.). Elsevier Mosby, Philadelphia 2011: pp Hellmann J. Commentary: Categorizing neonatal deaths: A crosscultural study in the United States, Canada and The Netherlands. In: 2011 Year Book of Neonatal and Perinatal Medicine (Fanaroff AA, Benitz WE, Donn SM, Neu J, Papile L-A, Polin RA, Van Marter LJ, eds.). Elsevier Mosby, Philadelphia 2011: pp Hellmann J. Commentary: Donation after cardiac death: The potential contribution of an infant organ donor population. In: 2011 Year Book of Neonatal and Perinatal Medicine (Fanaroff AA, Benitz WE, Donn SM, Neu J, Papile L-A, Polin RA, Van Marter LJ, eds.). Elsevier Mosby, Philadelphia 2011: pp Hellmann J. Commentary: Ethical complex decisions in the Neonatal Intensive Care Unit: Impact of the new French legislation on attitudes and practices of physicians and nurses. In: 2011 Year Book of Neonatal and Perinatal Medicine (Fanaroff AA, Benitz WE, Donn SM, Neu J, Papile L-A, Polin RA, Van Marter LJ, eds.). Elsevier Mosby, Philadelphia 2011: pp EL-Khuffash A, McNamara PJ. Cardiovascular disease in neonates. In: Textbook of Clinical Paediatrics, Second edition, (Stapleton FB, Whitley RJ, Oh W, Harfi HA, Nazer H, eds.). Lippincott Williams and Wilkins Offringa M. Methodology of follow up studies in breastfeeding. Chapter In: Een professionele kijk op borstvoeding (4599), (Anten-Kools EJ, van Wouwe JP, Oudesluys-Murphy AM, Semmekrot BA, eds.), Van Gorcum Offringa M, van der Lee H. Small sample approach, and statistical and epidemiological aspects. In: Handbook of Experimental Pharmacology (Seyberth HW et al, eds.), Springer-Verlag Berlin Heidelberg 2011:205: pp the hospital for sick children department of paediatrics Annual Report

62 Grants and Funding A CIPP framework for implementation and evaluation of a faculty development program for neonatal-perinatal medicine. Jefferies A, Finan E, Lee KS, Simmons B. Continuing Education Research and Development Grant, University of Toronto. $4,900 CAD to 2011 A critical appraisal of resuscitation practices in the NICU. Gibbins S, Dunn M, Asztalos E, Schurr P, Chow J, Kiss A. PBR Award Sunnybrook Health Sciences Centre. $12,358 CAD to 2011 A pilot study of serum angiopoietin as a predictive marker in the diagnosis of necrotizing enterocolitis in premature infants. Murthy S, Belik J. University of Toronto Postgraduate Research Awards. $10,000 CAD to 2012 A RCT to evaluate the impact of a fact sheet about childhood immunization pain management on parental knowledge. Shah V. Paediatric Consultants Grant. $5,000 CAD An environmental scan of weight assessment and management practices in paediatric spina bifida clinics across Canada. Church P. Spina Bifida and Hydrocephalus Association of Canada. $8,014 CAD Academic challenges for the preterm infant: Educator s knowledge, attitudes and identified barriers to providing aid to this population. Church P, Shah V, Basil K, Luther J, Lee SK. Physician Services Incorporated. $61,000 CAD to 2012 Antepartum care of high risk pregnancy team. Janssen P, Lee S, et al. Michael Smith Foundation for Health Research. $213,090 CAD to 2011 Annual Maternal-Fetal-Neonatal Research Day. Shah P. Ikaria Meeting. $5,000 CAD Can we change the face of immunization to one without pain? Implementation of a postnatal parental education intervention to optimize pain management during childhood immunization. Taddio A, Shah V, Ipp M, Franck L, Hetherington R, Stevens D. Canadian Institutes of Health Research. $100,000 CAD Canadian Child Health Clinician Scientist Program. Rosenblum N, Lee S, et al. Canadian Institutes of Health Research. $4,500,000 CAD to 2015 China-Canada Joint Health Research Initiative. CIHR team in neonatal clinical decision support: Real-time, multidimensional temporal analysis of complex high volume physiological data streams for the identification of nosocomial infection in the neonatal intensive care unit. McGregor C, James A, Lee S, Weiping W. Canadian Institutes of Health Research. $150,000 CAD to 2013 CHIPS-CHILD:Testing the developmental origins hypothesis. Magee L, Asztalos E. Canadian Institutes of Health Research. $1,190,436 CAD to 2015 CIHR team in children s pain. Stevens B, Lee S, et al. Canadian Institutes of Health Research. $4,054,611 CAD to 2011 CIHR team in maternal-infant care. Lee S. Canadian Institutes of Health Research $5,640,990 CAD to 2013 CIHR team in Pediatric Emergency Medicine. Klassen T, Lee S, et al. Canadian Institutes of Health Research. $3,845,904 CAD to 2011 Clinical prediction models for critically ill pregnant women: MEOWS and CIPHER. Von Dadelszen P, Lee S, et al. Canadian Institute of Health Research. $100,000 CAD to 2012 Comparison of 2% chlorhexidine in 70% isopropyl alcohol versus 2% aqueous chlorhexidine for skin antisepsis prior to venepuncture in very low birth weight infants: A planned non-inferiority trial. Shah V, Jain A, Desphande P, McGeer A. Physicians Services Incorporation Foundation. $136,000 CAD Control of hypertension in pregnancy study (CHIPS). Magee L, Asztalos E, Ross S, Hutton E, Murphy K, Logan S, Moutquin JM, Rey E, von Dadelszen P, Gruslin A, Helewa M, Lee S, Gafni A, Singer J. Canadian Institutes of Health Research. $3,743,575 CAD to 2014 Creating bone and joint health from the bedside to the bench and back. Frank C, Lee S, et al. Alberta Heritage Foundation for Medical Research. $5,000,000 CAD to 2013 Creating an innovative problem oriented documentation system to enhance clinical documentation for level 3 NICUs. Lee SK, James A, Ng E. ASickKids AFP Innovation Fund. $200,000 CAD to 2013 Developing and testing a prediction model for neurodevelopmental outcomes for the extremely preterm infant. Asztalos E. Alva Foundation. $250,000 CAD to 2015 Development and use of a standardized instrument for assessing children s competence to consent in drug trials: Are legally established age limits valid? Offringa M, et al. The Netherlands Organization for Health Research and Development ZonMW - Priority Medicines. $518,939 CAD to 2014 Development of a targeted neonatal echocardiography teaching application for smart phones and tablet PCs. El-Khuffash A. Ikaria Educational Grant. $35,000 CAD DO IT: Vitamin D outcomes and interventions in toddlers. Maguire JL. CIHR INMD Startup (Bridge) Funds. $100,000 CAD to 2012 DOMINO donor milk for improved neurodevelopmental outcomes. Unger S, O Connor D, Gibbins S, Azstalos E, Kiss A, Zupancic J. Canadian Institutes of Health Research. $920,270 CAD to 2015 Echocardiography and persistent pulmonary hypertension of the newborn. McNamara P (Supervising Investigator). School of Graduate Studies Fall Grant. $400 CAD Economic and mental burden of neural tube defect in Canada. Church P, Koren G. Bayer Schering Pharma. $80,000 CAD Effect of folic acid supplementation in pregnancy on preeclampsia. Wen S, Lee S, et al. Canadian Institutes of Health Research. $6,525,375 CAD to 2014 Effects of continuous positive airway pressure (CPAP) delivered by high flow nasal cannula (HFNC) versus nasal continuous positive airway pressure (NCPAP) on the diaphragm electrical activity in very low birth weight preterm infants. Ng E, Nasef N, El-Gouhary E, Schurr P, Reilly M, Beck J, Dunn M. Physicians Services Incorporated. $24,500 CAD to 2012 Effect of maternal skin-to-skin contact (SSC) in the delivery room on the skin colonization of preterm infants: A pilot randomized controlled study. Shah V. Physicians Services Incorporated. $11,850 CAD Efficacy and safety of methyxanthines in very low birth weight infants. Schmidt B, Asztalos E, Ohlsson A, et al. Canadian Institutes of Health Research. $2,038,135 CAD to 2015 Efficacy and safety of targeting lower arterial saturations to reduce oxygen toxicity and oxidative stress in very preterm infants: The Canadian Oxygen Trial (COT). Schmidt B, Asztalos E, Roberts R, Whyte R, Solimano A. Canadian Institutes of Health Research. $3,884,860 CAD to 2011 Electronic problem-oriented documentation (epod) for Level III neonatal intensive care. Lee S, Ng E, James A. Academic Health Sciences Alternative Funding Plan Innovation Fund : Ontario Ministry of Health and Long Term Care. $196,000 CAD to 2013 Enhanced surveillance of Canadian street youth. Barozzino T, Sgro M. Public Health Agency of Canada. $10,000 CAD to 2012 Enhancing breast milk production with domperidone in mothers of preterm neonates (EMPOWER Trial). Asztalos E. Canadian Institutes of Health Research. $999,454 CAD to 2016 EPIQ-II Project. James A (Site investigator, The Hospital for Sick Children site). Canadian Institutes of Health Research (Canadian Neonatal Network). $42,215 CAD to 2011 EPIQ-II Project. James A (Site investigator, The Hospital for Sick Children site). Canadian Institutes of Health Research (Canadian Neonatal Network). $10,000 CAD to 2012 Establishment of a Canadian-based international research network to improve quality of life, communication, and economic outcomes in head and neck cancer survivors. Rieger J, Lee S, et al. Canadian Institutes of Health Research. $100,000 CAD to the hospital for sick children department of paediatrics Annual Report

63 Evaluation of the education impact of debriefing post simulated neonatal resuscitation. Campbell D. Neonatal Resuscitation Program Grant, Canadian Pediatric Society. $16,200 CAD Family integrated care model for the neonatal intensive care unit: A cluster randomized trial. O Brien K, Lee S, Alvaro R, Srinivasan V, Asztalos E, Canning R, Catelin C, Da Silva O. Canadian Institutes of Health Research. $349,982 CAD to 2014 Family integrated care randomized trial. O Brien K, Lee S, Alvaro R, Srinivasan V, Asztalos E, Canning R, Catelin C, Da Silva O. Ministry of Health and Long-Term Care. $563,000 CAD to 2014 Fluconozole prophylaxis for the prevention of candidiasis in infants <750 grams birth weight. Benjamin D, Asztalos E, Smith P, Wade K. National Institute of Child Health and Human Development. $2,793,089 CAD to 2013 GentleR Study: A cluster randomized controlled trial. Lee SK, Shah V. ASickKids AFP Innovation Fund. $200,000 CAD to 2013 Global Research in Pediatrics: GRiP Network of Excellence. Offringa M, et al. EU, 7th Framework Program FP7. $12,973,482 CAD to 2015 Health law, ethics and policy training program. Flood C, Lee S, et al. Canadian Institutes of Health Research. $3,412,500 CAD to 2015 High-risk HIV newborn exposure in the inner city What goes in the decision to treat? Campbell D. SPRESS, Chair of Pediatrics, Student Research Fund. $5,000 CAD HOLD IT: Health outcomes from low vitamin D in toddlers. Maguire JL. CIHR Operating Grant. $563,000 CAD to 2014 Hypercapnia as therapy for neonatal pulmonary hypertension. Jankov RP. Canadian Institutes of Health Research Open Grants Program. $579,880 CAD to 2014 Impact of ACORN newborn education program in rural China. Du L, Singhal N, McMillan DD, Aziz K, Lee SK. Canadian Institutes of Health Research. $180,000 CAD to 2011 Implementation of a wireless positive patient identification system for the prevention of breast milk errors in the NICU. Unger S, O Brien K, Kotsopoulos K. Ministry of Health Innovation Funds. $60,014 CAD to 2012 Improving neonatal critical care through real-time analysis. McGregor C, James A. Canadian Institutes of Health Research. $315,186 CAD to 2013 Improving parental opinion on retrotransfer through effective discharge planning. Diambomba Y. ASickKids AFP Innovation Funds. $62,000 CAD Improving safety in the insertion and care of percutaneously inserted central catheters (PICCs) and umbilical catheters (UCs) in 3 neonatal intensive care units (NICUs). Lee KS, Dunn M, Jefferies A, Gibbins S, Doctor S. Canadian Medical Protective Association Collaborative Research Grant. $49,750 CAD to 2011 Inhibition of physiological apoptosis as a major component of chronic neonatal lung injury. Tanswell AK, Jankov RP. Canadian Institutes of Health Research Open Grants Program. $477,180 CAD to 2012 Initiative for translational hypertension research. Branko B, Lee SK, et al. University of Alberta. $300,000 CAD to 2011 Late preterm births: Meeting planning and dissemination. Bassil K, Lee KS, Shah PS. Canadian Institutes of Health Research. $10,000 CAD Maternal hemodynamic changes in multiple gestation pregnancy. Ladhani N, Shah P. Department of Obstetrics and Gynecology Research Fund. $20,000 CAD to 2012 Maternal-Infant Care (MICare) Research Centre. Lee S. Ministry of Health and Long-Term Care. $15,000,000 CAD to 2013 Measuring late preterm health and developmental outcomes through TARGet Kids! Maguire JL, Sgro M, Campbell D. St. Michael s Hospital Innovation Fund. $18,694 CAD to 2012 Measurement of brain volumes by MRI segmentation in infants born preterm: Relation with mental outcome. Meijler G, Bicenter study University Medical Center Utrecht and Leiden University Medical Center. ZonMW, the Netherlands Organization for Health Research and Development. k to 2011 Milk intake, vitamin D deficiency and fracture risk in young children. Maguire JL. Dairy Farmer s of Canada. $90,000 CAD to 2012 MiTy Kids (Metform in women with type 2 diabetes in pregnancy Kids Trial). Feig D, Lee S, Asztalos E, et al. Canadian Institutes of Health Research. $1,299,072 CAD to 2018 MiTy Trial (Metform in women with type 2 diabetes in pregnancy trial). Feig D, Lee S, Asztalos E et al. Canadian Institutes of Health Research. $1,735,119 CAD to 2015 Moderately early hydrocortisone for ventilated preterm infants at risk for bronchopulmonary dysplasia: A multicenter randomized placebo controlled trial: STOP-BPD. Offringa M, et al. The Netherlands Organization for Health Research and Development ZonMW - Priority Medicines. $778,409 CAD to 2014 Multiple courses of antenatal corticosteroids for preterm birth study: A 5-year follow-up (MACS-5). Asztalos E, Gafni A, Hannah M, Murphy K, Ohlsson A, Ross S, Saigal S, Willan A, Matthews S, Rovet J, Lee S, Amankwah K, Kelly EN, Lee SK, Delisle M-F, Guselle P, Sananes R. Canadian Institutes of Health Research. $3,585,161 CAD to 2012 Neuroimaging and patent ductus arteriosus ligation. Taylor M, Sledge J, McNamara PJ. NSERC (Natural Sciences and Engineering Research Council of Canada). $384,734 CAD to 2012 Neuroimaging measures of brain function in preterm neonates. Taylor MJ, Sled JG, McNamara P, Whyte HEA, Moore AM, Donner EJ, Shroff MM. Collaborative Health Research Program (CHRP). $361,860 CAD to 2011 NICHD Cooperative Multicenter Neonatal Research Network. Frantz I, Church P. NIH. $1,290,746 CAD to 2011 Non-invasive cardiac output monitoring in neonates using bioreactance: A comparison with echocardiography. El-Khuffash A. School of Graduate Studies Conference Grant, University of Toronto. $500 CAD Non-invasive cardiac output monitoring in neonates using bioreactance: A comparison with echocardiography. El-Khuffash A. Paediatric Academic Society Young Investigator s Travel Awards Program.$500 CAD Nutrition research trainee support. O Connor D, Unger S. The Hospital for Sick Children Foundation. $40,000 CAD to 2013 Ontario CASE (congenital anomalies surveillance enhancement) initiative submission. Moore AM, Fell D. PHAC. $80,000 CAD annually to 2013 Oxidative stress and neonatal pulmonary vascular resistance control. Belik J. Canadian Institutes of Health Research 5 year operating grant. $597,193 CAD to 2014 Paediatric pharmacogenomics medicine (PPM). Ito S, Ungar WJ, Hitzler J, Hurdower A, Moore AM, Koren G. MOHLTC Drug Innovation Fund. $589,697 CAD to 2014 Pain in infants at risk for neurological impairment: A program of research: Phase 3. (PINI 3). Stevens B, Gibbins S, McGrath P, Johnston C, Asztalos E, O Brien K, Howlett A, McNamara P, Pillai Riddell R. Canadian Institutes of Health Research. $851,050 CAD to 2012 Pharmacokinetics and pharmacodynamics of medication in asphyxiated newborns during controlled hypothermia. PharmaCool National Multicenter Study. Offringa M, et al. The Netherlands Organization for Health Research and Development ZonMW - Priority Medicines. $778,409 CAD to 2014 Post graduate medical education research award winner. El-Khuffash A. University of Toronto. $9,900 CAD Preclinical assessment of Rho-kinase inhibitors for chronic pulmonary hypertension. Jankov RP, McNamara PJ. Canadian Institutes of Health Research Open Grants Program. $655,855 CAD to the hospital for sick children department of paediatrics Annual Report

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64 Predicting neurobehaviorial outcome in preterm neonates. Taylor M, Whyte HE, Moore A, Shroff M, Rayboud C, Donner E, Frisk V. Canadian Institute of Health Research. $929,910 CAD to 2012 Predictive value of biochemical and echocardiography markers in neonates with PLCS. McNamara P, El-Khuffash A, Jain A. Physicians Services Incorporated. $15,900 CAD to 2011 Preterm birth and healthy outcomes. Olson D, Lee S, et al. Alberta Heritage Foundation for Medical Research. $5,000,000 CAD to 2013 PROMOTE: Preschoolers at risk Obesity and cardiometabolic risk factors: Towards early identification. Maguire JL. CIHR Startup Grant. $100,000 CAD to 2011 PROMOTE: Preschoolers at risk Obesity and cardiometabolic risk factors: Towards early identification. Maguire JL. PSI Operating Grant. $168,000 CAD to 2012 Role of reactive oxygen/nitrogen species in normal and aberrant postnatal lung growth. Tanswell AK. Canadian Institutes of Health Research. $557,560 CAD to 2015 Simulation in perinatal resuscitation, a quality improvement project. Campbell D. Innovation Fund, Physicians Services Association, SMH. $20,460 CAD Surveillance of early-onset neonatal sepsis and meningitis. Sgro M, Campbell D. AFP Innovation Fund. $42,000 CAD to 2012 Surveillance of early-onset neonatal sepsis and meningitis. Sgro M, Campbell D. Department of Pediatrics University of Saskatchewan. $5,000 CAD to 2012 Surveillance of early-onset neonatal sepsis and meningitis. Sgro M, Campbell D. Draegar Medical. $10,000 CAD to 2012 Surveillance of early-onset neonatal sepsis and meningitis. Sgro M, Campbell D. Mi-CARE Sub-Grant. $20,000 CAD to 2011 Surveillance of severe hyperbilirubinemia. Sgro M, Campbell D. Public Health Agency of Canada. $40,000 CAD to 2013 Sustainability of a multidimensional knowledge translation intervention to improve paediatric pain practices and outcomes. Stevens B, Lee S, et al. Canadian Institute of Health Research. $822,401 CAD to 2015 The Artemis Project: Real-time streaming of high volume, high fidelity physiological data for clinical decision support. James A, McGregor C. TD Bank Information Technology Group Annual Fund Raising Event. $150,000 CAD to 2013 The Canadian Pediatric Surgery Network (CAPSNET): Establishing best practices for gastroschisis and congenital diaphragmatic hernia. Bouchard S, Lee S, et al. Canadian Institutes of Health Research. $100,000 CAD to 2011 The development of an interactive web based educational module for neonatal functional echocardiography. Finan E, McNamara P. Deans Education Development Fund. $10,000 CAD to 2011 The effect of remote ischemic preconditioning on clinical and genomic responses to open heart surgery in children. Redington A, McCrindle B, Holtby H, Schwartz S, Van Arsdell G, Coles J, Ly L, Scherer S. Heart and Stroke Foundation of Ontario. $570,339 CAD to 2012 The first national retinopathy of prematurity survey: Formulating evidence-based screening recommendations. Sabri K, Lee S, et al. Canadian Institute of Health Research. $349,983 CAD to 2014 The Twin Birth Study: A multicentre randomized controlled trial comparing planned caesarean section with planned vaginal birth for twins at weeks gestation. Barrett J, Hannah M, Willan A, Ross S, Hutton E, Allen A, Joseph KS, Armson A, Farrell S, Ohlsson A, Asztalos E, Gafni A, Okun N, Leduc L. Canadian Institutes of Health Research. $8,608,045 CAD to 2011 The Twin Birth Study: A multicentre randomized controlled trial comparing planned caesarean section with planned vaginal birth for twins at weeks gestation. Barrett J, Hannah M, Willan A, Ross S, Hutton E, Allen A, Joseph KS, Armson A, Farrell S, Ohlsson A, Asztalos E, Gafni A, Okun N, Leduc L. Canadian Institutes of Health Research. $438,577 CAD to 2015 The use of echocardiographic and biochemical markers to predict low cardiac output following patent ductus arteriosus ligation in preterm infants. El-Khuffash A, McNamara P. The Physicians Services Incorporated Foundation Grant Award. $15,900 CAD to 2012 The use of NICOM in assessing the hemodynamic state of premature infants. Weisz D, Jain A, El-Khuffash A, McNamara P. Hospital for Sick Children Research Start-Up Grant Award. $3,000 CAD Safety and efficacy of methylxanthines in the very low birth weight infant: Effect on sleep on the school age child. Marcus C, Asztalos E. National Heart, Lung and Blood Institute (NHLBI). $267,567 CAD (yr 1) to 2013 SCREEN-ROP Study. Sabri K, Lee SK, Ells A, Shivanand S, Farrokhyar F (James A, Site investigator, The Hospital for Sick Children). Canadian Institutes of Health Research. $50 per subject to 2013 Severe neonatal hyperbilirubinemia after implementation of the Canadian Paediatric Society Guideline (2007). Shah V. Public Health Agency of Canada. $40,000 CAD TARGet Kids! Maguire JL. Operating Grant St. Michael s Foundation. $30,000 CAD to 2011 TARGet Kids!: A primary care research network in obesity prevention. Maguire JL. CIHR Emerging Team Grant in Childhood Obesity. $2,452,000 CAD to 2013 The Artemis Project: Real-time streaming of high volume, high fidelity physiological data for clinical decision support. James A, McGregor C. TD Bank Information Technology Group Annual Fund Raising Event. $150,000 CAD to 2012 The initiative for translational hypertension research: Tackling hypertension-related health management through knowledge translation, system re-design and direct translation of bench research to practice. Bramm B, Lee S, et al. University of Alberta. $300,000 CAD to 2011 The MASC (Medical and Social Complexity) screening tool derivation study phase 1 Tool construction and pilot testing. Maguire JL. Norman Saunders Complex Care Initiative. $43,000 CAD to 2012 The Women s Auxiliary Chair in Neonatology. Lee S. The Hospital for Sick Children. $350,000 CAD to 2013 Translating research on pain in children (TROPIC) study. Stevens B, Lee S, et al. Canadian Institutes of Health Research. $1,379,952 CAD to 2012 What is the impact of early childhood education and care (ECEC) program quality on children s developmental health? Perlman M, Shah PS. Canadian Institutes of Health Research. $56,040 CAD to the hospital for sick children department of paediatrics Annual Report

65 NEPHROLOGY overview Division Head Dr. Lisa Robinson Full-Time Dr. Christoph Licht Dr. Diane Hébert Dr. Elizabeth Harvey Dr. Norman Rosenblum Dr. Rulan Parekh Dr. Tino Piscione Dr. Valerie Langlois Part-Time Dr. Denis Geary Dr. Lindsay Teskey Dr. Rachel Pearl Dr. Seetha Radhakrishnan Adjunct Dr. Leo Levin Honorary Dr. Gerald Arbus Clinical Fellows Dr. Adil Albahhar Dr. Damien Noone Dr. Hilla Bahat Dr. Joana Dos Santos Dr. Khamisa Al-Mokali Dr. Mamdoh Alsehli Dr. Mandy Keijzer-veen Dr. Marsha Gooden Dr. Omar Alkandari Dr. Rebecca Spicer Dr. Sami Al-Baher In , the number of patients cared for by the Division of Nephrology, and the complexity of the care provided, continued to grow. The Nephrology Fellowship Training Program provides a unique combination of comprehensive clinical training and exposure to state-of-the-art scientific inquiry, and it attracts trainees from across the world. The Division is uniquely poised to lead in clinical, epidemiological, and translational research, and in basic scientific discovery. The research profile of the Division continues to soar, as demonstrated by the number of successful grant applications, the high quality of the journals in which the work is published, and the number of invitations to speak at leading national and international conferences. The Division is uniquely poised to lead in clinical, epidemiological, and translational research, and in basic scientific discovery. Administrative Staff Andrea Verdugo Bernard Singh Burnett Wint Dana Debernyj Juliet Rodrigo Ramnarine Mohabir department of paediatrics Annual Report

66 Honors and Awards Rosenblum, ND: Inaugural Visiting Professor, Centre for Molecular and Cellular Intervention (CMCI) and Department of Pediatrics, University of Utrecht, October Publications Bostrom MA, Kao WH, Li M, Abboud HE, Adler SG, Iyengar SK, Kimmel PL, Hanson RL, Nicholas SB, Rasooly RS, Sedor JR, Coresh J, Kohn OF, Leehey DJ, Thornley-Brown D, Bottinger EP, Lipkowitz MS, Meoni LA, Klag MJ, Lu L, Hicks PJ, Langefeld CD, Parekh RS, Bowden DW, Freedman BI, on behalf of the Family Investigation of Nephropathy and Diabetes Research Group. Genetic associations in African Americans with non-diabetic end-stage renal disease: Follow-up of a pooled GWAS. American Journal of Kidney Disease 2012: 59 (2): pp Chi L, Rosenblum ND. Investigating primary cilia in cultured metanephric mesenchyme cells. Methods in Molecular Biology 2012: 886: pp Feber J, Gaboury I, Ni A, Alos N, Arora S, Bell L, Blydt-Hansen T, Clarson C, Filler G, Hay J, Hébert D, Lentle B, Matzinger M, Midgley J, Moher D, Pinsk M, Rauch F, Rodd C, Shenouda N, Siminoski K, Ward LM, and the Canadian STOPP Consortium. Skeletal findings in children recently initiating glucocorticoids for the treatment of nephrotic syndrome. Osteoporosis International 2012: 23 (2): pp Frieling M, Williams A, Al Shareef T, Kala G, Teh JC, Langlois V, Allen U, Hébert D, Robinson LA. Novel influenza (H1N1) infection in pediatric renal transplant recipients: A single-center experience. Pediatric Transplantation 2012: 16 (2): pp Geary DF, Hodson EM, Craig JC. Interventions for bone disease in children with chronic kidney disease (Review). Evidence- Based Child Health: A Cochrane Review Journal 2011: 6 (2): pp Goyal A, Orth RC, Parekh RS, Wolfson T, Gamst A, Kuo MD. Endpoints for hemodialysis access procedures: Correlation between fistulography and intra-access blood flow measurements. Journal of Vascular Interventional Radiology 2011: 22 (12): pp Gross O, Licht C, Anders HJ, Hoppe B, Beck B, Tönshoff B, Höcker B, Wygoda S, Ehrich JHH, Pape L, Konrad M, Rascher W, Döetsch J, Müller-Wiefel DE, Hoyer P, and Study Group Members of the Gesellschaft für Pädiatrische Nephrologie (GPN), Knebelmann B, Pirson Y, Grunfeld J-P, Niaudet P, Cochat P, Heidet L, Lebbah S, Torra R, Friede T, Lange K, Müller GA, Weber M. Early angiotensin-converting enzyme inhibition in Alport syndrome delays renal failure and improves life expectancy. Kidney International 2012: 81 (5): pp Kashtan C, Ding J, Gregory M, Gross O, Heidet L, Knebelmann B, Rheault M, Licht C. Clinical practice recommendations for the treatment of Alport syndrome: A statement of the Alport Syndrome Research Collaborative. Pediatric Nephrology 2012 (Epub ahead of print). Koltin D, Rachmiel M, Wong BY, Cole DE, Harvey EA, Sochett E. Mild infantile hypercalcemia Diagnostic tests and outcomes. Journal of Paediatrics 2011: 159 (2): pp Lim SHL, Benseler S, Tyrrell P, Charron M, Harvey EA, Hébert D, Silverman E. Low bone mineral density is present in newly diagnosed pediatric systemic lupus erythematosus (psle) patients. Annals of Rheumatic Diseases 2011: 70 (11): pp Morgan G, Benson L, Geary DF. Middle aortic syndrome with renal involvement: A staged strategy to manage systemic hypertension. Catheterization and Cardiovascular Interventions 2012: 80 (2): pp E5-E8. Noone D, Al-Matrafi J, Tinckam K, Zipfel PF, Herzenberg AM, Thorner PS, Pluthero FG, Kahr WH, Filler G, Hébert D, Harvey EA, Licht C. Antibody mediated rejection associated with complement Factor H-related Protein 3/1 deficiency successfully treated with Eculizumab. American Journal of Transplantation 2012 (Epub ahead of print). Parker K, Burrows G, Nash H, Rosenblum ND. Discovering the hidden curriculum using program theory. Academic Medicine 2011: 86 (11): pp Petruzziello TN, Yuen DA, Page AV, Patel S, Soltyk A, Matouk CC, Wong D, Tsui AK, Turgeon PJ, Fish JE, Ho JJD, Steer BM, Khajoee V, Tigdi J, Lee WL, Motto DG, Advani A, Gilbert RE, Karumanchi SA, Robinson LA, Tarr PI, Liles WC, Brunton JL, Marsden PA. The CXCR4/CXCR7/SDF-1 pathway contributes to the pathogenesis of Shiga toxin-associated hemolytic uremic syndrome in humans and mice. Journal of Clinical Investigation 2012: 122 (2): pp Radhakrishnan S, Lunn A, Kirschfink M, Thorner P, Hébert D, Langlois V, Pluthero F, Licht C. Eculizumab and refractory membranoproliferative glomerulonephritis. New England Journal of Medicine 2012: 366 (12): pp Scialla JJ, Appel LJ, Astor BC, Miller ER III, Beddhu S, Woodward M, Parekh RS, Anderson CA. African American study of kidney disease and hypertension study group: Net endogenous acid production is associated with a faster decline in GFR in African Americans. Kidney International 2012: 82 (1): pp Scialla JJ, Leonard MB, Townsend RR, Appel L, Wolf M, Budoff MJ, Chen J, Lustigova E, Gadegbeku CA, Glenn M, Hanish A, Raj D, Rosas SE, Seliger SL, Weir MR, Parekh RS on behalf of the CRIC Study Group. Serum osteoprotegerin is associated with aortic pulse wave velocity in patients with chronic kidney disease. Clinical Journal of the American Society of Nephrology 2011: 6 (11): pp Temme J, Peters F, Lange K, Pirson Y, Heidet L, Torra R, Grunfeld J-P, Weber M, Licht C, Müller G-A, Gross O. Incidence of renal failure and nephroprotection by RAAS inhibition in heterozygous carriers of X-chromosomal and autosomal recessive Alport mutations. Kidney International 2012: 81 (8): pp Waters AM, Wu MYJ, Huang YW, Liu GY, Holmyard D, Onay T, Jones N, Egan SE, Robinson LA, Piscione TD. Notch promotes dynamin-dependent endocytosis of nephrin. Journal of the American Society of Nephrology 2012: 23 (1): pp Books and Chapters Chaturvedi S, Licht C. Hemolytic uremic syndrome. In: Clinician s Manual of Pediatric Nephrology (Chand DH, Valentini RP, eds.). World Scientific Publishing Co. Pte. Ltd., Singapore 2011: pp Hothi DK, Harvey EA. Common complications of hemodialysis. In: Pediatric Dialysis, Second Edition (Warady BA, Schaefer F, Alexander SR, eds.). Springer Science and Business Media, LLC, New York, NY 2012: pp Mukovozov I, Robinson LA. Slit/Robo signaling: Inhibition of directional leukocyte migration. In: Protein Structure (Faraggi E, ed.). InTech Open Access Publisher, Rijeka, Croatia 2012: pp Müller D, Geary DF. Intensified hemodialysis in children. In: Pediatric Dialysis, Second Edition (Warady BA, Schaefer F, Alexander SR, eds.). Springer Science and Business Media, LLC, New York, NY 2012: pp Patel S, Mukovozov I, Robinson LA. Assessment of the recycling of the membrane-bound chemokine, CX3CL1. In: Methods in Molecular Biology (Booth J, Rast J, eds.). Springer Science and Business Media, LLC, New York, NY 2011: 748: pp Rosenblum ND. Malformation of the kidney: Structural and functional consequences. In: Brenner and Rector s The Kidney, Ninth Edition (Brenner BM, Chertow G, Marsden P, Skorecki K, Taal M, Yu ASL, eds.). Elsevier Health Sciences, Philadelphia, PA 2011: pp Srisuwan K, Geary DF. Pediatric hemodialysis. In: Clinician s Manual of Pediatric Nephrology (Chand DH, Valentini RP, eds.). World Scientific Publishing Co. Pte. Ltd., Singapore 2011: pp VanDe Voorde RG III, Geary DF. Initiation of maintenance renal replacement therapy in children. In: Pediatric Dialysis, Second Edition (Warady BA, Schaefer F, Alexander SR, eds.). Springer Science and Business Media, LLC, New York, NY 2012: pp the hospital for sick children department of paediatrics Annual Report

67 Grants and Funding Alport SyndromeTreatments and Outcomes Registry (ASTOR). A prospective study of microalbuminuria in untreated boys with Alport syndrome. Licht C, Kashtan CE. ASTOR Study University of Minnesota. $36,000 USD. Jan 2009 to Dec 2012 Atypical hemolytic uremic syndrome: A new pathological role for complement and coagulation control in platelets. Licht C, Kahr WHA. Heart and Stroke Foundation of Ontario (HSFO). $290,704 CAD. Jul 2009 to Jun 2012 Canada Research Chair (Tier II). Robinson LA. Canada Research Chairs Program. $500,000 CAD. Jan 2010 to Jan 2015 Canada Research Chair in Developmental Nephrology (Tier I). Rosenblum ND. Canada Research Chairs Program. $150,000 CAD. Jun 2012 to Jun 2017 Canadian Child Health Clinician Scientist Program. Rosenblum ND. Strategic Training Initiative in Health Research (STIHR) Program. $3,000,000 CAD to 2012 Canadian Paediatric Clinician Scientist Program. Rosenblum ND. The Hospital for Sick Children Foundation. $3,000,000 CAD to 2012 Canadian Paediatric Clinician Scientist Program. Rosenblum ND. B.C. Research Institute for Children s and Women s Health. $1,500,000 CAD to 2012 Canadian Paediatric Clinician Scientist Program. Rosenblum ND. Women s and Children s Health Research Institute. $140,000 CAD to 2012 Canadian Paediatric Clinician Scientist Program. Rosenblum ND. Manitoba Institute of Child Health. $200,000 CAD to 2012 CIHR MD/PhD Grant. Rosenblum ND. Canadian Institutes of Health Research. $660,000 CAD to 2012 CIHR MD/PhD Grant. Rosenblum ND. Canadian Institutes of Health Research. $660,000 CAD to 2013 CIHR MD/PhD Grant. Rosenblum ND. Canadian Institutes of Health Research. $660,000 CAD to 2014 CIHR MD/PhD Grant. Rosenblum ND. Canadian Institutes of Health Research. $660,000 CAD to 2015 CIHR MD/PhD Grant. Rosenblum ND. Canadian Institutes of Health Research. $660,000 CAD to 2016 CIHR MD/PhD Grant. Rosenblum ND. Canadian Institutes of Health Research. $660,000 CAD to 2017 Control of cerebellum cell specification, migration and organization by SUFU. Rosenblum ND. Canadian Institutes of Health Research. $734,120 CAD. Jun 2011 to Jun 2016 Control of complement-mediated platelet activation in ahus via Eculizumab. Licht C. Alexion Pharmaceuticals, Inc. $15,000 USD. Jan 2012 to Dec 2013 Control of kidney cell determination and morphogenesis by BMPs and beta-catenin. Rosenblum ND. Canadian Institutes of Health Research. $767,080 CAD. Jan 2008 to Sep 2013 Conversion of stable renal transplant patients from immediaterelease Tacrolimus (Prograf) to extended release Tacrolimus (Advagraf). Hébert D, Robinson LA, Langlois V, Nalli N, Leung C, Pollock-BarZiv S. Astellas Pharma Canada Inc. $69,465 CAD. Mar 2009 to Dec 2011 Dysregulated hedgehog signaling and the pathogenesis of nonobstructive and obstructive hydronephrosis. Rosenblum ND. The Kidney Foundation of Canada. $100,000 CAD. Jul 2010 to Jun 2012 Eculizumab in Alport Syndrome. Licht C. The Kidney Foundation of Canada (Alport Syndrome Research Grant). $100,000 CAD. Jan 2012 to Dec 2013 Genetic screening of children with nephrotic syndrome. Parekh RS. Physician Services Incorporated. $170,000 CAD. May 2011 to Apr 2013 Ischemia reperfusion injury therapeutic agonistic anti Robo 1 antibody. Robinson LA. MaRS Innovation Proof of Principle Program. $100,000 CAD. Mar 2012 to Mar 2013 Ischemia reperfusion injury therapeutic Agonistic anti Robo 1 antibody. Robinson LA. MaRS Innovation Proof of Principle Program. $50,000 CAD. Jun 2011 to Mar 2012 Longitudinal study of dialysis (PACE study). Parekh RS. National Institutes of Health/National Institute of Diabetes and Digestive and Kidney Diseases. $2,260,165 USD. Sep 2007 to Sep 2012 Modulation of vascular injury by the motility regulators, slit and roundabout. Robinson LA. Canadian Institutes of Health Research. $616,945 CAD. Jul 2011 to Jun 2016 Multi-center controlled clinical trials in Alport syndrome A feasibility study. Kashtan CE, Licht C. National Institutes of Health (Exploratory/Developmental Research Grant Award). $47,898 USD. Jan 2012 to Dec 2014 Multi-mode spinning disc confocal platform for live cell imaging. Robinson LA, Klip A, Trimble W. Canada Foundation for Innovation. $964,325 CAD. Jul 2010 to Jun 2012 Omega 3 fatty acid supplementation to treat hypertriglyceridemia in children with chronic kidney disease. Secker D, Harvey EA, Vigneux A. The Hospital for Sick Children, Complementary and Alternative HealthCare and Paediatrics Research Grants Competition. $48,993 CAD. May 2008 to Jan 2012 Pediatric sleep disorders in children receiving dialysis. Amin R, Harvey EA, Narang I, Al-Mokali K. Pediatric Consultants Grant, SickKids. $9,520 CAD. May 2012 to May 2013 Primary EBV infection in the Immunosuppressed setting of organ transplantation: Determinants of the outcomes of virushost interaction. Allen UD, Beyene J, Dipchand AI, Grunebaum E, Hébert D, Husain S, Mazzulli T, Ng VL, Read SE, Robinson JL, Tellier R, Weitzman SS. Canadian Institutes of Health Research. $509,125 CAD. Apr 2010 to Oct 2014 Regulation of leukocyte-endothelial interactions. Robinson LA. Natural Sciences and Engineering Research Council of Canada. $170,000 CAD. Jun 2012 to Jun 2017 TAKE IT: Teen Adherance in Kidney Transplant Effectiveness of Intervention Trial. Foster B, Furth S, Hébert D. National Institutes of Health. $757,183 USD. Sep 2011 to May 2016 The aging kidney in HIV-infection: Biomarkers for early detection of injury. Shlipak MG, Parekh RS. National Institutes of Health/National Institute of Diabetes and Digestive and Kidney Diseases. $2,464,480 USD. May 2010 to 2015 The biology of chronic preconditioning: Genomic and physiologic mechanisms of response. Redington A, McCrindle B, Robinson LA. Canadian Institutes of Health Research. $200,000 CAD. Jul 2010 to Jun 2012 The nephroprotective potential of eculizumab using COL4A3 knockout (nephrosclerotic) mice in a proof-of-concept study. Licht C. Alexion Pharmaceuticals, Inc. $65,000 CAD. Jan 2011 to Dec 2013 The role of single nucleotide polymorphisms of iron regulatory genes in chronic kidney disease and cancer treatment induced anemia susceptibility and response to therapy. DeAngelis C, Parekh RS. McLaughlin Centre (Accelerator Grant). $40,000 CAD. May 2012 to Apr 2013 Translational studies of the liver disease in autosomal recessive polycystic kidney disease. Rosenblum ND, Kamath B. Liam s Light Foundation. $190,000 CAD. May 2011 to Jun the hospital for sick children department of paediatrics Annual Report

68 NEUROLOGY overview Division Head Dr. O. Carter Snead Full-Time Dr. Rand Askalan Dr. Brenda Banwell Dr. Gabrielle deveber Dr. Elizabeth Donner Dr. Cecil Hahn Dr. Daune MacGregor Dr. Berge Minassian Dr. Mahendranath Moharir Dr. Ingrid Tein Dr. Jiri Vajsar Dr. Shelly Weiss Dr. Ann Yeh Part-Time Dr. Danielle Andrade Dr. Jonathan Gladstone Dr. Cristina Go Dr. Hans Katzberg Dr. Jeffrey Kobayashi Dr. Betty Koo Dr. Wayne Langburt Dr. Christine Lay Dr. William Logan Dr. Gilbert Miller Dr. Peter Rumney Dr. Teesta Soman Cross-Appointees Dr. Evdokia Anagnostou Dr. Darcy Fehlings Dr. Golda Milo-Manson Dr. Wendy Roberts Dr. Elysa Widjaja Dr. Grace Yoon Scientific Staff Dr. Douglas Cheyne Dr. Miguel Cortez Dr. Elizabeth Kerr Dr. Maureen Lovett Dr. Ayako Ochi Dr. Hiroshi Otsubo Dr. Elizabeth Pang Dr. Jose Luis Perez Velazquez Dr. Mary Lou Smith Dr. Margot Taylor Dr. Robyn Westmacott Honourary Dr. David Berbrayer Dr. Flavio Coceani Dr. Katarina Haka-Ikse Dr. E. Gordon Murphy Dr. Diann Superina-Bell Residents Dr. Aqeela Alhashim Dr. Aly Aziz Dr. Ahmed Bamaga Dr. Cyrus Boelman Dr. John Brandsema Dr. Sarah Ironside Dr. Ana Marissa Lagman-Bartolome Dr. Carolyn Lai Dr. Navin Mishra Dr. Maryam Nabavi Nouri Dr. Dragos Nita Dr. Lance Rodan Dr. Jacob Shin Research Fellows and Associates Dr. Mahmoud Mohammadi Dr. Kazuo Okanari (Neurophysiology) Dr. Tohru Okanishi (Neurophysiology) Dr. Satoru Sakuma (Neurophysiology) Clinical Fellows Dr. Kevin Charles Jones Dr. Yair Sadaka Subspecialty Fellows Dr. Reem Al-Khater (Epilepsy) Dr. Ali Al-Otaibi (Epilepsy) Dr. Samiah Al-Zaidy (Neuromuscular) Dr. Andrea Andrade (Stroke) Dr. Sandi Bigi (Demyelinating Disorders) Dr. Maina Kava (Neuromuscular) Dr. Chusak Limotai (Epilepsy) Dr. Naila Makhani, (Demyelinating Disorders) Dr. Iris Noiman (Epilepsy) Dr. Eric Payne (Neurocritical Care) Dr. Ismail Serafi (EMG/Neurophysiology) Administrative Staff Vicki Corris Erin Crawford Maureen Hodge Fatima Ledo Marilyn McLaughlin Susy O Neill Wendy Ricketts Ramona Zacharias Barbara Zimnowodzki The major foci of the clinical activities of the Division of Neurology have traditionally been epilepsy, stroke, headache, neurometabolic disease, neuromuscular disease, neurogenetics, sleep disorders, movement disorders, demyelinating diseases, functional neuroimaging and neurointensive care. While maintaining these strengths, the Division of Neurology will add a significant strength in Neonatal Neurology with the arrival of our new Division Head, Dr. Steven Miller, and a new assistant professor in the Division, Dr. Emily Tam. Drs. Miller and Tam will bring remarkable expertise in neonatal neurology to the Division and make that a world-class domain immediately. Collaboration to improve the care of children with neurological disease. The Division of Neurology has a Royal College accredited training program in Child Neurology as well as postdoctoral training programs in epilepsy, stroke, neurometabolic disease and demyelinating diseases. In addition, there are a number of graduate students and postdoctoral fellows in clinical neurophysiology, neurogenetics, molecular and cellular neurobiology. The research foci of the Division of Neurology is epilepsy (both clinical and basic research), magnetoencephalography, stroke, dyslexia, burden of disease and quality of life, muscular dystrophy, multiple sclerosis, fatty acid oxidation disorders, molecular and cellular neurobiology, and as noted above, neonatal neurology. department of paediatrics Annual Report

69 Honours and Awards Aziz, A: The CanMEDs Trainee Award, Division of Neurology, The Hospital for Sick Children,June, Banwell, B: The CanMEDs Faculty Award, Division of Neurology, The Hospital for Sick Children, June, Banwell, B: The William A. Hawke Award, Clinical Excellence in Teaching, Division of Neurology, The Hospital for Sick Children, June, Cortez, MA: Winner of Seizure Editor s Choice Certificate and Editorial Comment. Mirzoev A, Bercovici E, Stewart LS, Cortez MA, Snead OC III, Desrocher M. Circadian profiles of focal epileptic seizures: A need for reappraisal. Seizure (6): Editorial comment by Dr. Tobias Loddenkemper. Chrono-epileptology: Time to reconsider seizure timing. 2012:21(6):411. Cortez, MA: Appointed for a second term, Chairman, Canadian Board of Registration of EEG Technologists, 2009 to Donner, E: Winner of the Division of Developmental Paediatrics, Dept. of Paediatrics Teaching Award, University of Toronto, June, Donner, E: Appointed Secretary Treasurer, Canadian League Against Epilepsy, January, Donner, E: Steering Committee Member (Planned the inaugural meeting June 2012), PAME (Partners Against Mortality in Epilepsy) International, Hahn, C: Elected Fellow, American Clinical Neurophysiology Society, Hahn, C: Elected President, 2011 to 2013, EEG Section, Canadian Society of Clinical Neurophysiologists. Hahn, C: Scientific Program Chair, Annual Meeting of American Clinical Neurophysiology Society, San Antonio, TX, Hahn, C: Elected Councilor, American Clinical Neurophysiology Society, Kobayashi, J: 27 Years Service, SickKids Pediatric Consultants, June Makhani, N: William J. Logan Award for Excellence in Clinical Teaching, Division of Neurology, The Hospital for Sick Children, Moharir, M: Nominated for PAIRO Excellence in Clinical Teaching Award, Moharir, M: Junior Faculty Award for Clinical Excellence in Paediatric Medical Care, Department of Paediatrics, University of Toronto, the Hospital for Sick Children, Nita, D: American Academy of Neurology, Annual Meeting Resident Scholarship Recipient, New Orleans, Nita, D: Prichard Day Award for Residents, April, Payne, E: Prichard Day Award for Fellows, April, Somaru, S: Best Poster Presentation, Canadian Association of Electroneurophysiology Technologists (CAET), Ottawa, Tein, I: President-Elect, International Child Neurology Association, ; President Tein, I: Winner of John H. Menkes Award, Endowed Memorial Lectureship in Pediatric Neurology, Dept. of Pediatric Neurology, Ronald Regan Medical Center, UCLA, California, USA, March Tein, I: Co-Chair, Scientific Program Committee (responsible for entire Congress program). XIIth International Child Neurology Congress, Brisbane, Australia, June 2010 to June 1, 2012 Tein, I: Chair, Research Task Force, International Child Neurology Association, November 2011 to June Weiss, S: Elected President, Canadian Sleep Society, Sept 2011 (3 year term). Weiss, S: Nominated to University of Toronto Academic Board - representative for Faculty of Medicine, April 2011 (2 year term). Yeh, A: Finalist: CNF Scientific Award. Exercise for fatigue and depression in pediatric MS. Child Neurology Foundation, Yeh, A: Short list. Poster Prize ECTRIMS Amsterdam, October, Publications Akiyama T, McCoy B, Go C, Ochi A, Elliott IM, Akiyama M, Donner EJ, Weiss SK, Snead OC, Rutka JT, Drake JM, Otsubo H. Focal resection of fast ripples on extraoperative intracranial EEG improves seizure outcome in pediatric epilepsy. Epilepsia 2011: 52 (10): pp Akiyama T, Osada M, Isowa M, Go CY, Ochi A, Elliott IM, Akiyama M, Snead OC, Rutka JT, Drake JM, Otsubo H. High kurtosis of intracranial electroencephalogram as a marker of ictogenicity in pediatric epilepsy surgery. Clinical Neurophysiology 2011: 123: pp Al-Zaidy S, Baskin B, Hawkins C, Yoon G, Ray PN, Vajsar J. Milder phenotype of congenital muscular dystrophy in a novel POMT1 mutation. Muscle and Nerve 2012: 45 (5): pp Ameis SH, Fan J, Rockel C, Voineskos AN, Lobaugh NJ, Soorya L, Wang AT, Hollander E, Anagnostou E. Impaired structural connectivity of socio-emotional circuits in autism spectrum disorders: A diffusion tensor imaging study. Public Library of Science One (PLoS One) 2011: 6 (11): e Epub 2011 Nov 23. PubMed PMID: ; PubMed Central PMCID: PMC Anagnostou E, Chaplin W, Watner D, Silverman JM, Smith CJ, Zagursky K, Kryzak LA, Corwin TE, Feirsen N, Tanel N, Hollander E. Factor analysis of repetitive behaviors in autism as measured by the Y-BOCS. Journal of Neuropsychiatry and Clinical Neuroscience 2011: 23 (3): pp Anagnostou E, Hansen R. Medical treatment overview: Traditional and novel psycho-pharmacological and complementary and alternative medications. Current Opinion in Pediatrics 2011: 23 (6): pp Appendino JP, McNamara PJ, Keyzers M, Stephens D, Hahn CD. The impact of amplitude-integrated electroencephalography on NICU practice. Canadian Journal of Neurological Sciences 2012: 39 (3): pp Askalan R, Wang C, Shi H, Armstrong E, Yager JY. The effect of postischemic hypothermia on apoptotic cell death in the neonatal rat brain. Developmental Neuroscience 2011: 33 (3-4): pp Aubert-Broche BM, Fonov V, Ghassemi R, Narayanan S, Arnold DL, Banwell B, Sled JG, Collins L. Regional brain atrophy in children with multiple sclerosis. NeuroImage 2011: 58 (2): pp Banwell B. Editorial: Multiple sclerosis in children. Multiple Sclerosis and Related Disorders 2012: 1 (1): pp 3-5. Banwell B, Dale RC. Editorial: Lymphohistiocytosis in the brain: Recognition of a potentially reversible aspect of primary HLH. Neurology 2012: 78 (15): pp Banwell B, Giovannoni G, Hawkes C, Lublin F. Editor s Welcome. Multiple Sclerosis and Related Disorders 2012: 1 (1): pp 1-2. Bernard TJ, Manco-Johnson MJ, Lo W, Mackay MT, Ganesan V, deveber G, Goldenberg NA, Armstrong-Wells J, Dowling MM, Roach ES, Tripputi M, Fullerton HJ, Furie KL, Benseler SM, Jordan LC, Kirton A, Ichord R. Towards a consensus-based classification of childhood arterial ischemic stroke. Stroke 2012: 43 (2): pp Beslow LA, Kasner SE, Smith SE, Mullen MT, Kirschen MP, Bastian RA, Dowling MM, Lo W, Jordan LC, Bernard TJ, Friedman N, deveber G, Kirton A, Abraham L, Licht DJ, Jawad AF, Ellenberg JH, Lautenbach E, Ichord RN. Concurrent validity and reliability of retrospective scoring of the Pediatric National Institutes of Health Stroke Scale. Stroke 2012: 43 (2): pp Bethune A, Tipu V, Sled JG, Narayanan S, Arnold DL, Mabbott D, Rockel C, Ghassemi R, Till C, Banwell B. Diffusion tensor imaging of white matter in children with multiple sclerosis. Journal of the Neurological Sciences 2011: 309 (1-2): pp the hospital for sick children department of paediatrics Annual Report

70 Bomba MD, Choly D, Pang EW. Phoneme discrimination and mismatch negativity in English and Japanese speakers. Neuroreport 2011: 22: pp Bowers KJ, deveber G, Ferriero D, Roach S, Vexler Z, Maria BL. Cerebrovascular disease in children: Recent advances in diagnosis and management. Journal of Child Neurology 2011: 26 (9): pp Bruce-Barrett C, Hodinott A, Manicat-Emo A, Flaming T, MacGregor D, Hogan I, Carew C, Cox S. Kids in transition: The rehab experience. Healthcare Quarterly 2011: Spec No 3: pp Chitnis T, Tenembaum SN, Banwell B, Krupp LB, Pohl D, Rostasy K, Yeh EA, Bykova O, Wassmer E, Tardieu M, Kornberg AJ, Ghezzi A, for the International Pediatric MS Study Group (IPMSSG). Consensus statement: Evaluation of new and existing therapeutics for pediatric multiple sclerosis. Multiple Sclerosis 2011: 18 (1): pp Cohen JA, Reingold SC, Polman CH, Wolinksy JS, Banwell B, on behalf of the International Advisory Committee on Clinical Trials in Multiple Sclerosis. Disability outcomes in multiple sclerosis clinical trials: Current status and future prospects. Lancet Neurology 2012: 11 (5): pp Coutinho J, de Bruijn SF, deveber G, Stam J. Anticoagulation for cerebral venous sinus thrombosis. Cochrane Database of Systematic Reviews 2011: (8): CD Dhaunchak AS, Becker C, Schulman H, De Faria O, Rajasekharan S, Banwell B, Colman Dr. Bar-Or A. Implication of perturbed axoglial apparatus in early pediatric multiple sclerosis. Annals of Neurology 2012: 71 (5): pp Dlamini N, Freeman JL, Mackay MT, Hawkins C, Shroff M, Fullerton HJ, deveber G. Intracranial dissection mimicking transient cerebral arteriopathy in childhood arterial ischemic stroke. Journal of Child Neurology 2011: 26 (9): pp Dror Y, Donadieu J, Koglmeier J, Dodge J, Toiviainen-Salo S, Makitie O, Kerr E, Seilder C, Shimamura A, Shah N, Cipolli M, Kuijpers T, Durie P, Rommens J, Siderius L, Lieu JM. Draft consensus guidelines for diagnosis and treatment of Shwachman- Diamond syndrome. Annals of the New York Academy of Science 2011: 1242 (1): pp Duerden EG, Mak-Fan KM, Oatley HK, Taylor MJ, Roberts SW. Regional differences in grey and white matter in children and adults with Autism Spectrum Disorder: An ALE meta-analysis. Autism Research 2012: 5 (1): pp Duerden EG, Oatley HK, Mak-Fan KM, McGrath PA, Taylor MJ, Szatmari P, Roberts SW. Risk factors associated with self-injurious behaviours in children and adolescents with autism spectrum disorders. Journal ADD 2012: Mar 16 (Epub ahead of print). Farooq O, Faden H, Cohen M, Yeh EA. Neurologic complications of H1N1 Influenza A. Journal of Child Neurology 2012: 27 (4): pp Fattah A, Cypel T, Donner EJ, Wang F, Alaman BA, Zuker RM. The first successful lower-extremity transplantation: 6 year follow-up and implications for cortical plasticity. American Journal of Transplantation 2011: 11 (12): pp Friefeld SJ, Westmacott R, MacGregor D, deveber G. Predictors of quality of life in pediatric survivors of arterial ischemic stroke and cerebral sinovenous thrombosis. Journal of Child Neurology 2011: 26 (9): pp Fuentes A, Collins DL, Garcia-Lorenzo D, Sled JG, Narayanan S, Arnold DL, Banwell B, Till C. Memory performance and normalized regional brain volumes in patients with pediatric-onset multiple sclerosis. Journal of International Neuropsychological Society 2012: 18 (3): pp Fullerton HJ, Elkind MS, Barkovich AJ, Glaser C, Glidden D, Hills NK, Leiva-Salinas C, Wintermark M, deveber G. The vascular effects of infection in pediatric stroke (VIPS) study. Journal of Child Neurology 2011: 26 (9): pp Gianakopoulos PJ, Zhang Y, Pencea N, Orlic-Milacic M, Mittal K, Windpassinger C, White SJ, Kroisel PM, Chow EM, Saunders CJ, Minassian BA, Vincent JB. Mutations in MECP2 exon 1 in classical Rett patients disrupt MECP2_e1 transcription, but not transcription of MECP2_e2. American Journal of Medical Genetics Part B, Neuropsychiatric Genetics 2012: 159B (2): pp Girard JM, Stone SS, Lohi H, Blaszykowski C, Teixeira C, Turnbull J, Wang A, Draginov A, Wang P, Zhao XC, Ackerley CA, Frankland PW, Minassian BA. Phosphorylation prevents polyglucosan transport in Lafora disease. Neurology 2012: Epub May 23: PMID Girgis RR, Slifstein M, Xu X, Frankle WG, Anagnostou E, Wasserman S, Pepa L, Kolevzon A, Abi-Dargham A, Laruelle M, Hollander E. The 5-HT(2A) receptor and serotonin transporter in Asperger s disorder: A PET study with (¹¹C)MDL and (¹¹C)DASB. Psychiatry Research 2011: 194 (3): pp Epub 2011 Nov 12. PubMed PMID: ; PubMed Central PMCID: PMC Go CY, Mackay MT, Weiss SK, Stephens D, Adams-Webber T, Ashwal S, Snead OC III. Evidence-based guideline update: Medical treatment of infantile spasms: Report of the Guideline Development Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology 2012: 78 (24): pp Hahn CD, Manlhiot C, Schmidt MR, Nielsen TT, Redington AR. Remote ischemic per-conditioning: A novel therapy for acute stroke? Stroke 2011: 42: (10): pp Helderman JB, O Shea TM, Kuban KC, Allred EN, Hecht JL, Dammann O, Paneth N, McElrath TF, Onderdonk A, Leviton A: ELGAN study Investigators. Antenatal antecedents of cognitive impairment at 24 months in extremely low gestational age newborns. Pediatrics 2012: 129 (3): pp Hewson S, Kerr E, Feigenbaum A. Long term follow-up of two siblings with colbalamin E (MTRR) deficiency, one treated prenatally. Molecular Genetics and Metabolism 2011: (102): pp 290. Hollander E, Soorya L, Chaplin W, Anagnostou E, Taylor BP, Ferretti CJ, Wasserman S, Swanson E, Settipani C. A double-blind placebo-controlled trial of fluoxetine for repetitive behaviors and global severity in adult autism spectrum disorders. American Journal of Psychiatry 2012: 169 (3): pp Erratum in: American Journal of Psychiatry 2012: 169 (5): pp 540. PubMed PMID: Hung Y, Smith ML, Taylor M. Functional dissociations in prefrontal-hippocampal working memory systems. Cortex 2012: Hung Y, Smith ML, Taylor MJ. Development of ACC-amygdala activations in processing unattended fear. Neuroimage 2012: 60: pp Ibrahim GM, Akiyama T, Ochi A, Otsubo H, Smith ML, Taylor MJ, Donner E, Rutka JT, Snead OC, Doesburg SM. Public Library of Science One (PLoS One). 2012: 7 (6): e Epub 2012 Jun 21. PMID Ibrahim GM, Albert GW, Fallah A, Withers T, Otsubo H, Ochi A, Akiyama T, Donner EJ, Weiss S, Snead OC, Drake JM, Rutka JT. Occipital lobe epilepsy in children: Characterization, evaluation and surgical outcomes. Epilepsy Research 2012: 99 (30): pp Ibrahim GW, Fallah A, Snead OC, Elliott I, Drake JM, Bernstein M, Rutka JT. Ethical issues in surgical decision making concerning children with medically intractable epilepsy. Epilepsy Behavior 2011: 22: pp Inage Y, Halliday W, Ochi A, Go C, Akiyama T, Akiyama M, Widjaja E, Teitelbaum A, Belkas J, Brian J, Perez Velazquez JL. Histopathology of cortex and white matter in pediatric epileptic spasms: Comparison with those of partial seizures. Brain and Development 2012: 34 (2): pp Kadis DS, Pang EW, Mills T, Taylor MJ, McAndrews MP, Smith ML. Characterizing the normal developmental trajectory of expressive language lateralization using magnetoencephalography. Journal of the International Neuropsychology Society 2011: 17: pp the hospital for sick children department of paediatrics Annual Report

71 Kerbrat A, Aubert-Broche B, Fonov V, Narayanan S, Sled JG, Arnold DA, Banwell B, Collins DL. Reduced head and brain size for age and disproportionately smaller thalami in child-onset MS. Neurology 2011: 78 (3): pp Kim KJ, Pearl PL, Jensen K, Snead OC, Malspina P, Jakobs C, Gibson KM. Succinic semialdehyde dehyrdogenase: Biochemicalmolecular-clinical disease mechanisms, redox regulation, and functional significance. Antioid Redox Signal 2011: 15 (3): pp Kirshenbaum GS, Clapcote SJ, Duffy S, Burgess CR, Petersen J, Jarowek KJ, Yucel YH, Cortez MA, Snead OC, Vilsen B, Peever JH, Ralph MR, Roder JC. Mania-like behavior induced by genetic dysfunction of the neuron-specific Na+, K+-ATPase a3 sodium pump. Proceedings of the National Academy of Science of the United States of America 2011: 108: pp Kirton A, Armstrong-Wells J, Chang T, deveber G, Rivkin MJ, Hernandez M, Carpenter J, Yager JY, Lynch JK, Ferriero DM, International Pediatric Stroke Study Investigators. Symptomatic neonatal arterial ischemic stroke: The International Pediatric Stroke Study. Pediatrics 2011: 128 (6): pp e1402-e1410. Kitchen L, Westmacott R, Friefeld S, MacGregor D, Curtis R, Allen A, Yau I, Askalan A, Moharir M, Domi T, deveber G. The Pediatric Stroke Outcome Measure: A validation and reliability study. Stroke 2012: 43 (6): pp Kolski H, Vajsar J. Paediatric myasthenia. Canadian Paediatric Surveillance Program 2010 Results. Canadian Paediatric Society 2011: pp Kostelecki W, Garcia Dominguez L, Perez Velazquez JL. Single trial classification of magnetoencephalographic recordings using granger causality. Journal of Neuroscience Methods 2011: 199: pp Kushki A, Chau T, Anagnostou E. Handwriting difficulties in children with autism spectrum disorders: A scoping review. Journal of Autism and Developmental Disorders 2011: 41 (12): pp Latinus M, Taylor MJ. Discriminating male and female voices: Differentiating pitch and gender. Brain Topography 2012: 25 (2): pp Lax ID, Lin SY, Duerden EG, Chakravarty M, Donner EJ, Lerch JP, Taylor MJ. Neuroanatomical consequences of very preterm birth in middle childhood. Brain Structure and Function 2012: May 11 (Epub ahead of print) PMID Lee W, Donner EJ, Nossin-Manor R, Whyte HE, Sled JG, Taylor MJ. Visual functional MRI of premature infants. Developmental Medicine and Child Neurology 2012: Epub June 21. PMID: Lin DC, Perez Velazquez JL, Nenadovic V. Synchronous fluctuations in broad-band processes and application to electroencephalographic brain data. Europhysics Letters 2012: 97: pp Liu YC, Lowe H, Zak M, Chan VW, Kobayashi J, Donner EJ. Can children with hyperlipidemia receive the ketogenic diet therapy for medication resistant epilepsy. Neurology 2011: 11 (3): pp 188. Logan JW, O Shea TM, Allred EN, Laughon MM, Bose CL, Dammann O, Batton DG, Engelke SC, Leviton A, ELGAN Study Investigators. Early postnatal hypotension and developmental delay at 24 months of age among extremely low gestational age newborns. Archives of Disease in Childhood, Fetal Neonatal Edition 2011: 96 (5): pp F Logan JW, O Shea TM, Allred EN, Laughon MM, Bose CL, Dammann O, Batton DG, Kuban KC, Paneth N, Leviton A, ELGAN Study Investigators. Early postnatal hypotension is not associated with indicators of white matter damage or cerebral palsy in extremely low gestational age newborns. Journal of Perinatology 2011: 31 (8): pp Lovett MW, Lacerenza L, De Palma M, Frijters JC. Evaluating the efficacy of remediation for struggling readers in high school. Journal of Learning Disabilities 2012: 45 (2): pp Luca N, Daengsuwan T, Dalmau J, Jones K, deveber G, Kobayashi J, Laxer RM, Benseler S. Anti-N-methly-D-aspartate receptor encephalitis: A newly recognized inflammatory brain disease in children. Arthritis and Rheumatism 2011: 63 (8): pp Mak-Fan KM, Morris D, Vidal J, Anagnostou E, Roberts W, Taylor MJ. White matter and development in children with an autism spectrum disorder. Autism 2012: Jun 14. (Epub ahead of print) PubMed PMID: Mak-Fan KM, Taylor MJ, Roberts W, Lerch JP. Measures of cortical grey matter structure and development in children with Autism Spectrum Disorder. Journal of Autism and Developmental Disorders 2012: 42 (3): pp Makhani N, Banwell B. Environmental risk factors for pediatric multiple sclerosis. European Neurological Review 2011: 6 (3): pp Makhani N, Banwell B. Noninfectious central nervous system inflammatory disorders. Seminars in Pediatric Neurology 2011: 18 (2): pp McCoy B, Sharma R, Ochi A, Go C, Otsubo O, Hutchison JS, Atenafu EG, Hahn CD. Predictors of nonconvulsive seizures among critically ill children. Epilepsia 2011: 52 (11): pp Mills T, Lalancette M, Moses SN, Taylor MJ, Quraan MA. Techniques for detection and localization of weak hippocampal and frontal sources using beamformers in MEG. Brain Topography 2012: Feb 16 (Epub ahead of print). Mirzoev A, Bercovici E, Stewart LS, Cortez MA, Snead OC III, Desrocher M. Circadian profiles of focal epileptic seizures: A need for reappraisal. Seizure 2012: Epub Apr 24. Mohamed IS, Otsubo H, Ferrari P, Ochi A, Snead OC, Cheyne D. Neuromagnetic cerebellar activation during seizures arising from the motor cortex. Epilepsy Research 2011: 96: pp Moharir MD, Shroff M, Pontigon AM, Askalan R, Yau I, MacGregor DL, deveber G. A prospective outcome study of neonatal cerebral sinovenous thrombosis. Journal of Child Neurology 2011: 26 (9): pp Morin MP, Patenaude Y, Sinsky AB, Banwell B, Sebire G. Solitary tumefactive demyelinating lesions in children. Journal of Child Neurology 2011: 26 (8): pp Morris RD, Lovett MW, Wolf M, Sevcik RA, Steinbach KA, Frijters JC, Shapiro M. Multiple-component remediation for developmental reading disabilities: IQ, socioeconomic status, and race as factors in remedial outcome. Journal of Learning Disabilities 2012: 45 (2): pp Nagarajan L, Ghosh S, Palumo L, Akiyama T, Otsubo H. Fast activity during EEG seizures in neonates. Epilepsy Research 2011: 97: pp Nagata Y, Bayless SJ, Mills T, Taylor MJ. Spatio-temporal localisation of attentional orienting to gaze and peripheral cues. Brain Research 2012: 1439: pp Nossin-Manor R, Chung AD, Whyte HEA, Shroff MM, Taylor MJ, Sled JG. Deep gray matter maturation in very preterm neonates: Regional variations and pathology-related age-dependent changes in Magnetization Transfer Ratio. Radiology 2012: Mar 13 (Epub ahead of print). O Brien BA, Wolf M, Lovett MW. A taxometric investigation of developmental dyslexia subtypes. Dyslexia 2012: 18 (1): pp O Shea TM, Allred EN, Kuban KC, Dammann O, Paneth N, Fichorova R, Hirtz D, Leviton A, Extremely Low Gestational Age Newborn (ELGAN) Study Investigators. Elevated concentrations of inflammation-related proteins in postnatal blood predict severe developmental delay at 2 years of age in extremely preterm infants. Journal of Pediatrics 2012: 160 (3): pp e4. O Shea TM, Allred EN, Kuban KC, Hirtz D, Specter B, Durfee S, Paneth N, Leviton A, ELGAN Study Investigators. Intraventricular hemorrhage and developmental outcomes at 24 months of age in extremely preterm infants. Journal of Child Neurology 2012: 27 (1): pp the hospital for sick children department of paediatrics Annual Report

72 Ochi A, Hung R, Weiss S, Widjaja E, To T, Nawa Y, Shima T, Go C, Akiyama T, Donner E, Drake J, Rutka J, Snead OC III, Otsubo H. Lateralized interictal epileptiform discharges during rapid eye movement sleep correlate with epileptogenic hemisphere in children with intractable epilepsy secondary to tuberous sclerosis complex. Epilepsia 2011: 52 (11): pp Pang EW. Practical aspects of running development studies in the MEG. Brain Topography 2011: 24 (3-4): pp Parrish J, Yeh EA. Acute disseminated encephalomyelitis. Advanced Experimental Medical Biology 2012: 724: pp Probstel AK, Dornmair K, Bittner R, Sperl P, Jenne D, Magalhaes S, Villalobos A, Breithaupt C, Weissert R, Jacob U, Krumbholz M, Kuempfel T, Blaschek A, Stark W, Gartner J, Pohl D, Rostasy K, Weber F, Forne I, Khademi M, Olsson T, Brilot F, Tantsis E, Dale RC, Wekerle H, Hohlfeld R, Banwell B, Bar-Or A, Meinl E, Derfuss T. Antibodies to myelin oligodendrocyte glycoprotein are transient in childhood acute disseminated encephalomyelitis. Neurology 2011: 77 (6): pp Sananes R, Manlhiot C, Kelly E, Hornberger LK, Williams WG, MacGregor D, Buncic R, McCrindle BW. Neurodevelopmental outcomes after open heart operations before 3 months of age. Annals of Thoracic Surgery 2012: 93 (5): pp Sato D, Lionel AC, Leblond CS, Prasad A, Pinto D, Walker S, O Connor I, Russell C, Drmic IE, Hamdan FF, Michaud JL, Endris V, Roeth R, Delorme R, Huguet G, Leboyer M, Rastam M, Gillberg C, Lathrop M, Stavropoulos DJ, Anagnostou E, Weksberg R, Fombonne E, Zwaigenbaum L, Fernandez BA, Roberts W, Rappold GA, Marshall CR, Bourgeron T, Szatmari P, Scherer SW. SHANK1 deletions in males with Autism Spectrum Disorder. American Journal of Human Genetics 2012: 90 (5): pp Saver JL, Warach S, Janis S, Odenkirchen J, Becker K, Benavente O, Broderick J, Dromerick A, Duncan P, Elkind MSV, Johnston K, Kidwell CS, Meschia JF, Schwamm L, for the NINDS stroke Common Data Element Working Group. Standardizing the structure of stroke clinical and epidemiological research data: The NINDS Stroke Common Data Element (CDE) project. Stroke 2012: 3 (4): pp Scahill L, McCracken JT, Bearss K, Robinson F, Hollander E, King B, Bregman J, Sikich L, Dukes K, Sullivan L, Anagnostou E, Donnelly C, Kim YS, Ritz L, Hirtz D, Wagner A. Design and subject characteristics in the federally-funded citalopram trial in children with pervasive developmental disorders. Journal of Autism and Developmental Disorders 2012: 42 (3): pp Schechter T, Finkelstein Y, Ali M, Kahr W, Williams S, Chan AK, deveber G, Brandão LR. Unfractionated heparin dosing in young infants: Clinical outcomes in a cohort monitored with anti-factor Xa levels. Journal of Thrombosis and Haemostasis 2012: 10 (3): pp Schechter T, Kirton A, Laughlin S, Pontigon AM, Finkelstein Y, MacGregor D, Chan A, deveber G, Brandao L. Safety of anticoagulants in children with arterial ischemic stroke. Blood 2012: 119 (4): pp Seppälä E, Jokinen T, Fukata M, Fukata Y, Webster M, Karlsson E, Kilpinen S, Steffen F, Dietschi E, Leeb T, Eklund R, Zhao X, Rilstone J, Lindblad-Toh K, Minassian BA, Lohi H. LGI2 truncation causes a remitting focal epilepsy in dogs. Public Library of Science One (PLoS) Genetics 2011: 7 (7): e Stafstrom CE, Arnason BGW, Baram TZ, Catania A, Cortez MA, Glauser TA, Pranzatelli MR, Riikonen R, Michael A, Rogawski MA, Shlomo Shinnar S, Swann JW. Treatment of infantile spasms; Emerging insights from clinical and basic science perspectives. Journal of Child Neurology 2011: 26 (11): pp Stewart L, Cortez MA, Snead OC III. Environmental enrichment improves behavioral outcome in the AY-9944 model of childhood atypical absence epilepsy. International Journal of Neuroscience 2012: Epub Apr 30. Stockler S, Plecko B, Gospe SM Jr, Coulter-Mackie M, Connolly M, van Karnebeek C, Mercimek-Mahmutoglu S, Hartmann H, Scharer G, Struijs E, Tein I, Jakobs C, Clayton P, Van Hove JL. Pyridoxine dependent epilepsy and antiquitin deficiency: Clinical and molecular characteristics and recommendations for diagnosis, treatment and follow-up. Molecular Genetics and Metabolism 2011: 104 (1-2): pp Tan M, deveber G, Shroff M, Moharir M, Pontigon A, Widjaja E, Kirton A. Sagittal sinus compression is associated with neonatal cerebral sinovenous thrombosis. Pediatrics 2011: 128 (2): pp e429-e435. Taylor MJ, Donner EJ, Pang EW. fmri and MEG in the study of typical and atypical cognitive development. Neurophysiologie Clinique/Clinical Neurophysiology 2012: 42 (1-2): pp Taylor MJ, Mills T, Pang EW. The development of face recognition; Hippocampal and frontal lobe contributions determined with MEG. Brain Topography 2011: (3-4): pp Tellez-Zenteno, JF, Ronquillo LH, Jette N, Burneo JG, Nguyen DK, Donner EJ, Sadler M, Javidan MM, Gross DW, Wiebe S, Canadian Epilepsy Surgery Study Group. Discontinuation of antiepileptic drugs after successful epilepsy surgery. A Canadian survey. Epilepsy Research 2012: May 15. (Epub ahead of print). PMID: Thomas T, Branson HM, Shroff M, Verhey LH, Stephens D, Magalhaes S, Banwell B. The demographic, clinical, and magnetic resonance imaging (MRI) features of transverse myelitis in children. Journal of Child Neurology 2011: 27 (1): pp Thompson MD, Noble-Topham S, Percy ME, Andrade DM, Ebers GC. Chromosome 1p36 in migraine with aura: Association study of the 5HT(1D) locus. Neuroreport 2012: 23 (1): pp Thompson MD, Roscioli T, Marcelis C, Nezarati MM, Stolte- Dijkstra I, Sharom FJ, Lu P, Phillips JA, Sweeney E, Robinson PN, Krawitz P, Yntema HG, Andrade DM, Brunner HG, Cole DE. Phenotypic heterogeneity in hyperphosphatasia with seizures and neurologic deficit (Mabry syndrome). American Journal of Medical Genetics (Part A) 2012: 158A (3): pp Till C, Deotto A, Tipu V, Sled JG, Bethune A, Narayanan S, Arnold D, Banwell B. White matter integrity and math performance in pediatric multiple sclerosis: A diffusion tensor imaging study. Neuroreport 2011: 22 (18): pp Torres CV, Fallah A, Ibrahim GM, Chesier S, Otsubo H, Ochi A, Chuang S, Snead OC, Holowka S, Rutka JT. The role of magnetoencephalography in children undergoing hemispherectomy. Journal of Neurosurgery and Pediatrics 2011: 8: pp Tropak MB, Shi H, Li J, Dai X, Redington AN, Askalan R. Potent neuroprotection induced by remote preconditioning in a rat model of neonatal cerebral hypoxic-ischemic injury. Journal of Thoracic Cardiovascular Surgery 2011: 142 (1): pp van Eimeren VF, Billinghurst L, Askalan R, Laughlin S, Brandão LR, Williams S, Kahr WHA. Cerebral sinus venous thrombosis in a child with hyperthyroidism. Pediatric Blood Cancer 2012: 58 (1): pp Verhey LH, Branson HM, Shroff MM, Callen DJA, Sled JG, Narayanan S, Sadovnick D, Bar-Or A, Arnold Dl, Marie RA, Banwell B. MRI parameters for prediction of multiple sclerosis in children with acute CNS demyelination: A prospective national cohort study. Lancet Neurology 2011: 10 (12): pp Vidal J, Mills T, Pang EW, Taylor MJ. Response inhibition in adults and teenagers: Spatiotemporal differences in the prefrontal cortex. Brain and Cognition 2012: 79: pp Vincent AK, Noor A, Janson A, Minassian BA, Ayub M, Vincent JB, Morel CF. Identification of genomic deletions spanning the PCDH19 gene in two unrelated girls with intellectual disability and seizures. Clinical Genetics 2011: Epub Nov 17: PMID Visanji N, Wong, JC, Wang SX, Cappel B, Kleinschmidt- DeMasters BK, Handler MH, Ochi A, Otsubo H, Rutka JT, Go C, Weiss S, Vinters HV, Hawkins CE, DeSouza LV, Siu M, Hazrati LN. A proteomic analysis of pediatric seizure cases associated with astrocytic inclusions. Epilepsia 2012: 53 (3): pp e Waldman AT, Banwell B. Editorial: Acquiring new insights: Incidence of acquired demyelination and MS in U.S. children. Neurology 2011: 77 (12): pp the hospital for sick children department of paediatrics Annual Report

73 Weinstock-Guttman B, Ramanathan M, Marr K, Hojnacki D, Benedict RHB, Morgan C, Yeh EA, Carl E, Kennedy C, Reuther J, Brooks C, Hunt K, Elfadil M, Andrews M, Zivadinov R. Clinical correlates of chronic cerebrospinal venous insufficiency in multiple sclerosis. British Medical Journal Neurology 2012: 12: pp 26. Epub ahead of print. Widjaja E, Mahmoodabadi SZ, Snead OC, Almehdar A, Smith ML. Widespread cortical thinning in children with frontal lobe epilepsy. Epilepsia 2011: 52: pp Widjaja E, Mahmoodabadi Z, Go C, Raybaud C, Chuang S, Snead OC, Smith ML. Reduced cortical thickness in children with new-onset seizures. American Journal of Neuroradiology 2012: (Epub ahead of print). Widjaja E, Snead OC III, Otsubo H, Crings SG, Zarei S. Diffusion tensor imaging assessment of the epileptogenic zone in children with localization-related epilepsy. American Journal of Neuroradiology 2011: 32: pp Widjaja E, Snead OC, Zarei S, Chuang S, Raybaud C, Smith ML. Reduced cortical thickness in children with new-onset seizures. American Journal of Neuroradiology 2012: 33 (4): pp Willer T, Lee H, Lommel M, Yoshida-Moriguchi T, Beltran Valero de Bernabe D, Venzke D, Cirak S, Schachter H, Vajsar J, Voit T, Muntoni F, Loder AS, Dobyns WB, Winder TL. Strahl S, Matthews KD, Nelson SF, Moore SA, Campbell KP. ISPD loss-of-function mutations disrupt dystroglycan O-mannosylation and cause Walker-Warburg syndrome. Nature Genetics 2012: 44 (5): pp Williams TS, Westmacott R, Dlamini N, Granite L, Dirks P, Askalan R, MacGregor D, Moharir M, deveber G. Intellectual ability and executive function in pediatric moyamoya vasculopathy. Developmental Medicine and Child Neurology 2012: 54 (1): pp Wu Y, Blichowski M, Zafiris J, Daskalakis ZJ, Wu Z, Liu CC, Cortez MA, Snead OC III. Evidence that clozapine directly interacts on the GABAB receptor. Neuroreport 2011: 22 (13): pp Yeh EA, Weinstock-Guttman B. Fingolimod: An oral agent for the treatment of multiple sclerosis. Advances in Therapy 2011: 28 (4): pp Yeh EA. Management of children with multiple sclerosis. Pediatric Drugs 2012: 14 (3): pp Yeh EA. Therapeutic options in pediatric demyelinating disorders. Current Treatment Options in Neurology 2011: 13 (6): pp Yeh EA. Which MRI findings predict MS development in children? National Review Neurology 2012: 28 8 (4): pp You X, Bernal B, Guillen M, Ayala M, Barreto A, Rishe N, Sullivan J, Dlugos D, Berl M, VanMeter J, Morris D, Donner E, Bjornson B, Smith M-L, Gaillard WD, Adjouadi M. A decisional space for fmri pattern separation using the principal component analysis. A study of the language network in pediatric localization related epilepsy. Human Brain Mapping 2012: Mar 28: doi /hbm (Epub ahead of print). Zivadinov R, Cutter G, Marr K, Ramanathan M, Benedict RHB, Elfadil M, Bergsland N, Moran C, Carl E, Hojnacki D, Yeh EA, Willis L, Cherneva M, Hussein S, Durfee J, Kennedy C, Dwyer M, Weinstock-Guttnan B. Prevalence, sensitivity and specificity of chronic cerebrospinal venous insufficiency in multiple sclerosis. Neurology 2011: 12: 77 (2): pp Zolkipli Z, Mai L, Lamhonwah AM, Tein I. The mdx mouse as a model for carnitine deficiency in the pathogenesis of Duchenne muscular dystrophy. Muscle and Nerve 2012: On line Mar 2, DOI: /mus Zolkipli Z, Sherlock M, Biggar WD, Taylor G, Hutchison JS, Peliowski A, Alman BA, Ling SC, Tein I. Abnormal fatty acid metabolism in spinal muscular atrophy may predispose to perioperative risks. European Journal of Paediatric Neurology 2012: Jan 19 (Epub ahead of print). Books and Chapters Anagnostou E, Bear M, Dawson G. Commentary: Future directions in the treatment of autism spectrum disorders. In: Autism Spectrum Disorders (Amaral D, Dawson G, Geschwind D, eds.). Oxford University Press Anagnostou E, Rusoff J. Anticonvulsants in the treatment of autism. In: Textbook of Autism Spectrum Disorders (Hollander E, Kolevzon A, Coyle J, eds.). APPI Press Banwell B, Ghezzi A. Clinical presentation of pediatric multiple sclerosis. In: Demyelinating Disorders of the Central Nervous System in Childhood (Chabas D, Waubant E, eds.). Cambridge University Press, Cambridge 2011: pp deveber G. The epidemiology of childhood stroke. In: Stroke and Cerebrovascular Disease in Childhood, International Review of Child Neurology Series, published on behalf of the International Child Neurology Association, First Edition (Ganesan V, Kirkham F, eds.). Mac Keith Press, London UK 2011: pp deveber G. Cerebral sinovenous thrombosis. In: Stroke and Cerebrovascular Disease in Childhood, International Review of Child Neurology Series, published on behalf of the International Child Neurology Association, First Edition (Ganesan V, Kirkham F, eds.). Mac Keith Press, London UK 2011: pp Gordon A, Carr L, Ganesean V, Kirkham F, deveber G. Outcome after stroke. In: Stroke and Cerebrovascular Disease in Childhood, International Review of Child Neurology Series, published on behalf of the International Child Neurology Association, First Edition (Ganesan V, Kirkham F, eds.). Mac Keith Press, London UK 2011: pp Hahn CD, Shemie S, Donner E: Status epilepticus. In: Pediatric Critical Care, Fourth Edition (Fuhrman B, Zimmerman J, eds.). Elsevier, Philadelphia 2011: pp Han HA, Cortez MA, Snead OC. GABAB receptor and absence epilepsy. In: Jasper s Basic Mechanisms of the Epilepsies, Fourth Edition (Noebels JL, Avoli M, Rogawski MA, Olsen RW, Delgado-Escueta AV, eds.). Oxford University Press, New York NY 2012: pp Ichord R, Yardumian A, Kirkham FJ, deveber G. Acute management in stroke and cerebrovascular disease in childhood. In: Stroke and Cerebrovascular Disease in Childhood, International Review of Child Neurology Series, published on behalf of the International Child Neurology Association, First Edition (Ganesan V, Kirkham F, eds.). Mac Keith Press, London UK 2011: pp Kirton A, deveber G. Cerebrovascular disease. In: Pediatric Neurology Principles and Practice, Fifth Edition (Swaiman K, Ashwal S, Ferriero D, eds.). Mosby Elsevier, Philadelphia PA 2012: pp Kirton A, deveber G. Pediatric stroke syndromes. In: Nelson Textbook of Pediatrics, Nineteenth Edition (Kleigan R, Stanton B, St. Geme J, Schor N, Behrman R, eds.). Elsevier, Philadelphia PA 2011: pp MacGregor DL. Trauma to the posterior fossa. In: Cerebellar Disorders of Childhood - Clinics in Developmental Medicine No (Boltshauser E, Schmahmann J, eds.). 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74 Makhani N, Banwell B. Acquired disorders affecting white matter. In: Pediatric Neurology, Principles and Practice, Fifth Edition (Swaiman KF, Ashwal S, Ferriero DM, Schor NF, eds.). Mosby 2012: pp Moharir M, deveber G. Cerebral sinovenous thrombosis in infants and children. In: Medlink Neurology 2011 (Gilman S, ed.). San Diego: Medlink Corporation. Available at Ochi A, Go CY, Otsubo H. Clinical MEG analyses for children with intractabal epilepsy. In: Magnetoencephalography (Pang EW, ed.). Intech Janeza Trdine, Rijeka, Croatia 2011: pp Parrish JB, Yeh, EA. Acute disseminated encephalomyelitis. In: Neurodegenerative Diseases (Ahmad SI, ed.). Landes Bioscience/Springer, Texas: 2011: pp Perez Velazquez JL. Possible actions of gap junctional coupling in reward-seeking behaviours. In: Gap Junctions in the Nervous System: Physiological and Pathological Roles (Dere E, ed.), Elsevier Academic Press, San Diego, Picton W, Taylor MJ, Durieux-Smit A. Brainstem auditory evoked potentials in pediatrics. In: Clinical Neurology, Sixth Edition (Aminoff MJ, ed.). Churchill Livingstone, New York, 2012: pp Smith ML, Lah S, Elliott IM. Outcomes of pediatric epilepsy surgery. In: Neuropsychology in the Care of People with Epilepsy (Helmstaedter C, Hermann B, Lassonde M, Kahane P, Arzimanoglou A, eds.). John Libbey Eurotext, 2011: pp Tein I. Metabolic myopathies. In: Neuromuscular Disorders (Tawil RN, Venance S, eds.). Wiley Blackwell, West Sussex 2011: pp Tein I. Metabolic myopathies. In: Pediatric Neurology, Fifth Edition. (Swaiman KF, Ashwal S, Ferriero D, Schor NF, eds.). Saunders 2012: pp Teitelbaum A, Belkas J, Brian J, Perez Velazquez JL. Distinct patterns of cortical coordinated activity in autism. In: Autism Spectrum Disorders (Richardson CE, Wood RA, eds.). New Research, Nova Science Publishers, Yeh EA, Weinstock-Guttman B. Pediatric multiple sclerosis. In: Multiple Sclerosis Therapeutics, Fourth edition. (Cohen JA, Rudick RA, eds.) Cambridge University Press, 2011: pp Grants and Funding A novel approach to protect the developing brain against ischemic injury. Askalan R. Heart and Stroke Foundation of Canada Grant-in-Aid. $160,000 CAD. Jul to Jun A pilot dose finding study of pioglitazone in children with ASD. Anagnostou E, Brian J, Dupuis A. Dean s Fund. $10,000 CAD to 2011 A pilot dose finding study of pioglitazone in children with ASD. Anagnostou E, Brian J. PSI. $127,700 CAD to 2013 An international multi-centre joint initiative to plan and design a randomized controlled trial (RCT) of anti-coagulation therapy in neonatal cerebral sinovenous thrombosis (NCSVT). Moharir M, deveber G, de Vries L, Branson H, Chan A, de Haan T, Jordan L, Kirkham F, McNamara P, Pontigon AM, Steinlin M, Shroff M, van Ommen H, Volmer B. International Pediatric Research Foundation. $25,000 USD. Apr 2012 to Jul 2013 Autism spectrum and associated neurodevelopmental disorders: Genomes to outcomes. Scherer S, Anagnostou E, Vincent J, Roberts W, Brian J, Szatmari P, Fernandez B, Weksberg R, Paterson A, Fombonne E, Zwaigenbaum L, Arnold P, Schachar R, Bassett A, Drmic I, Cytrynbaum. Ontario Research Fund. $234,621 CAD to 2013 Autism Treatment Network Registry. Loh A, Roberts W, Anagnostou E, Marcon M, Weiss SK, Brian J, Feigenbaum A, Schulze A, Logan W, Chitiyat D, Weksberg R, Green P, Mitchell S, Berall G, Bradley E, Freeman N, Stinson J, Ornstein M, Atkinson A, Goldfarb C, Flanagan J, Superina D, Jiminez E, Jones Stokreef N, Orsino A, Isaacs B, Handley Derry M. Autism Speaks Canada. $420,000 CAD. Aug 2011 to Jul 2013 Better nights/better days: Improving psychosocial health outcomes in children with behavioural insomnia. Corkum PV, Andreou P, Gruber R, Rusak B, Weiss SK, Barwick M, Hall W, Stremler R, Witmans M, Chambers C, McGrath P, Wade T, Godbout R, Reid G, Watters C. Canadian Institutes of Health Research. $2,000,000 CAD. Oct 2010 to Sep 2015 Biological markers of pediatric cerebrovascular inflammation and stroke. Kirton A, deveber G. Alberta Children s Hospital Research Institute. $36,100 CAD. Jan 2012 to Jan 2013 Biomarkers in sudden unexpected death in epilepsy. Donner EJ. Ontario Brain Institute. $80,634 CAD to 2013 Biometric authentication using handwriting biomechanics. Chau T, Anagnostou E, Fleury A. Natural Sciences and Engineering Research Council of Canada (NSERC). $340,000 CAD to 2013 Brain and behavioural plasticity after self-regulation intervention in FASD. Rovet J, Koren G, Lerch J, Taylor MJ. Canadian Institutes of Health Research BSB. $423,249 CAD. Sep 2010 to Aug 2013 Candidate gene analysis of genes located in within regions of chromosomes linked to Mabry Syndrome (Hyperphosphatasia with neurologic deficit and seizures OMIM#239300). Andrade D, Thompson M. Scottish Rite Charitable Foundation of Canada. $105,000 CAD. Oct 2010 to Oct 2013 Care and research to optimize HIV-positive women s health during preconception, pregnancy and motherhood. Interdisciplinary HIV Pregnancy Research Group: Loutfy MR, Serghides L, Yudin M, Hangel JB, Bayoumi AM, Bitnun SA, Brophy JC, Calzavara L, Carvalhal A, Greene S, Gruslin AM, Hamilton GS, La Porte C, Lye SJ, Murphy KE, Newmeyer TS, Raboud JM, Rachlis AR, Ralph ED, Rosenthal KL, Shapiro HM, Shynlova O, Silverman, M, Smaill FM, Smith ML, Walmsley SL, Wobeser WL. Canadian Institutes of Health Research - CIHR Emerging Team Grant. $900,000 CAD to 2014 Centre for Investigation of Neuroplasticity in Neurodevelopment. Snead OC. CFI/ORF $9,535,612 CAD. Jan 2007 to Dec 2012 Centre for the Study of Complex Childhood Diseases. Post M, Tein I (Member CFI Team). Canada Foundation for Innovation. $21,000,000 CAD. Jul 2007 to Jun 2012 Cerebral venous hemodynamics in pediatric multiple sclerosis. Banwell B, McGowan C, Laughlin S, Shroff M, Arnold D, Narayanan S, Bar-Or A, Sled J, Moharir M, Yeung R, Marie RA. Multiple Sclerosis Society. $196,579 CAD. Jul 2010 to Jun 2012 Characterization of genetic and molecular mechanisms underlying infantile spasms. Osborne L, Minassian BA. Canadian Institutes of Health Research. $549,785 CAD. Apr 2008 to Mar 2013 Characterization of the carnitine transporter gene OCTN3 in patients with peroxisomal dysfunction using Next-Generation Sequencing. Tein I, Lamhonwah A. Rare Diseases Foundation Grant. $3500 CAD. Jan 2012 to Dec 2012 Charting the territory: Determining and documenting trajectories for families where a child has a life-threatening condition. Siden H, Steele R, Cadell S, Davies B, Straatman L, Brant R, Rapoport A, Weiss SK. Canadian Institutes of Health Research. $1,343,000 (SickKids portion: $20,594/year) CAD. Oct 2010 to Sep 2015 Childhood Obesity Research - CIHR Team. McCrindle B, Tein I, Anderson H, Hamilton J, Parkin P, Pencharz P. Canadian Institutes of Health Research. $2,364,738 CAD. Jul 2008 to Jun 2013 Clinical-demographic, epidemiology, pathobiology, neuroimaging features and outcomes of acute demyelination in Canadian children: Phase 2. Yeh A, Banwell B, Bar-Or A, Sadovnick D, Marrie RA, Arnold D, Till C, Costello F, Narayanan S, Sled J, Tellier R. MS Society of Canada. $4,563,585 CAD. Jul 2009 to Jun 2013 Collaborative Academic Rehabilitation Engineering (CARE). Chau T, Baecker R, Santerre JP, Naguib HE, Colantino A, Fernie GR, Wong W, Steele C, Maki BE, Popovic MR, Mihailidis A. Natural Sciences and Engineering Research Council, Collaborative Research and Training Experience (CREATE) Program. $1,650,000 CAD. Jul 2009 to Jun 2015 CP Integrated Neuroscience Discovery Network. deveber G, Shroff M, Scherer S. Ontario Brain Institute. $344,881 CAD. Oct 2011 to Sep the hospital for sick children department of paediatrics Annual Report

75 Creating a novel mentorship program for junior faculty at a pediatric academic health sciences center - The role of a modified developmental evaluation (MDE) model. Weiss SK, Parker K, Marimianakis T, Schneider R, Leslie K. SickKids Pediatric Consultants Education Research Grant. $4,800 CAD. Nov to Oct Demographic, environmental and genetic factors that modify pediatric multiple sclerosis course. Waubant E, Yeh EA. NIH R01. $2,000,000 USD to 2016 Developing instructional approaches suited to the cognitive and motivational needs of struggling adult readers: Center for the Study of Adult Literacy. Greenberg D, Lovett MW, Graesser A, Frijters JC, Branum-Martin L. The first National Research and Development Center on Cognition and Adult Literacy, Institute of Education Sciences (US Department of Education) $10,000,000 USD to 2017 Development of an MEG-compatible articulography system for the assessment of brain function and oromotor dynamics. Donner EJ, Cheyne D, Chau T, Pang EW, Steele CM, van Lieshout P. Natural Sciences and Engineering Research Council of Canada and Canadian Institute of Health Research. $390,000 CAD to 2013 Development of an MEG-compatible articulography system for the assessment of brain function and oromotor dynamices. Taylor MJ, Doesburg S, Anagnostou E, Pang EW, Donner E. Collaborative Health Research Projects, Canadian Institutes of Health Research. $875,000 CAD to 2013 Development of enhanced pediatric neuroscience clinical care through international partnerships: Providing and evaluating best practices for both on-site (short-term) and distance (long-term) continuing medical education. Weiss SK, Parakh M, Shroff M, Moharir M, BenDavid G, Martimianakis T. SickKids International. $20,000 CAD. Jan 2012 to Dec 2012 Development of frontal lobe cognitive functions: Timing, structure and connectivity. Pang EW, Cheyne D, De Nil L, Roberts SW. Canadian Institutes of Health Research BSC. $872,000 CAD to 2017 Developmental investigation of memory capacity using functional neuroimaging. Taylor MJ. NSERC Discovery Grant, $150,000 CAD to 2014 Dissociating cognitive and motor components of healthy and dysfunctional expressive language development in children. Cheyne D, Chau T, Van Lieshout P, Steele C, Pang EW. Canadian Institutes of Health Research. $411,184 CAD to 2013 Dysregulation of fatty acid oxidation by defective SMN1 gene: Implications for therapy. Tein I. Rare Diseases Foundation Grant. $3,500 CAD. May 2012 to Apr 2013 Effects of creatine supplementation on muscle pathophysiology in children with dermatomyositis. Stephens S, Feldman B, Tein I, Wells G, Schulze A. Rare Diseases Foundation Grant. $3,500 CAD. May 2012 to Apr 2013 Efficacy of L-arginine therapy on vasodilatation and mitochondria in MELAS syndrome. Tein I. United Mitochondrial Diseases Foundation. $75,000 CAD. Jul 2010 to Mar 2013 Efficacy of the ketogenic diet. Donner EJ. Ontario Brain Institute. $47,268 CAD to 2013 End MS RRTC, Ontario, Manitoba. Yeh A, Harauz G, Gaffhir O. MS Society of Canada. $300,000 (Stage 1) CAD. Apr 2012 to Apr 2015 Ensuring effective newborn screening: The case of cystic fibrosis. Miller F, Guttuman A, Bombard Y, Carroll J, Chakrabaty P, Durie P, Kerr E, Ratjen F, Taylor M, Tam K. Canadian Institutes of Health Research. $636,506 CAD. Sep 2010 to Aug 2014 Environmental enrichment, epigenetics, and infantile spasms: New approaches to intractable epilepsy - The Epilepsy Discovery Project. Snead OC III, Cortez MA, Stewart LS, Eubanks J, McGowan P. Ontario Brain Institute. $100,500 CAD. Oct 2011 to Mar 2013 Epilepsy genetics: Seizing the advances. A Plenary Session of the 2012 Canadian League Against Epilepsy Meeting. Minassian BA. Canadian Institutes of Health Research. $17,175 CAD. Nov 2011 to Oct 2012 Ergometric training in mitochondrial myopathies. Tein I. Knights of Columbus Fund for Mitochondrial Disorders. $1000 CAD. Jan 2012 to Dec 2012 Exploring stochasticity in the spatio-temporal patterns of coordinated brain activity. Perez-Velazquez JP. NSERC Discovery Grant. $155,000 CAD Feasibility of international biological sample research in pediatric MS. Banwell B, Bar-Or A, Tardieu M, Chitnis T, on behalf of the International Pediatric Multiple Sclerosis Study Group. Multiple Sclerosis International Federation. $52,856 CAD. Jan 2011 to Jan 2013 Genes, reading and dyslexia. Gruen J. Subcontracted sites: Lovett MW, Wolf M, Pennington B, Willcutt E, Boada R. The Manton Foundation to Yale University, $5,000,000 USD to 2014 Genetic causes of temporal lobe epilepsy. Andrade D, Bassett A, Krings A, Krings T, Minassian B, Chow E. Physicians Service Incorporated. $168,944 CAD. Apr 2011 to Mar 2013 Genetics and reading disabilities. Barr CL, Lovett MW, Kerr EN. Canadian Institutes of Health Research. $1,053,327 CAD. Jan 2008 to Jul 2013 Genetics of temporal lobe epilepsy. Andrade D, Minassian B, Bassett A, Krings T, Chow E. Ontario Brain Institute. $70,000 CAD. Dec 2011 to Jul 2013 Genomic influences on brain and behavioural trajectories in autism spectrum disorders. Zwaigenbaum L, Anagnostou E, Scherer S, Evans A, Roberts W, Bryson S, Fombonne E, Mirenda P, Hall G, Szatmari P. NeuroDevNet. $2,643,200 CAD to 2013 Global migration and human autoimmune disease. Banwell B, Croitoru, K, Danska J, Watts T, Gommermann J. Connaught Foundation (Ontario), University of Toronto. $10,000 CAD. Jan 2011 to Apr 2012 Health-related quality of life in children with new-onset epilepsy: A long-term follow-up. Smith ML, Speechley K, Camfield C, Ferro M, Levin S, Wiebe S, Zho G. Canadian Institutes of Health Research. $561,784 CAD to 2015 How does the brain s clockwork help it to reorganize old memories while we sleep? Richards B, Frankland P, Cortez MA. Spring CIHR Team Research and Training Program in Sleep and Biological Rhythms Competition. $4,000 CAD. Aug 2011 to Jul 2012 Identifying juvenile myoclonic epilepsy genes through whole-genome sequencing in large families. Andrade D, Delgado-Escueta A, Minassian B. University of Toronto McLaughlin Centre. $200,000 CAD. Jul 2011 to Jun 2013 Identifying the disease genes for the prototypical and most common generalized and focal epilepsies of children through whole exome sequencing. Minassian BA, Andrade D. University of Toronto McLaughlin Centre. $50,000 CAD. May 2011 to Apr 2012 Impact of seizures in infants and children with ischemic stroke. deveber G. The Pediatric Epilepsy Research Foundation. $198,000 USD. Jul 2011 to Jun 2013 Infantile spasms in Down syndrome: Correction of the epileptic phenotype. Snead OC, Cortez MA, Eubanks J, Salter M. Canadian Institutes of Health Research. $755,000 CAD. Jan to Mar Intranasal oxytocin for social cognition/function in adults with autism. Anagnostou E, Szatmari P. Canadian Institutes of Health Research. $1,080,000 CAD to 2016 Intranasal oxytocin for the treatment of children and adolescents with autism spectrum disorders. Anagnostou E, Roberts W, Brian J, Dupuis A, Jacobs S, Cook E. Department of Defense. $805,791 CAD to 2013 Long-term outcomes of pediatric epilepsy surgery: Sub project in Integrated Discovery System for Epilepsy (New Approaches to Intractable Seizures). Smith ML. Ontario Brain Institute. $69,000 CAD to 2013 Longitudinal study of brain mechanisms underlying visual working memory in children with autism. Taylor MJ, Anagnostou E, Lerch J. Canadian Institutes of Health Research. $660,320 CAD to the hospital for sick children department of paediatrics Annual Report

76 Mapping and modulation of brain plasticity in perinatal stroke. Kirton A, deveber G, Chen R, Demchuk A, Hill MD, Barlow K, Howard J. Heart and Stroke Foundation of Canada. $300,000 CAD. Sep 2009 to Aug 2014 MEB Diseases. Vajsar J. SickKids Foundation. $37,000 CAD. Dec 2006 to Jul 2012 Mechanisms of neurotoxicity in mitochondrial SCAD deficiency. Tein I. Neurometabolic Research Fund. $2,200 CAD. Jan 2011 to Dec 2011 Multimodal neuroimaging and neurobehavioural outcomes in preterm neonates. Taylor M, Donner EJ Whyte H, Shroff M, Moore M. Canadian Institutes of Health Research. $925,000 CAD to 2012 Neurological Registry Best Practice Guidelines and Implementation Toolkit. Donner EJ, Korngut B for the Study Group. Public Health Agency of Canada. $209,496 CAD to 2013 New approaches to intractable epilepsy The Epilepsy Discovery Project. Burnham M, Burneo J, Andrade D, Carlen P, Hwang P, McAndrews MP, Persad N, Poulter M, Ronen G, Shapiro M, Snead C, Steven D, Valiante T, Whiting S. Ontario Brain Institute. $2,896,481 CAD. Dec 2011 to Dec 2012 New approaches to intractable epilepsy: The Epilepsy Discovery Project. Minassian BA. The Ontario Brain Institute. $75,073 CAD. Jan 2012 to Mar 2013 Nodular heterotopias as a biomarker for pharmacoresistant schizophrenia. Andrade D, Bassett A, Krings T. NARSAD (USA). Young Investigator. $59,648 USD. Mar 2011 to Mar 2013 Nonconvulsive seizures among critically ill children. Hahn CD, Hutchison J, Snead C, Parshuram C, Otsubo H. SickKids Foundation/CIHR - New Investigator Research Grant. $431,694 CAD. Oct 2009 to Dec 2013 Nonconvulsive seizures among critically ill children. Hahn CD, Hutchison J, Snead C, Parshuram C, Otsubo H, Dennis M. PSI Foundation. $167,000 CAD. Apr 2010 to Dec 2013 Oral vitamin D for pediatric multiple sclerosis. Banwell B, Makhani N, Hanwell H. Dairy Farmers of Ontario. $80,000 CAD. Sep 2011 to Sep 2013 Paediatric myasthenia. Vajsar J, Kolski H. Canadian Paediatric Surveillance Program. $42,000 CAD. Jan 2010 to Dec 2011 Prevalence of creatine deficiency syndromes and genetic variability in creatine metabolism in children with autism spectrum disorder: A pilot study. Schulze A, Loh A, Anagnostou E, Scherer S, Roberts W, Bauman M, Tsai A. Autism Intervention Research - Physical Disorders. $212,446 CAD to 2012 Province of Ontario Neurodevelopmental Disorders Network. Anagnostou E, Scherer S, Szatmari P, Arnold P, Schachar R. Ontario Brain Institute. $3,500,000 CAD to 2013 Quantitative neuroimaging markers of executive dysfunction in children with frontal lobe epilepsy. Widjaja E, Snead OC. AUR GE Radiology Research Academic Fellowship (GERRAF) Award. $140,000 CAD to 2013 Quantitative non-invasive MRI measures of cerebral blood flow and cerebrovascular reactivity in children with sickle cell disease. Kassner A, deveber G. Canadian Institutes of Health Research. $360,000 CAD. Oct 2010 to Sep 2013 RCT of melatonin to improve sleep in children who have both epilepsy and neurodevelopmental disorders. Weiss S. Complementary and Alternative health care in Pediatrics. Hospital for Sick Children. $121,000 CAD. May 2010 to May 2012 Registry for SUDEP in Children. Donner EJ, Carmant L, Kinney H. Citizens United for Research in Epilepsy. $99,900 CAD to 2012 Regulation of expression of organic cation/carnitine (OCTN/ Cn) transporter family in developing murine heart. Tein I. Mead-Johnson Grant. $17,500 CAD. Apr 2011 to Mar 2012 Sleep education program for parents of children with autism. Malow B, Weiss SK, Loh A. Autism Intervention Research Program. $116,803 CAD. Jul 2011 Structural and functional neuroimaging correlates of cognitive impairment in childhood-onset multiple sclerosis. Till C, Banwell B, Narayanan S, Akbar N. Scottish Rite Charitable Foundation of Canada $105,000 CAD. Oct 2011 to Oct 2014 The Children s Stroke Project. deveber G. Anonymous Children s Foundation. $1,800,000 CAD. Jul 2009 to Jul 2014 The clinical-demographic, epidemiology, pathobiology, neuroimaging features and outcome of acute demyelination in Canadian children. Banwell B, Amit Bar-Or A, Sadovnick D, Marrie RA, Arnold D, on behalf of the Canadian Pediatric Demyelinating Disease Network. Multiple Sclerosis Scientific Research Foundation. $4,653,550 CAD. Jul 2009 to Jun 2013 The study of toddlers with autism and regression (STAR) protocol: Biomarkers of inflammation and immune activation in ASD. Loh A, James J, Menon D, Anagnostou E, Pardo C, Benseler S, Schulze A, Feigenbaum A, Logan W, Brian J, Burns K, Asghar S, Banwell B, Spence S, Bauman M, Zimmerman A, Beversdorf D, Sohl K. Health Resources and Services Administration, Autism Intervention Research Network for Physical Disorders. $213,854 CAD. Jan 2011 to Aug 2012 Thrombolysis in pediatric stroke. Amlie-Lefond C, deveber G. National Institutes of Health. $201,234 CAD. Sep 2011 to Aug 2016 Towards the correction of the Rett-like phenotype of MeCP2-deficient mice. Snead OC. Canadian Institutes of Health Research. $150,000 CAD. Oct 2010 to Sep 2014 Transcranial magnetic stimulation to evaluate the role of clozapine potentiation of GABA in the treatment of schizophrenia. Snead OC. Ontario Mental Health Foundation. $75,000 CAD to 2011 Treatment of overweight induced by antipsychotic medication in young people with ASD. Anagnostou E, Veenstra-VanderWeele J, Handen B, Aman MG. HRSA. $1,800,000 CAD to 2015 Typical and atypical development of frontal lobe systems and the maturation of social cognitive function. Taylor MJ, Pang EW, Doesburg S, Anagnostou E. Canadian Institutes of Health Research BSC. $766,896 CAD. Jan 2012 to 2016 Typical and atypical development of frontal lobe systems and the maturation of social cognitive function. Taylor M, Anagnostou E. Canadian Institutes of Health Research. $450,000 CAD to 2016 Unravelling the pathogenesis of the autophagic vacuolar myopathies. Minassian BA, Ackerley C, Manolson MF. Canadian Institutes of Health Research. $779,395 CAD. Apr to Mar Validating the role of actigraphy in assessing sleep patterns and sleep disorders in children with epilepsy. Weiss SK, Sadaka Y. SickKids Funding from Chase an Idea in Pediatric Neuroscience. $29,000 CAD. Aug 2011 to Jul 2012 Validation of monitoring changes in brain synchrony to anticipate seizures and implement feedback stimulation to stop seizure occurrence. Perez-Velazquez JP. Ontario Brain Institute. $155,700 CAD to 2013 Vascular effects of infection in pediatric stroke. deveber G, Fullerton H. National Institutes of Health. $6,815,231 (+$47,070 USD for salary support). Jul 2009 to Apr 2014 Vitamin D clinical trial. Yeh EA. Dairy Farmers of Canada. $80,000 CAD. Jan 2012 to Apr 2013 Working memory function in children with autism. Taylor M, Anagnostou E. Canadian Institutes of Health Research. $353,921 CAD to 2013 Working memory in children with autism. Taylor MJ, Smith ML, Lerch JP, Anagnostou E. Canadian Institutes of Health Research. $660,318 CAD to the hospital for sick children department of paediatrics Annual Report

77 PAEDIATRIC EMERGENCY MEDICINE overview Division Head Dr. Stephen C. Porter Full-Time Dr. Suzanne Beno Dr. Kathy Boutis Dr. Kevin Chan Dr. Seen Chung Dr. Yaron Finkelstein Dr. Jason Fischer Dr. Stephen Freedman Dr. Anna Jarvis (retired) Dr. Kelly Keogh Dr. Luba Komar Dr. Amina Lalani Dr. Sanjay Mehta Dr. Bruce Minnes Dr. Trent Mizzi Dr. William Mounstephen Dr. Olivia Ostrow Dr. Jonathan Pirie Dr. Tania Principi Dr. Savithiri Ratnapalan Dr. Suzan Schneeweiss Dr. Suzanne Schuh Dr. Dennis Scolnik Dr. Jennifer Thull-Freedman Half-Time Dr. Claudio Fregonas Dr. Paul Kadar Dr. Talya Wise Part-Time Dr. Daniela Ardelean Dr. Sherri Baine Dr. Anna Banerji Dr. Alexander Barron Dr. Michelle Batthish Dr. Glenda Bendiak Dr. Elizabeth Berger Dr. Nirit Bernhard Dr. Maala Bhatt Dr. Nicholas Blanchette Dr. Joey Bonifacio Dr. Michelle Bridge Dr. Malini Dave Dr. Avram Denburg Dr. Anh Do Dr. Janine Flanagan Dr. John Freedman Dr. Beth Gamulka Dr. Sabine George Dr. Peter Glazer Dr. Donna Goldenberg Dr. Silvio Gonzalez Dr. Sumit Gupta Dr. Javier Gutierrez Dr. Akhter Hamid Dr. Mark Handley-Derry Dr. Leah Harrington Dr. Shauna Jain Dr. Edward Jin Dr. Eddy Lau Dr. Yen Le Dr. Patricia Li Dr. Lily Lim Dr. Nadia Luca Dr. Arif Manji Dr. Joanna Massam Dr. Steven Moss Dr. Pat Neelands Dr. Carole O Beirne Dr. Melissa Parker Dr. Lillian Rojnica Dr. Mahmoud Sakran Dr. Tania Samanta Dr. Audrey Segal Dr. Domenick Shelton Dr. Gordon Soon Dr. Heshy Sturm Dr. Mark Tessaro Dr. Alene Toulany Dr. Pamela Valentino Dr. Anita Villani Dr. Jeff Weisbrot Dr. Kevin Weingarten Dr. Sheldon Wise Cross Appointees Dr. Susanna Benseler Dr. Hartmut Grasemann Dr. Hilary Whyte Dr. Brent Williams Fellows Maher Al Kuwaiti Shafiqa Al Sharif Nesreen Al-Wallan Ahmed Alterkait Debasis Das Adhikari Yousef Etoom Roaa Jamjoom Anna Kempinska Eric Koelink Dina Kulik Charisse Kwan Caitriona Mullarkey Quang Ngo Isabelle Steiner Mahmoud Sakran Emad Siddiqui Tanya Solano Kirstin Weerdenburg Administrative Staff Brenda Brady Caseita Dewar Valerie Foote Angie Frisk Ritsa Iriotakis Julia Kim Sandra Miller Majda Savaglio Pauline Smith Annabel Sousa Maggie Stevanovic Wendy Topham Catherine Wong The academic year for the Division of Paediatric Emergency Medicine can be summarized in two words busy and productive. We certainly were busy busy with patients (over 63,000 visits!) and busy with the training of undergraduates and post-graduate learners (more than 450 in the art and science of paediatric emergency medicine). And the academic year was certainly productive, both in terms of scholarly output and grants awarded to our faculty. The story for clinical operations in was the story of SPEED Streamlining the Patient Experience from Entry to Discharge. The department made substantial gains in efficiency through the improvement ideas generated and put into practice by a multi-disciplinary group of ED and hospital leaders, including our operations director Dr. Trent Mizzi. The SPEED initiative conceptualized and proved the value of a rapid assessment zone model of care to focus attention on our largest segment of patients those judged as too sick for urgent care, but not so sick as to require immediate resuscitation and care. These efficiency gains translated into the hospital gaining close to a million dollars in variable funding awarded by the Ministry of Health. Grant funding exceeded 3 million dollars and was achieved across 10 different faculty and fellows within the Division Academic success was visible on many fronts. Dr. Kathy Boutis was promoted to Associate Professor of Paediatrics on the strength of her clinical research program in musculoskeletal injuries. Dr. Jason Fischer was awarded the outstanding clinical teacher prize from the University of Toronto Emergency Medicine Program. Dr. Fischer also established the first emergency ultrasound fellowship training program for PEM in Canada in fall Divisional faculty published more than 35 scientific papers and contributed to five different books defining best practice in our field. Grant funding exceeding 3 million dollars was achieved across 10 different faculty and fellows within the Division, and that figure does not include the federal grant awarded to a consortium of academic PEM centers and general emergency departments entitled TREKK (Translating Emergency Knowledge for Kids). The Division of PEM at SickKids is a coordinating node TREKK in southwestern Ontario, and Dr. Stephen Porter will serve as the Ontario lead for the effort. department of paediatrics Annual Report

78 Honors and Awards Internal Finkelstein, Y: Winner of Career Development Award. Division of Emergency Medicine, The Hospital for Sick Children, Toronto, Ontario, Fischer, J: Winner of the James C. Fallis Teaching Award. Division of Emergency Medicine, The Hospital for Sick Children, Fischer, J: Winner of the Outstanding Scholar Award. Division of Emergency Medicine, The Hospital for Sick Children, Lalani, A: Award for Excellence in Leadership Role. Division of Pediatric Emergency Medicine, The Hospital for Sick Children, Ratnapalan, S: Winner of Communicator Award. Paediatric Emergency Medicine. The Hospital for Sick Children, Schneeweiss, S: Winner of Excellence in Leadership Role. Division of Paediatric Emergency Medicine, The Hospital for Sick Children, Scolnik, D: Winner of Excellence in Health Advocate Role. Division of Paediatric Emergency Medicine, The Hospital for Sick Children, External Finkelstein, Y: Honorable Mention for the 2012 Ronald D. Mann Best Paper Award in Pharmcoepidemiology and Drug Safety. International Society of Pharmacoepidemiology (ISPE) and Wiley-Blackwell ( Palatability, adherence and prescribing patterns of antiretroviral drugs for children with human immunodeficiency virus infection in Canada, ) (Senior Author), presented at the 8th International Conference on Pharmacoepidemiology and Therapeutic Risk Management, Barcelona, Spain, Jan to December Fischer, J: Winner of the Outstanding Clinical Teacher Award. University of Toronto Emergency Medicine, Toronto, Publications Al Odhayani A, Ratnapalan S. Teaching communication skills. Canadian Family Physician 2011: 57 (10): pp Brown K, Cieslak P, Cohen-Silver, Jarvis A, Mounstephen W, Ratnapalan S. Charting errors in a teaching hospital. Pediatric Emergency Care 2012: 28 (3): pp Burry E, Punnett A, Mehta A, Thull-Freedman J, Robinson L, Gupta S. Identification of educational and infrastructural barriers to prompt antibiotic delivery in febrile neutropenia: A quality improvement initiative. Pediatric Blood Cancer 2011: Dec 6 (Epub ahead of print). Cieslak P, McEvoy J, Mizzi T, Mounstephen W, Ratnapalan S. Physicians perceptions of background noise in a pediatric emergency department. Pediatric Emergency Care 2011: 27 (9): pp Finkelstein Y, Hutson JR, Wax P, Brent J. Toxico-surveillance of infant and toddler poisoning in the United States: Use of the Toxicology Investigators Consortium (ToxIC) Registry. Journal Medical Toxicology 2012: April 20 (Epub ahead of print). Finkelstein Y, Schechter T, Lim A, Kahr W, Williams S, Chan AL, Deveber G, Brandao LR. Unfractionated heparin dosing in young infants: Clinical outcomes in a cohort monitored by Anti-Xa levels. Journal of Thrombosis and Haemostasis 2012: 10(3): pp Finkelstein Y, Soon G, Acuna P, George M, Pope E, Ito S, Shear N, Koren G, Shannon MW, Garcia-Bournissen F. Recurrence and long-term outcome of Stevens-Johnson syndrome and toxic epidermal necrolysis in children. Pediatrics 2011: 128 (4): pp Freedman SB, Parkin PC, Willan AR, Schuh S. Rapid versus standard intravenous rehydration in paediatric gastroenteritis: A pragmatic, blinded, randomised clinical trial. British Medical Journal 2011: 343: d6976. doi: /bmj.d6976. Freedman SB, Stewart C, Rumantir M, Thull-Freedman JD. Predictors of clinically significant upper gastrointestinal hemorrhage amongst children with hematemesis presenting to an emergency department. Journal of Pediatric Gastroenterology, Hepatology and Nutrition 2012: 54(6): pp Freedman SB, Tung C, Cho D, Rumantir M, Chan KJ. Time series analysis of ondansetron use in pediatric gastroenteritis. Journal of Pediatric Gastroenterology, Hepatology and Nutrition 2012: 54(3): pp Frenkel O, Mansour K, Fischer JW. Ultrasound-guided femoral nerve block for pain control in an infant with a femur fracture due to nonaccidental trauma. Pediatric Emergency Care 2012: 28(2): pp Glatstein M, Constantini S, Scolnik D, Shimoni N, Roth J. Ventriculo-peritoneal shunt catheter protrusion through the anus: Case report of an uncommon complication and literature review. Child s Nervous System 2011: Aug 7 (Epub ahead of print). Glatstein MM, Roth J, Scolnik D, Haham A, Rimon A, Koren L, Constantini S. Late presentation of massive pleural effusion from intrathoracic migration of a ventriculoperitoneal shunt catheter. Case report and review of the literature. Pediatric Emergency Care Journal 2012: 28(2): pp Glatstein M, Rozen R, Scolnik D, Rimon A, Grisaru-Soen G, Freedman S, Reif S. Radiologic predictors of hyponatremia in children hospitalized with community-acquired pneumonia. Pediatric Emergency Care Journal 2012: 28(8): pp Glatstein M, Scolnik D, Bensira L, Domany KA, Shan M, Vala S. Lung abscess due to non-tuberculous, non-mycobacterium fortuitum in a neonate. Pediatric Pulmonology Journal 2012: Mar 26. doi: /ppul (Epub ahead of print). Goldman R, Kapoor A, Mehta SV. Children admitted to the hospital after returning to the emergency room within 72 hours. Pediatric Emergency Care 2011: 27(9): pp Gravel J, Gouin S, Goldman RD, Osmond MH, Fitzpatrick E, Boutis K, Guimont C, Joubert G, Millar K, Curtis S, Sinclair D, Amre D. The Canadian Triage and Acuity Scale for Children: A prospective multi-centre evaluation. Annals of Emergency Medicine 2012 (Epub ahead of print). Hoppa EC, Porter SC. A content analysis of parents written communication of needs and expectations for emergency care of their children. Pediatric Emergency Care 2011: 27: pp Jaskolka D, Brown N, Cohen E, Connolly B, Mounstephen W. Evaluating the implementation of a QI initiative: Weekend gastrojejunostomy tube maintenance service in a tertiary pediatric center. Canadian Association of Radiology Journal 2012: April 12 (Epub ahead of print) PMID: Kinlin L, Freedman SB. Evaluation of a clinical dehydration scale in children requiring intravenous rehydration. Pediatrics 2012: 129:5 e1211-e1219; published ahead of print April 23, doi: /peds Lin D, Seabrook JA, Matsui DM, King SM, Rieder MJ, Finkelstein Y. Palatability, adherence and prescribing patterns of antiretroviral drugs for children with human immunodeficiency virus infection in Canada. Pharmacoepidemiology and Drug Safety 2011: 20(12): pp Mehta S, American College of Emergency Physicians. Boarding of pediatric patients in the emergency department. Annals of Emergency Medicine 2012: 59(5): pp Nurmohamed L, Moretti ME, Schechter T, Einarson A, Johnson D, Lavigne SV, Erebara A, Koren G, Finkelstein Y. Outcome following high-dose methotrexate in pregnancies misdiagnosed as ectopic. American Journal of Obstetrics and Gynecology 2011: 205 (6): pp 533 e1-3. Pusic MV, Andrews JS, Kessler DO, Teng DC, Pecaric MR, Ruzal-Shapiro C, Boutis K. Determining the optimal case mix of abnormals to normals for learning radiograph interpretation: A randomized control trial. Medical Education 2012: 46 (3): pp the hospital for sick children department of paediatrics Annual Report

79 Ratnapalan S, Brown K, Cieslak P, Cohen-Silver J, Jarvis A, Mounstephen W. Charting errors in a teaching hospital. Pediatric Emergency Care 2012: Feb 16 (Epub ahead of print). Ratnapalan S, Cieslak P, McEvoy J, Mizzi T, Mounstephen W. Physicians perceptions of background noise in a pediatric emergency department. Pediatric Emergency Care 2011: 27: (9) pp Ratnapalan S, Zaher E. Practice-based small group learning programs. Canadian Family Physician 2012: 58 (6): pp Schechter T, Kirton A, Laughlin S, Pontigon A, Finkelstein Y, Macgregor D, Chan A, Deveber G, Brandão LR. Safety of anticoagulants in children with arterial ischemic stroke. Blood 2012: 26:119(4): pp Somji Z, Plint A, MacGowen C, Al-Saleh A, Boutis K. Diagnostic coding of abusive fractures in two children s emergency departments. Child Abuse and Neglect 2011: 35 (11): pp Epub November 19, Thull-Freedman J. Of 3700 children thought to have noncardiac chest pain at initial paediatric cardiology clinic evaluation, none suffered cardiac death over a median of 4 years follow-up. Evidence-Based Medicine 2012: Apr 17. Valani R, Sriharan A, Scolnik D. Integrating CanMEDS competencies into global health electives: An innovative elective program. Canadian Journal of Emergency Medical Care 2011: 13(1): pp Willan A, Goeree R, Boutis K. Applying value of information methods in the evaluation of research evidence. Journal of Clinical Epidemiology 2012: 65 (8): pp Epub 2012 May 18. Books and Chapters Finkelstein Y, Browne AW, Dugani S, Hutson J, McSheffrey G, Stefater MA (eds.). Central nervous system drugs (Faculty contributor). In: Pharmacology You See: A High Yield Pharmacology Review for Health Professionals. Toronto Notes and McGraw Hill Publishers, Toronto 2011: pp Mehta SV. Chest pain. In: Fleisher and Ludwig s 5-Minute Pediatric Emergency Medicine Consult (Hoffman RJ, Wang V, Scarfone RJ, Godambe SA, Piletti RP, eds.). Lippincott Williams and Wilkins, Philadelphia, PA 2011: pp Porter SC. Emergency department clinical pathway for children and youth with mental health conditions/addictions Work Group. Report to the Ontario Ministry of Health s Child and Youth Advisory Committee. (Ministry appointed workgroup member). Ratnapalan S. Paediatric sedation. In: Essential Emergency Procedural Sedation and Pain Management. (Valani R, Shah KH, eds.) Lippincott Williams and Wilkins, Schneeweiss S. Pediatric pain management. In: Essential Emergency Procedural Sedation and Pain Management (Valani R, Shah KH, eds.). Lippincott Williams and Wilkins, Philadelphia, PA 2012: pp Scolnik D, Glatstein M. Intussusception associated with Henoch-Schonlein purpura. In: Gastrointestinal Disorders. ISBN Scolnik D, Glatstein M. Migraine headaches. In: Fleisher and Ludwig s 5-Minute Pediatric Emergency Medicine Consult. (Hoffman RJ, Wang VJ, Scarfone RJ, et al, eds.). Lippincott, Williams and Wilkins. Philadelphia, PA, Thull-Freedman J. Renal vein thrombosis. In: Fleisher and Ludwig s 5 Minute Pediatric Emergency Medicine Consult. (Hoffman R, et al, eds.) Lippincott, Williams and Wilkins. Philadelphia, PA, Grants and Funding Internal Fracture severity in overweight versus non-overweight children presenting to a paediatric emergency department. Kwan C, Howard A, Boutis K. RTSUF. $3,000 CAD. Jan 2011 to Dec 2012 Health advocacy training in pediatric emergency medicine fellowships in Canada and the US. Kwan C, Boutis K, Ratnapalan S, Lalani A. Division of Pediatric Emergency Medicine Research Funds. $3,875 CAD Ketamine neurotoxicity in children undergoing procedural sedation. Finkelstein Y, Boutis K, Hutchison J, Maynes J PEM Divisional Research Grant, Hospital for Sick Children. $7,500 CAD. Jan to Dec Pediatric Emergency Medicine Collaborative Research Grant. Fischer J. Division of Emergency Medicine. $3500 CAD. Feb 2012 Serum biomarkers of brain injury after administration of ketamine to young children: A pilot study. Finkelstein Y, Hutchison M, Post M, Solano M, Boutis K. Division of Pediatric Emergency Medicine Research Funds. $7,500 CAD. Jan 2012 External Abdominal x-ray use and the misdiagnosis rate in children with constipation. Freedman SB, Thull-Freedman J, Manson D, Schuh S, Rumantir M. Paediatric Consultants Partnership Grant for Creative Professional Activity. $9,641 CAD. Jan to Dec Cellular toxicity of anaesthetics in children with mitochondrial disease. Finkelstein Y, Hutson J, Boutis K, Maynes J. British Columbia Children s Hospital Foundation (BCCHF) and Rare Disease Foundation Award. $3,500 CAD. Jan to Dec Children with acute gastroenteritis: Predictors of increased length of stay in the emergency department. Schindler N, Freedman SB, Black, K. IWK Health Centre. $2,000 CAD. Jan to Dec Dating fractures on radiographs: How accurate is this compared to post-mortem histopathology. Shouldice M, Moharir M, Keppron C, Chiasson D, Stimec J, Lubo-Mueller E, Boutis K. Pediatric Consultants, Hospital for Sick Children. $7,455 CAD. Aug 2011 to Sep 2013 Dose-response efficacy and safety of IGF-1 in girls with Rett syndrome. Finkelstein Y, Khwaja O, Rappaport L, Holm I, Sur M, Gregas M Ontario Rett Syndrome Association Research Grant Award. $25,000 CAD. Jan to Dec Evaluating the impact of emergency room services for children and adolescents with autism spectrum disorder. Zwaigenbaum L, Nicholas DB, Roberts W, Muskat B, Newton A, Ratnapalan S, Sharon R, Cohen-Silver J. Autism Treatment Network / Autism Intervention Research Network on Physical Health. $299,583 CAD Hinting strategies for improving the efficiency of medical student learning of deliberately practiced web-based radiographs. Boutis K, Pusic M, Pecaric M, Kalet A, Shiau M. Educational Dean s Fund. $20,000 CAD. Sep 2011 to Sep the hospital for sick children department of paediatrics Annual Report

80 Identifying genomic determinants associated with ketamine neurotoxicity in young children. Finkelstein Y, Hutchison M, Post M, Boutis K, Kim R. McLaughlin Centre. $50,000 CAD. Mar 2012 Impact of emergency department probiotict treatment of pediatric gastroenteritis: Randomized controlled trial. Freedman SB, Schuh S, Farion K, Sherman P, Johnson D, Willan A, Goeree R, Gouin S, O Connell K, Roskind C, Gorelick M. The Canadian Institute for Health Research (CIHR) $1,500,000 CAD. Jan to Dec Impact of emergency department probiotic treatment of pediatric gastroenteritis: Randomized controlled trial. Freedman SB, Schuh S, Farion K, Sherman P, Johnson D, Willan A, Goeree R, Gouin S, O Connell K, Roskind C, Gorelick M, Schnadower D. The National Institute of Health. $2,210,029 CAD. Jan to Dec Implementation of emergency response team within a pediatric emergency department and a mixed methods examination of staff perception of team effectiveness. Thull-Freedman J, Etoom Y, Capstick C, Jeffs L. SickKids Paediatric International Patient Safety and Quality Funding Opportunity. $1,500 CAD to 2013 Inhaled magnesium in refractory pediatric asthma. Schuh S, Freedman SB, Ducharme F, Black K, Johnson D, Beer D, Plint A, Zemek R, Guimont C, Willan A, Thompson G, Coates A. Thrasher Research Fund. $500,920 CAD. Jan to Dec Intentional self-poisoning in Canadian teens: Identifying risk factors for future suicide and repeat attempts to facilitate secondary prevention A population-based study. Finkelstein Y, Juurlink D, Sivilotti M. Pediatric Consultants Partnership Grant for Creative Professional Activity, Hospital for Sick Children. $9,800 CAD. Jan to Dec Learning retention and the optimal timing of refresher education. Boutis K, Pusic M, Pecaric M, Beckstead J, Babyn P. Royal College Medical Education Research Grant. $40,000 CAD. Apr 2011 to Dec 2014 Management of acute gastroenteritis: Synthesizing evidence to inform North American practice. Freedman SB, Hartling L, Johnson D, Gouin S, Black K, Fitzpatrick E, Bialy L, Klassen T, Belanger F, Hilliard B, Jabbour M. Canadian Institutes of Health Research. $100,000 CAD. Jan to Dec Ondansetron use in children with dehydration in a developing country. Freedman SB, Bhutta Z. Gates Foundation. $100,000 CAD. Jan to Dec Ondansetron use in children with dehydration in a developing country. Freedman SB, Bhutta Z. Thrasher Research Foundation. $400,000 CAD. Jan to Dec Pediatric Research and Clinical Summer (PeRCS) Program Award, Hospital for Sick Children. (Student: Hutson J) $2,500 CAD. Jan to Dec Pharmacogenetic predictors of toxicity and cognitive deficits among leukemia survivors. Cole P, Finkelstein Y, Waber D, Dahlberg S. St. Baldrick s Foundation 2012 Research Grant. $50,000 US. Jan to Dec Pharmacogenomic determinants of ketamine neurotoxicity in young vhildren. Finkelstein Y, Boutis K, Hutchison J, Post M, Soriano S, Kim R. McLauglin Centre for Genomic Medicine 2012 Accelorator Grant, University of Toronto. $50,000 CAD. Jan to Dec Pharmacological treatment of Rett syndrome by stimulation of synaptic maturation with IGF-1. Khwaja O, Shannon M, Rappaport L, Holm I, Sur M, Gregas M, Finkelstein Y. Harvard University s Catalyst Pilot Grant for Clinical and Translational Research. $50,000 US. Jan to Dec Pharmacological treatment of Rett syndrome by stimulation of synaptic maturation with IGF-1. Khwaja O, Shannon M, Rappaport L, Holm I, Sur M, Gregas M, Finkelstein Y. International Rett Syndrome Association. $200,000 US. Jan to Dec Paramedics attitude towards death notification in the field. Douglas L, Cheskes S, Feldman M, Ratnapalan S. Paramedics Research Consortium $14,074 CAD to 2013 Population based cohort to determine effect of ED pediatric gastroenteritis strategies on admission and return visit rates. Bahm A, Freedman SB, Guttmann A. Physicians Services Incorporated. $20,000 CAD. Jan to Dec Prognosticators of persistent concussion symptoms following pediatric minor traumatic head injury. Zemek RL, Osmond M, Vassilyadi M, Freedman SB. Canadian Institutes of Health Research. $25,000 CAD. Jan to Dec Rectal amoxicillin An alternative route for pediatric delivery? Tessaro M, Finkelstein Y, Scolnik D. Apotex. $15,000 CAD to 2012 Salter-Harris I fracture of the distal fibula: Clinical versus reality. Boutis K, Narayanan U, Schuh S, Plint A, Stimec J, Miller E, Brison R. Physician Services Incorporated. $170,000 CAD. Jan 2012 to Dec 2014 The association between parental literacy and language fluency, understanding of discharge instructions and outcomes of care for children with respiratory illness. Stack A, Damian F, Samuels-Kalow M, Meginniss A, Nolan M, Porter S. Quality Improvement Grant (PPSQ, Children s Hospital Boston) $16,959 USD. Jun 2010 to present The association between parental understanding and implementation of discharge instructions and outcomes of care for infants with viral respiratory illness. Samuels-Kalow M, Porter S, Stack A. Resident Research Grant, Emergency Medicine Foundation $ 5,000 CAD. Jul 2010 to Jul 2011 The pharamcokinetics of antibiotics delivered in the emergency department setting. Scolnik D, Finkelstein Y, Tessaro M. Apotex Fund. $15,000 CAD. Jan to Dec The learning curve of elbow radiograph interpretation. Boutis K, Pusic M, Pecaric M. Pediatric Consultants, Medical Education. $5,000 CAD. Feb 2011 to Mar 2012 Translating emergency knowledge for kids (TREKK). Klassen T, Hartling L, Jabbour M, Johnson D, Scott S, Sinclair D, Lang E, Freedman SB, Black B, Gouin S, Stang A. Networks of Centres of Excellence (NCE) Program - Knowledge Mobilization. $1,200,000 CAD. Jan to Dec the hospital for sick children department of paediatrics Annual Report

81 PAEDIATRIC MEDICINE overview Division Head Dr. Jeremy Friedman Full-Time Dr. Hosanna Au Dr. Carolyn Beck Dr. Stacey Bernstein Dr. Catherine Birken Dr. Zia Bismilla Dr. Trey Coffey Dr. Eyal Cohen Dr. Emma Cory Dr. Astrid Guttmann Dr. Paul Kadar Dr. Irene Lara-Corrales Dr. Sanjay Mahant Dr. Charlotte Moore Hepburn Dr. Patricia Parkin Dr. Elena Pope Dr. Adam Rapoport Dr. Daniel Roth Dr. Michelle Shouldice Dr. Susan Tallett Dr. Michael Weinstein Dr. Randi Zlotnik Shaul Major Part-Time Dr. Mark Feldman Dr. Saul Greenberg Dr. Christine Newman Dr. Michael Peer Dr. Miriam Weinstein Part-Time Dr. Tony Barozzino Dr. Nhung Ho Dr. Moshe Ipp Dr. Sheila Jacobson Dr. Perla Lansang Dr. Michael Sgro Dr. Robert Hilliard Associate Dr. Nirit Bernhard Dr. Burke Baird Dr. Beth Gamulka Dr. Marissa Joseph Dr. Ronik Kanani Dr. Patricia Li Dr. Sanjeev Luthra Dr. Jonathon Maguire Dr. Yehuda Nofech-Mozes Dr. Gagandeep Saund Dr. Sarah Schwartz Dr. Shawna Silver Dr. Anne Wormsbecker Adjunct Dr. Uzma Ahmed Dr. Ibrahim Al-Hashmi Dr. Murrog Aldabbagh Dr. Tara Allen Dr. Anwar Asady Dr. Celia (Taha) Allen Dr. Sanja Avdic Dr. Peter Azzopardi Dr. Ranjit Baboolal Dr. Helen Bai Dr. Steven Baker Dr. Anna Banerji Dr. Alan Berzen Dr. Ann Bayliss Dr. Kesele Bezu Dr. Melanie Binnington Dr. Nicholas Blanchette Dr. Caroline Calpin Dr. Salvatore Cammisuli Dr. Jordan Carr Dr. Avril Castagna Dr. Luigi Castagna Dr. Bert Cellupica Dr. Sohail Cheema Dr. Chee Chen Dr. Brian Chisamore Dr. Patricia Colangelo Dr. Carlos E. Contreras Dr. Erin Culbert Dr. Karoon Danayan Dr. Hilary de Veber Dr. Shalini Desai Dr. Yenge Diambomba Dr. Paul Dick Dr. Suhid (Tony) D Souza Dr. Carol Durno Dr. Prabo Dwight Dr. Gian Egger Dr. Stephen Richard Feanny Dr. Diane Flanders Dr. Claudio Fregonas Dr. Gerald Friedman Dr. Marvin H. Gans Dr. Hartley A. Garfield Dr. Donna Goldenberg Dr. Leslie Goodis Dr. Michelle Gordon Dr. Masoud Grouhi Dr. Denis Gurwitz Dr. Katarzyna Gwiazda Dr. Shujauddin Hafiz Dr. Mark E. Handley-Derry Dr. Miriam Hansen Dr. Jane Healey Dr. Rania Hiram-Karasnis Dr. Vincent Ho Dr. Alan P. Hudak Dr. Carolyn Hunt Dr. Clare Hutchinson Dr. Dayanand Jagdeo Dr. Edward G. Jin Dr. Rhoda Kagan Dr. Peggy Kirkpatrick Dr. Dror Koltin Dr. Susan Kovacs Dr. Beverley Kupfert Dr. Eddy Lau Dr. Ho Yin Lau Dr. Zina Levichek Dr. Maurice Levy Dr. James MacKinnon The Division (comprising General Paediatrics, Community Paediatrics, SCAN, Dermatology and Palliative Care) continues to play an integral role in the core activities of the Department of Paediatrics. On the inpatient unit we continue to see increases in patient volumes (3,626 discharges in 11/12). This amounts to more than 22 per cent of the total hospital discharges and over 50 per cent of the admissions from the ED. Despite the increases in volume and an ongoing gradual increase in acuity and complexity, the statistics for time spent in the ED waiting for a bed have improved dramatically. There has been a slight increase in funded nursed beds but these results have been achieved primarily through some excellent innovations including reorganization of our existing resources with the opening of a streamlined care unit and an ambulatory daycare program, and improving the process for bed management. This increased efficiency has been achieved without sacrificing quality, as evidenced by continued excellent results in the Picker patient satisfaction survey. This is the result of an active group working in Quality Improvement led by Trey Coffey. The group has focused particularly on family-centered rounds, medication reconciliation, development of CPGs (bronchiolitis, asthma) and has just begun a project aimed at improving the discharge process. The Complex Care Program continues to develop into a national and international model of care for children requiring complex care. The program was recognized by Accreditation Canada as a leading practice and the number of visiting delegations from the US, Europe and elsewhere speaks to the innovative work being done by the team. Academic productivity, publications and grant funding continues to increase and educational opportunities continue to be developed. The new Janis Rotman Innovations in Homecare research fellowship program with its first two fellows starting in 2012 will be closely aligned with the program and build on the community/home aspect. This is a tremendously engaged and passionate group with a great commitment to the vision and mission of the hospital and the department. department of paediatrics Annual Report

82 Dr. Laura McAdam Dr. Aubrey Maze Dr. Anilkumar Menon Dr. Saba Merchant Dr. Diana Mills-Tettey Dr. Golda Milo-Manson Dr. Antonella Morra Dr. Steven Moss Dr. Shudeshna Nag Dr. Sharon Naymark Dr. Patricia Neelands Dr. Marjan Nezarati Dr. Lynne Nield Dr. Angela Orsino Dr. Ivan Osswald Dr. Indira Pachai Dr. Marty Perlmutar Dr. Constantine Petrou Dr. John Philpott Dr. Michelle Porepa Dr. Vladimir Pucholt Dr. Seetha Radhakrishnan Dr. Kushal Raghubir Dr. Harkamal Randhawa Dr. Lillian Rojnica Dr. Karen Rosenstein Dr. Tania Samanta Dr. Janet Saunderson Dr. Rahul Saxena Dr. Clive J. Schwartz Dr. Furquan Shaikh Dr. David H. Shawn Dr. Angelo Simone Dr. Nerandra Singh Dr. Shirley Sit Dr. William Gary Smith Dr. Jill Solomon Dr. Diann Superina Dr. Wanda Szymonowicz Dr. Leah Tatum Dr. Catherine Taylor Dr. Lindsay Teskey Dr. Maria Triassi-Asper Dr. Julia Upton Dr. Charmaine van Schaik Dr. Camilla Vaz Dr. Guy Widrich Dr. Peter D. Wong Dr. Hiro Yamishiro Dr. Heather Yang Dr. Latifa Yeung Dr. Ethel Ying Dr. Mostafa Zaazou Dr. Michael Zajdman Cross-appointees Dr. Glen B. Berall Dr. Elizabeth Berger Dr. Bonnie J. Cameron Dr. Douglas Campbell Dr. Gordon J. Donsky Dr. Katy Driver Dr. Leona Fishman Dr. Janine Flanagan Dr. Lee Ann Gallant Dr. Rose Geist Dr. Sabine R. George Dr. William B. Hanley Dr. Jaya Islur Dr. D. Anna Jarvis Dr. Ian C. Kitai Dr. Howard M. Langer Dr. Leo Levin Dr. Patricia M. Linders Dr. Gilbert Miller Dr. Hadi Mohsenibod Dr. John D.M. Murray Dr. Larry B. Pancer Dr. Jay D. Rosenfield Dr. Suzanne Schuh Dr. Jeffrey J. Weisbrot Dr. Sheldon A. Wise Honorary Dr. Lionel B.H. Boxall Dr. Andrew Chlebus Dr. Till A. Davy Dr. William Davidson Dr. James Dorey Dr. Andrew D. Duic Dr. Anthony S.R. Duke Dr. Robert Farber Dr. Michelle Farine Dr. Hyder H. Fazal Dr. William Feldman Dr. Adelaide L. Fleming Dr. Raphael Folman Dr. John S. Freedman Dr. Morton Goldbach Dr. Stuart Gordner Dr. James D. Hilton Dr. Tommy Ho Dr. Anthony Y.T. Hui Dr. Dirk W. Huyer Dr. Martin H. Kosoy Dr. Cinda B.S. Lee Dr. Michael Lester Dr. David C. Maresky Dr. Marcellina Mian Dr. Janette A. Milne Dr. Paul Munk Dr. Jack Newman Dr. Raymond C.W. Ng Dr. Evangelia Papadouris Dr. S. Joseph Porepa Dr. Percy Rajchgot Dr. Helene A. Richards Dr. Chandrakant P. Shah Dr. Peter G. Strachan Dr. Robert E. Sutton Dr. Joseph Telch Fellows Dr. Kawsari Abdullah Dr. Javier Adrian Gutierrez Dr. Fadiah Al-Khattabi Dr. Elizabeth Berger Dr. Ajith Chakkittakandiyil Dr. Mara Cole Dr. Maria Garcia Romero Dr. Akhter Hamid Dr. Katie Mennie Dr. Niraj Mistry Dr. Javed Mohammed Dr. Julia Morinis Dr. Tram Nguyen Dr. Claudia Posso de la Rios Dr. Harkamal Randhawa Dr. Balaji Rengarajan Dr. Gustavo Rubio Gomez The Division s role in education has continued its excellence at all levels of training; medical students, core residents and fellows. Much attention has been paid and will continue to be required to ensure that the inpatient experience remains the most highly ranked clinical rotation for both the junior and senior residents at SickKids. Individual faculty continue to win a significant number of the departmental teaching awards. While Susan Tallett has just retired as the Director of the SickKids Learning Institute, Stacey Bernstein has been appointed as the University of Toronto Clerkship Director. The fellowship program has grown in size, scope and stature with 25 fellows in various fellowship tracks for the year. Graduates of the program have left to take up faculty positions in general paediatrics across the country and internationally. Research in the Division has grown in leaps and bounds. The focus is primarily through the two arms of the divisional research group (PORT); community-based paediatric outcomes (eg Vitamin D, obesity, iron deficiency) and hospitalist research (eg complex pneumonia, IV fluids, complex care). TARGet Kids!, an initiative of the PORT group, is breaking new ground in community-based research and has been highly successful in rapidly building a network of primary care researchers and achieving grant funding and publications. In addition, we have faculty doing outstanding research in areas which include Population Health Science, Global Health, and Paediatric Dermatology. Funding through major grants is in the millions of dollars through external granting agencies like CIHR, PSI, Thrasher Foundation, Bill and Melinda Gates Foundation etc. Publications in high-impact journals have grown exponentially. A new focus for the Division over the last three years is the area of child health policy, through the hiring of Charlotte Moore Hepburn to lead this initiative with strong support from other faculty involved in population health, complex care and global health. This initiative is in its early stages but promises to add a strategically important piece to the Division in terms of advocacy and evidence-based research into policy changes, as well as promoting new models of care delivery. Two of the founding members of our Division, Robert Hilliard and Susan Tallett, have retired as full-time staff after illustrious careers spanning more than 30 years. The division celebrated its 30th year anniversary in the fall of 2011 and simultaneously recognized the contributions of these two outstanding contributors and original division members with a day of presentations by current faculty as well as the first presentation of divisional awards for outstanding contributors. Individual faculty have excelled and shown leadership on the national and international stage in the clinical, educational, research and advocacy spheres. Just a few examples from the last few years include Astrid Guttmann s many achievements in population health research; Patricia Parkin, Jonathon Maguire and Catherine Birken s exciting leadership and successes in building a community research network through TARGet Kids!; Eyal Cohen s research and leadership in developing new models of care for children with complex needs; Sanjay Mahant s leadership of the hospitalist section in Canada and his involvement in the new PRIS initiative in North America; Michelle Shouldice s leadership and development of collaborations on the Canadian SCAN scene; and Stacey Bernstein s dynamic leadership in undergraduate medical education culminating in her appointment as the new clerkship director at the University of Toronto. In addition to all the individual awards and accolades, what remains clear is the tremendously high morale and camaraderie amongst divisional faculty. This is a tremendously engaged and passionate group with a great commitment to the vision and mission of the hospital and the department. Honors and Awards Barozzino, T: Awarded the Postgraduate Community Paediatric Teaching Award (excellence in teaching communitybased paediatric residents), Department of Paediatrics, University of Toronto, June Birken, C: Awarded the Dr. William Feldman Award for Excellence in Paediatric Medicine Research, Division of Paediatric Medicine, Hospital for Sick Children, Toronto, October 18, Campbell, D: Awarded the Marvin Gerstein Award, Postgraduate Teaching (nominated by core paediatric residents for excellence in teaching), Department of Paediatrics, University of Toronto,June Cohen, E Lacombe-Duncan, A, Keilty K, Bruce-Barrett C, Kingsnorth S, Floyd K: Winner - Patient/Family-Centered Care Award, 8th Annual Paediatric Patient Safety Symposium - Optimizing Quality in the Era of Efficiency, The Hospital for Sick Children, Abstract Inter-organizational Partnership for Children with Medical Complexity: The Integrated Complex Care Model Cohen, E: Promotion: Associate Professor, Department of Pediatrics, University of Toronto, June Friedman, J: Promotion: Professor, Department of Paediatrics, University of Toronto, June Ipp, M et al: Canadian Society for Pharmacology and Therapeutics Annual Publication Award for best original research : Reducing the pain of childhood vaccination: an evidence-based clinical practice guideline. Ipp, M, Taddio A: 2012 Noni MacDonald Award - Paediatric and Child Health Journal Award for published article that made an important contribution to paediatric practice Pain management during vaccination: Recommendations from a Canadian clinical practice guideline. Jacobson, S: Awarded the R.H.A. Haslam Preclerkship Teaching Award (excellence in preclerkship teaching), Department of Paediatrics, University of Toronto, June Kupfert, B, Yang, H: Awarded the Lionel Weinstein Clerkship Award (excellence in clerkship teaching), Department of Paediatrics, University of Toronto, June Parkin, P: Physician Research Award for Career Excellence, Department of Paediatrics, Hospital for Sick Children, June Peer, M, Shouldice, M, Silver, S: Winner of POWER Teacher Award (for outstanding contributions to postgraduate teaching), Department of Paediatrics, University of Toronto, June Pope, E: Awarded Clinical Excellence Award 2011, Division of Paediatric Medicine, Hospital for Sick Children, October 18, Pope, E, Chakkittakandiyil A, Weinstein M: Third Place, Poster Award: Society for Pediatric Dermatology 37th Annual Meeting 2011, Baltimore, Maryland, USA: Expanding the therapeutic repertoire of infantile hemangiomas: prospective pilot study using oral Nadolol (poster). Society for Pediatric Dermatology, Baltimore, Maryland, USA. Jul 9, 2011 Shouldice, M: Winner of Robert Hilliard Award for Excellence in Paediatric Medicine Education, The Hospital for Sick Children, Division of Paediatric Medicine, October 18, department of paediatrics Annual Report

83 Dr. Sarah Schwartz Dr. Abdul Shahein Dr. Anne Wormsbecker Research Associates/Students Mrudula Avileli Sarah Carsley Lucy Chen Olivia Cook Tonya D Amour Jessica Danquah Julie DeGroot Jasmine Dunston Inam Elahi Hanna Fadzeyeva Renee Farrell Nicole Fischer Matea Florentina-Nadia Anne Fuller Sara Glazer Kaitlin Graham Tara Hahmann Kun (Eric) Huh Haleh Javidnia Kanthi Kavikondala Marina Khovratovich Sarah Kwan Ashley Lacombe-Duncan Megan Laupacis Shelly-Anne Li Tina Li Bradley Lichtblau Jennifer MacInnis Tarandeep Malhi Janet McMullen Jessica Omand Nandita Perumal Brendon Pezzack David Pirrello Victor Pope Laura Quigley Subitha Rajakumaran Sudha Raman Leila Salehi Juela Sejdo Flora Shan Gaurav Sharma Kim Simpson Tasha Stoltz Laurie Thompson Adrian Tsang Jane Wang Jeanne Zeller Administrative Support Staff Tiziana Altobelli Jackee Colquhoun Cindy Groff Heather Haywood Jasmine Hylton Jennifer Kingston Josie Marcantonio Daffodil Morrison Kathryn Robertson Dove Steele Christina Stevancec Donna Whitely Weinstein, M: Awarded the Claus Wirsig Humanitarian Award, Toronto, June Weisbrot, J: Awarded the Undergraduate Community Teaching Award (excellence in teaching medical students), Department of Paediatrics, University of Toronto, June Publications Adler D, Zlotnik Shaul R. Disciplining bioethics: Towards a standard of methodological rigor in bioethics research. Accountability in Research 2012: 19(3): pp Al Hawsawi K, Pope E. Pediatric psychocutaneous disorders: A review of primary psychiatric disorders with dermatological manifestations. American Journal of Clinical Dermatology 2011: 12(4): Amit M, Cummings C, Feldman M, Gander S, Grueger B, Rowan-Legg A, Canadian Paediatric Society, Community Paediatrics Committee. Melatonin for the management of sleep disorders in children and adolescents. Paediatrics and Child Health 2012: 17(6): pp Balma Mena A, Chakkittakandiyil A, Weinstein M, Lansang P, Ho N, Cammisuli S, Pope E. Propanolol in the management of infantile hemangiomas: Clinical response and predictors. Journal of Cutaneous Medicine and Surgery 2012: 16(3): pp Balma A, Weinstein M, Rosenthal A, Henry P, Gahzarian D, Miller J, Maul E, McGuirl J, Moorthy L. Index of Suspicion Case 1: Pruritic, erythematous, papular, and papulovesicular rash in an 8-year-old, Case 2: Cough with green phlegm in an 8-year-old, Case 3: Polyarthritis, Raynaud phenomenon, hoarseness, tapering fingers, and digital ulcers in a 6-year-old. Pediatric Review 2011: 32(8): pp Bell E, Shouldice M, Levin A. Abusive head trauma: A perpetrator confesses. Child Abuse and Neglect 2011: 35(1): pp Berger E, Kuper A, Albert M, Shouldice M. The CanMEDS portfolio: A tool for reflection in a fellowship program. Clinical Teacher 2011: 8(3) pp: Berry JG, Agrawal R, Kuo D, Cohen E, Risko W, Hall M, Srivastava R. Characteristics of hospitalizations for patients who utilize a structured clinical-care program for children with medical complexity. Journal of Pediatrics 2011: 159: pp Birtwhistle R, Pottie K, Shaw E, Dickinson J, Brauer P, Fortin M, Bell N, Singh H, Tonelli M, Connor Gorber S, Lewin G, Joffres M, Parkin P. Commentary: Canadian Task Force on Preventive Healthcare: We re Back! Canadian Family Physician 2012: 58: pp Chakkittakandiyil A, Phillips R, Frieden IJ, Siegfried E, Lara-Corrales I, Lam J, Bergmann J, Bekhor P, Poorsattar S, Pope E. Timolol Maleate 0.5% or 0.1% gel-forming solution for infantile hemangiomas: A retrospective, multicenter, cohort study. Pediatric Dermatology 2012: 29(1): pp Chiara J, Luca N, Lara-Corrales I, Pope E. Eczema herpeticum in children: Clinical features and factors predictive of hospitalization. Journal of Pediatrics 2012: May 9. (Epub ahead of print) PMID Chibuk T, Cohen E, Robinson JL, Mahant S, Hartfield D. Pediatric complicated pneumonia: Diagnosis and management of empyema. Paediatrics and Child Health 2011: 16(7): pp Chiu YE, Drolet B, Blei F, Carcao M, Fangusaro J, Kelly ME, Krol A, Lofgren S, Mancini AJ, Metry DW, Recht M, Silverman RA, Tom WL, Pope E. Variable response to Propanolol treatment of kaposiform hemangioendothelioma, tufted angioma, and Kasabach-Merritt phenomenon. Pediatric Blood and Cancer 2012: Feb (Epub ahead of print) PMID Coffey M, Laxer RM. Quality and safety in pediatric complex care. Current Pediatric Reviews 2012: 8(2): pp Cohen E, Bruce-Barrett C, Kingsnorth S, Keilty K, Cooper A, Daub S. The Integrated Complex Care Model (ICCM): Lessons learned from inter-organizational partnership. Healthcare Quarterly 2011: 14: pp Cohen E, Friedman JN. Caring for children with medical complexity: Definitions, challenges and solutions. Current Pediatric Reviews 2012: 8(2): pp Cummings C, Feldman M, Gander S, Grueger B, Rowan-Legg A, Nieman P, Canadian Paediatric Society (Community Paediatrics Committee). Positional plagiocephaly. Paediatrics and Child Health 2011: 16(8): pp Cummings C, Feldman M, Grueger B, Rowan-Legg A, Nieman P, Canadian Paediatric Society, Community Paediatrics Section, Consultants: Lang M, Patel H, Rowan-Legg A. Managing functional constipation in children. Paediatrics and Child Health 2011: 16(10): pp Czoli C, Da Silva M, Zlotnik Shaul R, d Agincourt-Canning L, Simpson C, Boydell K, Rashkovan N, Vanin S. Accountability and pediatric physician-researchers: Are theoretical models compatible with Canadian lived experience? Philosophy Ethics and Humanities in Medicine 2011: 6(15). Daneman D, Friedman JN. Hot Topic: Children who are medically fragile. Current Pediatric Reviews 2012: 8(2): pp Drolet BA, Pope E, Juern A, Sata A, Howell B, Brown Puttgen K, Lara-Corrales I, Gilliam A, Mancini A, Powell J, Siegel D, Metry D, Frieden IJ. Gastrointestinal bleeding in infantile hemangioma: A complication of segmental, rather than multifocal infantile hemangioma. Journal of Pediatrics 2012: 160(6): pp e3. Fain ET, Mannion M, Pope E, Young DW, Laxer RM, Cron RQ. Brain cavernomas associated with en coup de sabre linear scleroderma: Two case reports. Pediatric Rheumatology Online Journal 2011: 9(1): pp 18. Finkelstein Y, Soon GS, Acuna P, George M, Pope E, Ito S, Shear N, Koren G, Shannon M, Garcia-Bournissen F. Recurrence and outcomes of Stevens-Johnson syndrome and toxic epidermal necrolysis in children. Pediatrics 2011: 128(4): pp Freedman SB, Parkin PC, Willan AR, Schuh S. Rapid versus standard intravenous rehydration in paediatric gastroenteritis: A pragmatic, blinded, randomised clinical trial. British Medical Journal 2011: 343:d6976. Ho NTC, Pope E, Weinstein M, Greenberg S, Webster C, Krafchik B. A double-blind, randomized, placebo-controlled trial of topical tacrolimus 0 1% vs. clobetasol propionate 0 05% in childhood vitiligo. British Journal of Dermatology 2011: 165(3): pp department of paediatrics Annual Report

84 Ipp M, Taddio A. Pain management during vaccination: Recommendations from a Canadian clinical practice guideline. Paediatric and Child Health 2011: 16: pp Jaskolka D, Brown N, Cohen E, Mounstephen W, Connolly B. Evaluating the implementation of a QI initiative: Weekend gastrojejunostomy tube maintenance service in a tertiary pediatric center. Canadian Association of Radiologists Journal 2012: Apr 12 (Epub ahead of print). Kazem S, van der Meijden E, Kooijman S, Rosenberg AS, Hugley LC, Browning JC, Sadler G, Busam K, Pope E, Benoit B, Fleckman P, devries E, Eekhof JA, Feltkamp MC. Trichodysplasia spinulosa is characterized by active polyomavirus infection. Journal of Clinical Virology 2012: 53(3): pp Kuo DZ, Cohen E, Agrawal R, Berry JG, Casey PH. A national profile of caregiver challenges among more medically complex children with special health care needs. Archives of Pediatrics and Adolescent Medicine 2011: 165(11): pp Kuster SP, Shah PS, Coleman BL, Lam P-P, Tong A, Wormsbecker A, McGeer A. Incidence of influenza in healthy adults and healthcare workers: A systematic review and meta-analysis. PLoS ONE 2011: 6(10): e Lara-Corrales I, Parkin PC, Stephens D, Hamilton J, Koren G, Weinstein M, Sibbald G, Pope E. The efficacy of Trimethoprim in wound healing of patients with epidermolysis bullosa: A feasibility trial. Journal of American Academy of Dermatology 2012: 66(2): pp Li S, Taroq K, Pope E, Dedeoglu F, Hong S, Jacobe H, Egla Rabinovich C, Laxer RM, Higgins GC, Lasky A, Baszis K, Becker M, Campillo S, Cartwright V, Cidon M, Inman CJ, Jerath R, O Neil KM, Vora S, Zeft A, Wallace CA, Ilowite NT, Fuhlbrigge RC. Development of consensus treatment plans for juvenile localized scleroderma. Arthritis Care and Research (Hoboken) 2012 Apr 13: (Epub ahead of print) PMID Luca P, Coffey M, Adams S, Ragone A, Cohen E, Matlow A. Using a novel patient medication list for ambulatory pediatric patients within a hospital-based complex care program. Joint Commission Journal on Quality and Patient Safety 2011: 37(12): pp Maguire JL, Birken CS, O Connor DL, Macarthur C, Thorpe K, Mamdani M, Parkin PC for the TARGet Kids! collaboration. Prevalence and predictors of low vitamin D in northern urban Canadian toddlers. Paediatrics and Child Health 2011: 16(2): pp e11-e15. Maguire JL, Kulik DM, Laupacis A, Kuppermann N, Uleryk E, Parkin PC. Clinical prediction rules for children A systematic review. Pediatrics 2011: 128(3): pp e666-e677 (epub). Mahant S, Jovcevska V, Cohen E. Decision-making around gastrostomy feeding in children with neurologic disability: Systematic review of qualitative studies. Pediatrics 2011: 127: pp Mirsky L, Chakkittakandiyil A, Laxer R, O Brien C, Pope E. Relapse after systemic treatment in pediatric morphea. British Journal of Dermatology 2012: 166(2): pp Moore Hepburn C, Duby J, Guttmann A. Children with medical complexity: Forwarding a policy agenda. Current Pediatric Reviews 2012: 8: pp Morinis J, Maguire J, Khovratovich M, McCrindle BW, Parkin PC, Birken CS. Paediatric obesity research in early childhood and the primary care setting: The TARGet Kids! Research Network. International Journal of Environmental Research and Public Health 2012: 9(4): pp O Connor D, Weishuhn K, Rovet J, Mirabella G, Jefferies A, Campbell DM, Asztalos E, Feldman M, Whyte H, Westall C. Visual development of human milk-fed preterm infants provided with extra energy and nutrients after hospital discharge. Journal of Parenteral and Enteral Nutrition 2011 Dec: epub. Ortiz-Alvarez O, Mikrogianakis A, and Canadian Paediatric Society, Acute Care Committee (including Friedman JN). Managing the paediatric patient with an acute asthma exacerbation. Paediatrics and Child Health 2012:17(5): pp Parshuram C, Duncan HP, Joffe AR, Farrell CA, Lacroix JR, Middaugh KL, Hutchison JD, Wensley D, Beyene J, Parkin PC, for the Canadian Critical Care Trials Group. Multicenter validation of the Bedside Early Warning System Score. American Journal of Respiratory and Critical Care Medicine 2011: 15(4): R184 (Epub ahead of print). Pope E, Doria A, Theriault M, Mohanta A, Laxer R. Topical Imiquimod 5% cream for pediatric plaque morphea: A prospective, multiple-baseline, open-label pilot study. Dermatology 2011: 223 (4): pp Pope E, Lara-Corrales I, Mellerio JE, Martinez AE, Schultz G, Burrell R, Goodman L, Coutts P, Wagner J, Allen U, Sibbald RG. A consensus approach to wound care in Epidermolysis Bullosa. Journal of American Academy of Dermatology 2012: Mar (Epub ahead of print). Rana J, Alterkait A, Weinstein M. Picture of the month-scurvy. Archives of Pediatrics and Adolescent Medicine 2012: 165(5): pp Roth DE, Bassani DG. China s progress in neonatal mortality. Lancet (Commentaries/Letters to the Editor) 2011: 378 (9801): pp Russell K, Foisy M, Parkin P, Macpherson A. The promotion of bicycle helmet use in children and youth: An overview of reviews. Evidence-based Child Health: A Cochrane Review Journal 2011: Evidence Based Child Health 6: (2011) DOI: /ebch.901. Science M, Johnstone J, Roth DE, Guyatt G, Loeb M. Zinc for the treatment of the common cold: A systematic review and meta-analysis of randomized controlled trials. Canadian Medical Association Journal 2012: 184(10): pp E551-61; E-pub May 7, Sgro M, Shah P, Campbell D, Tenuta A, Shivananda S, Lee SK. Early onset neonatal sepsis: Rate and organism pattern between 2003 and Journal of Perinatology 2011: 31(12): pp Sheahan L, Da Silva M, Czoli C, Zlotnik Shaul R. A Canadian perspective on a child s consent to research within a context of family centered care: From incompatibility to synergy. Journal of Clinical Research and Bioethics 2012: 3(1). Siegel DH, Tefft KA, Kelly T, Johnson C, Metry D, Burrows P, Pope E, Cordisco M, Holland KE, Moheshwari M, Keith P, Garzon M, Hess C, Frieden IJ, Fullerton HJ, Drolet BA. Stroke in children with posterior fossa brain malformations, hemagiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities (PHACE) syndrome: A systemic review of the literature. Stroke 2012: 43(6): pp Simon TD, Mahant S, Cohen E. Pediatric hospital medicine and children with medical complexity: Past, present, and future. Current Problems in Pediatrics and Adolescent Health Care 2012: 42(5): pp Stade B, Beyene J, Buller K, Ross S, Patterson K, Stevens B, Sgro M, Ungar W, Watson W, Koren G. Feeling different: The experience of living with fetal alcohol spectrum disorder. Journal of Population Therapeutics and Clinical Pharmacology 2011: 18(3): pp e475-e785. Epub 2011 Oct 3. Starmer AJ, Spector ND, Srivastava R, Allen AD, Landrigan CP, Sectish TC; the I-PASS Study Group*. I-PASS, a mnemonic to standardize verbal handoffs (*member of the investigative team cited in the acknowledgements section of the manuscript). Pediatrics 2012: 129(2): pp Taddio A, Hogan ME, Girgis A, Gerges S, Moyer P, Wang L, Murphy C, Ho T, Greenberg S, Ipp M. Addressing parental concern about pain during childhood vaccination: Is there enough time to include pain management in the ambulatory setting? The Clinical Journal of Pain 2012: 28(3): pp Thampi N, Bitnum A, Barner D, Rowe T, Kelvin DJ, Richardson SE, Parkin P, Tran D. Influenza-associated encephalopathy with elevated antibody titers to pandemic (H1N1) 2009 influenza. Journal of Child Neurology 2011: 26(4): pp Thomas R, Landells I, Lynde C, Withers M, Weinstein M, Kunynetz R, Haber R, Balbul A, Dion P-L. Canadian consensus on skin barrier therapy in atopic dermatitis. Journal of Cutaneous Medicine and Surgery 2012: 16(suppl 1): pp S1-16. Urquia M, Hilliard B, Bocking A, Sgro M, Mamdani M, Heale J, Glazier R, Ray J. Birthweight curves tailored to maternal world region. Journal of Obstetrics and Gynaecology Canada 2012: 34(2): pp Wahi G, Parkin PC, Beyene J, Uleryk EM, Birken CS. Effectiveness of interventions aimed at reducing screen time in children: A systematic review and meta-analysis of randomized controlled trials. Archives of Pediatrics and Adolescent Medicine 2011: 165(11): pp the hospital for sick children department of paediatrics Annual Report

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85 Wengle J, Hamilton J, Manlhiot C, Bradley T, Katzman D, Sananes R, Adeli K, Birken CS, Abadilla A, McCrindle B. The golden keys to health: A healthy lifestyle intervention with individual mentorship for overweight and obese adolescents. Paediatrics and Child Health 2011: 16(8): pp Wilgoose P, Cory E, Shouldice M. Case 2: Green vaginal discharge in a 7-year-old girl. (Clinician s Corner) Pediatrics and Child Health 2012:17: pp Wright JG, Zlotnik Shaul R. Risk and innovation and SickKids. Qmentum Quarterly 2012: 4(1): pp Yu A, Stephens D, Feldman B, Parkin P, Kahr WHA, Brandao L, Shouldice M, Levin A. The role of prothrombotic factors in the ocular manifestations of abusive and non-abusive head trauma: A feasibility study. Child Abuse and Neglect 2012:36: pp Books and Chapters Chang K, Pope E, Taylor G. Cutaneous and subcutaneous lymphomas in children. In: Diagnostic Pediatric Hematopathology (Proytcheva M, ed.). Cambridge University Press 2011, New York, New York, USA. pp Cory E, Naymark S. Child maltreatment. In: The Hospital for Sick Children Handbook of Pediatrics, Eleventh Edition (Dipchand A, Friedman J, eds.) (Bismilla A, Gupta S, Lam C, resident eds). Saunders Elsevier, Toronto, Ontario. pp Lara Corrales I, Pope E. Childhood histiocytosis. In: Harpers Textbook of Pediatric Dermatology, Third Edition (Irvine A, Hoger P, Yan A, eds.). Wiley Blackwell Publishing, Oxford, UK. 2011: Vol 1: pp Laxer RM, Pope E, O Brien C. Localized scleroderma in a child. In: Case Studies Systemic Sclerosis. (Silver RM, Denton CP, eds.) Springer Verlag London Ltd, London, UK 2011: pp Pope E. Cutaneous lymphomas. In: Harpers Textbook of Pediatric Dermatology, Third Edition (Irvine A, Hoger P, Yan A, eds.). Wiley Blackwell Publishing, Oxford, UK. 2011: Vol 1: pp Grants and Funding A phase 3 multicenter, randomized, double-blind, placebocontrolled study evaluating the efficacy and safety of ustekinumab in the treatment of adolescent subjects with moderate to severe plaque-type psoriasis. Pope E, Lara-Corrales I. Centocor Ortho Biotech Inc. $15,356 CAD. 2012/01 ongoing A process evaluation of the Integrated Complex Care Coordination Model (ICCM). Cohen E, Kingsnorth S, Kielty K, Bruce-Barrett C, Daub S, Kitch T. Norman Saunders Complex Care Initiative Grant Competition. $20,490 CAD to 2012 A randomised clinical trial to evaluate the impact of a fact sheet about childhood immunization pain management on parental knowledge. Ipp M, Taddio A, Stephens D, Shah V. Paediatric Consultants Education Grant, Hospital for Sick Children. $5,000 CAD to 2013 A randomized, controlled, multidose, multicentre, adaptive Phase II/III Study in infants with proliferating infantile hemangiomas requiring systemic therapy to compare four regimens of Propanolol (1 or 3 mg/kg/day for 3 or 6 months) to placebo (double blind). Pope E, Bradley T, Chakkittakandiyil A, Lara-Corrales I. Pierre Fabre Dermatologie. $373,044 CAD to 2020 Aetiology of neonatal infections in South Asia. Saha S, Roth D, Baqui A, Schrag S, El Arifeen S, Zaidi A, Bhutta A, Kumar V. Bill and Melinda Gates Foundation. $11,589,897 USD to 2013 Bundling effective resident handoff practices to improve patient safety. Landrigan C, Coffey M, Mahant S, Matlow A, et al. National Institutes of Health. $20,000 CAD to 2013 Café Scientifique. Tipping the scales: Weighing the health impacts of obesity in Canadian kids. Hamilton J, Birken CS, Nicholson J, Pleinys R, Dosch HM, Burrows G (panelists). Canadian Institutes of Health Research. $3,000 CAD. Nov 2011 Can we change the face of immunization to one without pain? Implementation of a postnatal parental education intervention to optimize pain management during childhood immunization. Taddio A, Ipp M, Shah V, Franck L, Stephens. Canadian Institutes of Health Research. $100,000 CAD to 2012 Canadian Paediatric Surveillance Program: Severe iron-deficiency anemia in infants and young children. Zlotkin S, Parkin PC, McCourt C, Wong S, Grabowski J, Carcao M, Belletrutti M. In-kind Grant provided by the Canadian Paediatric Surveillance Program, a partnership between the Canadian Paediatric Society and the Public Health Agency of Canada to 2011 CIHR Emerging Team in Childhood Obesity Research. Mc- Crindle B, Parkin PC, Pencharz P, Hamilton J, Anderson G, Birken C, Wells G, Riddell, M, Hanley A, O Connor D, Dettmer E, Bellisimo N, Langer J, Tein I, Feldman M, Ipp M, Jacobson S, Peer M, Taylor C, Berall G, Adeli K, Ling S, Narang I, Geist R, Bradley S, Barwick M, Ernst D. Canadian Institutes of Health Research. $2,452,522 CAD to 2013 CIHR Team in traffic and road injury prevention program for Canadian children. Snowdon AW, Howard AW, Altenhof WJ, Kobti A, Gilliland JA, Ahmed SE, Angell GB, Bruce B, Bussiere D, Georgie VE, Hussine A, Kent RD, Leitch KK, Thrasher C, Worster AS, Birken CS, McClafferty K, Shkrum M. Canadian Institutes of Health Research. $1,744,000 CAD to 2015 Clonality of T cells in peripheral blood of children with cutaneous lymphomas: A cohort pilot study. Pope E, Weitzman S, Zeller J, Chakkittakandiyil A, Alexander S, Abdelhaleem M, Ngan B. Division of Haematology/Oncology Leukemia/ Lymphoma Research Fund. $20,000 CAD to 2015 Clonality of T cells in peripheral blood of children with cutaneous lymphomas: A cohort pilot study. Pope E, Weitzman S, Zeller J, Chakkittakandiyil A, Alexander S, Abdelhaleem M, Ngan B. Conker Ride Cancer Foundation. $20,000 CAD to 2015 Collaborative community-based complex care coordination. Cohen E, Nicholas D, Spalding K, Narayanan U, Friedman J. Norman Saunders Complex Care Initiative Grant Competition. $47,450 CAD to 2011 Dating fractures on radiographs: How accurate is this compared to post-mortem histopathology? Shouldice M, Keppron C, Chiasson D, Stimec J, Lobo-Mueller E, Boutis K, Moharir M. Pediatric Consultants Creative Professional Activities Grant, Department of Pediatrics, the Hospital for Sick Children. $7, CAD Democratizing communication in health care: Development and usability testing of an online communication tool for collaborative care in complex patients. Husain AF, Cohen E, Cafazzo J, Eaton RC, Goldman R, Jamieson T, Rapoport A, Stinson J. Canadian Institutes of Health Research. $200,000 CAD to 2013 Development and validation of toddler NutriSTEP. Randal-Simpson JA, Keller HH, Beyers JE, Birken CS. Canadian Institutes of Health Research, Operating Grant. $200,809 CAD to 2012 Developing and evaluating a support network for families caring for medically complex children. Muskat B, Adam S, Cohen E, Hetherington R, Netten K, Soscia J. Norman Saunders Complex Care Initiative Grant Competition. $24,549 CAD to 2013 Developing tools for localized scleroderma comparative effectiveness studies. Li S, Fuhlbrigge R, Becker ML, Lasky A, Torok C, Ferguson P, Hong S, Pope E, Laxer R, Higgins G, Rabinobich E, Dedeoglu F, Punaro M, Stewart K, Mason TG, Feldman B, Nyirenda T. Arthritis Foundation. $200,000 USD to 2014 DO IT: Vitamin D outcomes and interventions in toddlers. Maguire JL, Birken, CS, Hoch J, Loeb M, Mamdani M, Mazzulli T, Parkin PC, Thorpe KE, Vieth R. Canadian Institutes of Health Research Operating Grant Priority Announcement: INMD Startup Funds (Bridge funding). $100,000 CAD to 2012 DO IT Trial: Vitamin D outcomes and interventions in toddlers. Principal Investigator: Maguire JL, Birken CS, Hoch J, Thorpe K, Loeb M, Mamdani M, Mazzulli T, Parkin PC, Thorpe K, Vieth R. Canadian Institutes of Health Research, Operating Grant. $429,479 CAD to 2014 DO IT! Trial Extension: Vitamin D outcomes and interventions in toddlers: A TARGet Kids! randomized controlled trial. Maguire JL, Birken CS, Hoch J, Loeb M, Mamdani M, Mazzulli T, Parkin PC, Thorpe K, Vieth R. Thrasher Research Foundation. $448,884 CAD to the hospital for sick children department of paediatrics Annual Report

86 Does complicated pneumonia lead to chronic lung disease? Cohen E, Weinstein M, Mahant S, Dell S, Traubici J, Wadha A. Paediatric Consultants Partnership Creative Professional Activities Grant, The Hospital for Sick Children. $9,900 CAD to 2011 Evaluation of the SickKids Team Obesity Management Program (STOMP). Hamilton J, Birken CS. Provincial Council for Maternal and Child Health (PCMCH) $26,729 CAD to 2013 Food insecurity, nutrition and body mass index in early childhood: A TARGet Kids! Study. Fuller A (Resident), Birken CS (Supervisor), Maguire J, Parkin PC, Lebovic G. Academic Pediatric Association Young Investigator Award (YIA) Program. $4,842 CAD to 2013 Gastric flora in gastrostomy fed children with neurologic impairment on acid suppression medication. Rapoport A, Cohen E, Mahant S, Richardson S, Avitzur Y. Pediatric Consultants Partnership Creative Professional Activities Grant, The Hospital for Sick Children. $10,000 CAD to 2012 HOLD IT: Health outcomes of low vitamin D in toddlers: A TARGet Kids! longitudinal cohort study. Maguire JL, Birken, CS, Jiang D, Mamdani M, McCrindle B, O Connor DL, Parkin PC, Vieth R, Feldman M, Ipp M, Jacobson S, Peer M, Taylor C, Chisamore B, Lau E, Do A, Neelands P, Perlmutar M, Saunderson J, Vaughan J. Canadian Institutes of Health Research, Operating Grant. $563,183 CAD to 2014 Improving resident handoff in teaching hospitals: Understanding implementation and effectiveness of a handoff bundle. Coffey M, Starmer A, Mahant S, Matlow A, Bismilla Z, Landigran C. Physician Services Incorporated. $154,820 CAD to 2013 Infant outcomes in the first year of life associated with maternal H1N1 vaccination. Fell D, Moore Hepburn C, Smith G, Walker M, Wen SW, Wilson K. Canadian Institutes of Health Research Operating Grant. $111,642 CAD to 2013 Integration of pulse oximetry into the routine assessment of sick young infants at first-level clinics in Karachi, Pakistan. Roth D, Mir F, Zaidi A. Grand Challenges Canada. $113,000 CAD to 2012 International registry for paediatric cutaneous T-cell lymphoma: A longitudinal, prospective, observational study of cutaneous T-cell lymphoma in the paediatric population. Pope E, Weitzman S, Taylor G. CDF - La Roche-Posay Research Grant $10,000 CAD to 2012; Canadian Dermatology Foundation $5,000 CAD to 2012 Intrapleural DNase and Tissue Plasminogen Activator in Pediatric Empyema (DTPA trial). Cohen E, Connolly B, Hoch J, Laberge S, Maclusky I, Mahant S, Mamdani M, Ratjen F, Thorpe K. Canadian Institutes of Health Research. $315,121 CAD to 2016 Intravenous fluid-related symptomatic acute hyponatremia in Canadian children. Friedman JN, Beck CE, Bohn D, Choong K, Hartfield D, Holland J, Lacroix J, Puligandla P. Hospital for Sick Children Paediatric Consultants CPA Grant. $10,000 CAD to 2013 Intravenous fluid-related symptomatic acute hyponatremia in Canadian children. Beck CE, Bohn D, Choong K, Hartfield D, Holland J, Lacroix J, Puligandla P, Friedman J. Quality and Risk Management, Hospital for Sick Children $10,000 CAD to 2012 Is electrolyte maintenance solution administration required in low-risk children with gastroenteritis? Freedman S, Schuh S, Parkin P, Boutis K, Goia C, Atchison D. Physicians Services Inc. Foundation. $169,200 CAD to 2012 Joined Up Government: Optimizing governance models to serve the needs of children with medical complexity. Moore Hepburn C, Hayeems R. Norman Saunders Complex Care Initiative Grant. $25,397 CAD to 2012 Low milk intake, vitamin D deficiency and fracture risk in young children. Maguire JL, Birken CS, Howard A, Adeli K, Sochett E, Thorpe K, Parkin PC. Dairy Farmers of Canada. $90,000 CAD to 2013 OptEC: Optimizing Early Child Development in the primary care physician practice setting: Pragmatic RCT of iron treatment for young children with non-anemic iron deficiency. Parkin PC, Birken CS, Fehlings D, Macarthur C, Maguire J, Mamak E, Mamdani M, Thorpe K, Zlotkin S. Canadian Institutes of Health Research, Open Operating Grant. $541,567 CAD to 2016 Optimizing the diagnostic and service delivery system for autism spectrum disorder. Moore Hepburn C, Schwartz S, Atkinson A, Shouldice M. Pediatric Consultants Creative Professional Activities Grant, Department of Pediatrics, Hospital for Sick Children. $6,032 CAD to 2013 Patient-level predictors of adverse events in children: What is the role of complexity? Cohen E, Coffey T, Matlow A, Flintoft V, Friedman J, Berry J, Guttmann A. Paediatric Consultants Partnership Creative Professional Activities Grant, Hospital for Sick Children. $8,920 CAD to 2011 Pediatricians as managers: A needs-based assessment of the CanMEDS Physician as Manager role in post-graduate pediatric medicine training programs. Moore Hepburn C, Schwartz S, Atkinson A, Anderson G. Paediatric Consultants Education Innovation and Development Grant. $4,975 CAD to 2013 Pediatric Outcomes Research. Parkin PC, Macarthur C. Hospital for Sick Children Foundation. $750,000 CAD to 2013 Physician leadership and engagement for health systems improvement and accountability. Denis JL, Lawless B, Leblanc D, Moore Hepburn C. Canadian Institutes of Health Research-Expedited Knowledge Synthesis/Evidence Informed Healthcare Renewal Grant. $81,164 CAD Pragmatic randomized controlled trial of continuous versus intermittent oxygen saturation monitoring in infants hospitalized for acute bronchiolitis: An internal pilot study. Mahant S, Wahi G, Thorpe KE, Schuh S, Parkin P. Paediatric Consultants Partnership, Hospital for Sick Children. $10,000 CAD to 2012 Prenatal calcium to prevent preeclampsia and preterm birth in resource-poor rural settings. Roth D, Zlotkin S, Bassani D, Aimone A, Al-Mahmud A, Baqui A, Abrams S. Grand Challenges Canada - Saving Lives at Birth: A Grand Challenge for Development. $270,000 CAD to 2013 PROMOTE: Preschoolers at risk - Obesity and cardiometabolic risk factors: Towards early identification. Birken CS, Adeli K, Hamilton J, Hanley A, Maguire J, McCrindle B, Parkin PC, Feldman M, Ipp M, Jacobson S, Peer M, Taylor C, Chisamore B, Lau E, Do A, Neelands P, Perlmutar M, Saunderson J, Vaughan J. Physicians Services Inc. Foundation. $168,200 CAD to 2012 PROMOTE: Preschoolers at risk Obesity trajectories and cardiometabolic factors: Towards early identification. Birken CS, Adeli K, Hamilton J, Hanley A, Maguire J, McCrindle B, Parkin PC, Feldman M, Ipp M, Jacobson S, Peer M, Taylor C, Chisamore B, Lau E, Do A, Neelands P, Perlmutar M, Saunderson J, Vaughan J. Canadian Institutes of Health Research, Operating Grant. $655,641 CAD to 2016 Severe influenza in infants, children and youth: Role of clinical and epidemiological risk factors. Tran D, Bettinger J, D ery P,Gubbay J, Halperin S, Ipp M, Lesaux N, Loeb M, Mcgeer A, Moore D, Richardson S, Salvadori M, Schuh S, Vaudry W. Canadian Institutes of Health Research. $100,000 CAD to 2012 Short and long term outcomes of complicated pediatric pneumonia. Cohen E, Weinstein M, Dell M, Mahant S, Traubici J, Mekky M. Faculty of Medicine New Faculty Grant, University of Toronto. $10,000 CAD to the hospital for sick children department of paediatrics Annual Report

87 Sleep disturbance in family caregivers of technology dependent children. Keilty K, Stremler R, Cohen E, Pullenayegum E, Spalding K. Ontario Respiratory Care Society (ORCS) of the Ontario Lung Association ($15,000). Canadian Respiratory Health Professionals (CRHP) of the Canadian Lung Association ($8,348) Total $23,348 CAD to 2013 Teaching medical error disclosure skills to postgraduate internal medicine, surgery and pediatrics trainees. Stroud L, Wong B, Coffey M, Atkinson A, Matlow A, Papia G, McDonald-Blumer H Royal College/Associated Medical Services CanMEDS Research and Development Grant (AMS CanMEDS R&D). $24,803 CAD to 2014 The bedside paediatric early warning system: A cluster RCT of mortality and processes of care. Parshuram C, Hutchison J, Parkin PC, Beyene J, Dryden-Palmer K. Canadian Institutes of Health Research, Operating Grant. $3,266,764 CAD to 2014 The effect antenatal vitamin D supplementation on maternalfetal vitamin D status and neonatal immune function: A randomized controlled trial in Bangladesh. Baqui A, Roth D, Raqib R, El Arifeen S. Thrasher Research Fund. $189,314 CAD to 2012 The impact of skin camouflage on the quality of life of children with skin disease and their parents. Salsberg J, Pope E, Weinstein MG, Shear N. Canadian Dermatology Foundation. $5,000 CAD to 2012 The MASC (Medical and Social Complexity) screening tool derivation study phase 1 - Tool construction and pilot-testing. Maguire JL, Birken CS, Cohen E, Adams S, McNeill T, Netten K, Pai N, Parkin PC. Norman Saunders Complex Care Initiative Grant Competition. $42,982 CAD to 2011 The medical home of children and youth in Canada. Guttmann A, Cohen E, Brownell M, Barwick M. Canadian Institutes for Health Research and the Canadian Child and Youth Health Coalition. $104,000 CAD to 2012 The migratory status of the child and limited access to health care: Equity and ethical challenges. Rousseau C, Rummens JW, Glass K, Goldring L, Hamilton H, Hanley J, Jimenez-Sigouin V, Measham T, Montgomery C, Munoz M, Nadeau L, Ouimet M, Ruiz-Casares M, Simich L, Vanthuyne K, Wazana A, Zelkowitz P, Zlotnik Shaul R. Canadian Institutes of Health Research. $167,771 CAD to 2012 The prevalence of poverty among families of children with life-limiting illnesses: An exploratory 5 year review of children receiving care from the Palliative and Bereavement Care Service at SickKids. Beaune L, Ford-Jones L, Chapman LA, Ing S, Rapoport A, Zlotnik Shaul R, Vanderlaan R. The Innovation Fund in Children s Palliative Care Research. $2,700 CAD to 2012 The TARGet Kids! research study. Maguire JL, Birken CS, Parkin PC. St. Michael s Hospital Foundation. $489,650 CAD to 2015 The weight of place: The role of neighborhood deprivation on childhood obesity. Morinis J, Birken CS, Maguire J, Parkin PC, O Campo P, Glazier R, Matheson F, Gozdyra P, Lebovic G. Academic Pediatric Association Young Investigator Award (YIA) Program. $10,000 CAD to 2013 Trainee perceptions of the educational value of clinical cases on an inpatient ward: An exploration of patient- and traineelevel predictors. Cohen E, Mahant S, Wadha A, Bismilla Z. Department of Paediatrics Education Research Grants. $5,000 CAD to 2012 Transition to Adulthood with Cyberguide Evaluation (TRACE) among youth with chronic health conditions: Long-term utilization in adult health care and program transfer to a second pediatric site. Gorter JW, Punthakee Z, Morrison A, Galuppi B, Nagel K, Arora S, Brill H, Don-Wauchope, Grant C, Stewart D, Rosenbaum P, Cohen E, Amaria K. Academic Health Sciences Centre Alternative Funding Plan Innovation Fund. $196,493 CAD to 2013 Vitamin D deficiency in pediatric patients with atopic dermatitis and psoriasis: Prevalence and risk factors. Pope E, Lara-Corrales I. Canadian Dermatology Foundation. $10,000 CAD to 2012 Vitamin D deficiency in pediatric patients with atopic dermatitis and psoriasis: Prevalence and risk factors. Lara-Corrales I, Pope E, Maguire J, Parkin P. Weston Research Award, Society for Pediatric Dermatology. $20,000 USD to 2012 Vitamin D supplementation for preschoolers: A TARGet Kids! randomized controlled trial. Maguire JL, Mamdani M, Birken CS, Parkin PC. Dean s Fund New Staff Competition, Fall 2009, University of Toronto, Faculty of Medicine. $9,976 CAD to 2012 Non-Peer-Reviewed Grants Effectiveness of parent-led interventions in reducing infant hypersensitivity to pain: A longitudinal randomized controlled trial. Taddio A, Ipp M, Pillai-Ridell. Pfizer. $847,457 CAD to the hospital for sick children department of paediatrics Annual Report

88 RESPIRATORY MEDICINE overview Division Head Dr. Felix Ratjen Full-Time Dr. Reshma Amin *(maternity leave Sep 2011-Oct 2012) Dr. Glenda Bendiak Dr. Sharon Dell Dr. Hartmut Grasemann Dr. Theo Moraes Dr. Indra Narang Dr. Melinda Solomon Dr. Padmaja Subbarao Dr. Neil Sweezey Part-Time Dr. Peter Bikangaga Dr. Joanna Massam Dr. Charlotte Miller Dr. Elinor Simons Dr. Connie Yang Clinical Fellows Dr. Ahmad Almosawi Dr. Salman Al-Khalifa Dr. Mansour Al-Qwaiee Dr. Nasser Al-Harbi Dr. Surendran Thavagnanam Dr. Joseph Mathew Dr. Zoe Nugent Subspecialty Residents Dr. Sundeep Bola Dr. Jackie Chiang Administrative Staff Hanadi Attar-Elbard May Brydges *(maternity leave Dec 2011-Jan 2013) Natasha Lalji Jeff Patton Victoria Snell The Division of Respiratory Medicine continues to provide comprehensive care for patients affected by such paediatric pulmonary diseases as asthma, cystic fibrosis (CF), interstitial lung diseases, primary ciliary dyskinesia (PCD), hereditary hemorrhagic telangiectasia (HHT), sleep-related breathing disorders, complex respiratory care requiring ventilatory support, as well as congenital malformations involving the respiratory tract. We provide consultation support for the hospital in all areas of paediatric respiratory disease. The division also provides various diagnostic services including flexible bronchoscopy. In , we broadened our diagnostic spectrum of tests to assess diseases with airway inflammation, including test of nitric oxide in exhaled air, as well as gas-mixing techniques as more sensitive tests for obstructive airway diseases such as multiple breath washout. The infant pulmonary function laboratory offers a unique spectrum of tests that are not available at any other centre in Canada. This has led to the potential for diagnostic tools to objectively assess lung disease in infants and young children. The service is available for the assessment of both patients in the hospital and outpatients. We are currently in the process of developing pulmonary function tests for pre-school children and have started to use incentive spirometry in pre-school children in our CF clinic. We provide consultation support for the hospital in all areas of paediatric respiratory disease. As part of the Toronto Lung Transplantation Program, the Division provides pre- and post-transplantation care for children with end-stage lung disease. Dr. Melinda Solomon continues to be the medical director of the largest paediatric lung transplant program in the country. The program is now open for performing infant lung transplantation as the only centre in the country in select patients. Recent achievements of the program include the first successful ABO incompatible lung transplantation in an infant (Grasemann H, Solomon M, et al, American Journal of Transplant 2012:12:pp ). department of paediatrics Annual Report

89 Dr. Melinda Solomon continues to chair the Transplant Centre Education Committee. The committee is responsible for educating the Transplant Centre faculty, allied health professionals, trainees, paediatric transplant patient and their families. The committee organizes weekly education rounds to the Transplant Centre members, develops education guideline for families and is revising patient handbooks. Under the direction of Dr. Melinda Solomon, our paediatric cystic fibrosis clinic was one again accredited through CF Canada. The paediatric flexible bronchoscopy service continues to grow under Dr. Solomon s leadership, with over 100 procedures performed annually. The training program is led by Dr. Melinda Solomon and has independent Royal College of Physicians and Surgeons of Canada accreditation. It is the largest training program for paediatric respiratory medicine in the country and provides the full range of training that is required for Royal College of Physicians and Surgeons of Canada certification. The infant pulmonary function laboratory offers a unique spectrum of tests that are not available at any other centre in Canada. Dr. Sharon Dell has become Program Director for the Clinical Epidemiology & Health Care Research program within the Faculty of Medicine at the University of Toronto, the first SickKids staff member to take on this role. The program offers both a master s degree and a doctoral-level degree and has an international reputation for excellence in training clinicians in clinical, health services and decision sciences research. Dr. Dell is involved in an international program to develop a new classification for interstitial lung diseases and a NIH-sponsored study on primary ciliary dyskinesia. Dr. Dell, in collaboration with scientists from Health Canada, Environment Canada and Berkeley University, leads a research program examining effects of environmental exposures on asthma outcomes in Toronto school children. She also conducts multi-centre research in primary ciliary dyskinesia in the NIH sponsored Genetic Diseases of Mucociliary Clearance Consortium and in interstitial lung disease with the Children s Interstitial Lung Disease Consortium. Dr. Padmaja Subbarao is Deputy Director of the Canadian Healthy Infant Longitudinal Development (CHILD) Study, the largest birth cohort in Canadian history. The role of the study is to elucidate the factors involved in the development of asthma and allergies in early childhood; it is supported by CIHR and AllerGen. Dr. Subbarao is leading this project as a co-principal investigator with Dr. Malcolm Sears from McMaster University. She has also been successful in attracting additional private funding for the infant pulmonary function cohort in this study that will provide the largest study using lung function measurements in this age group to date. In addition, Dr. Subbarao has continued to work on pioneering new methods of lung function assessment such as multiple breath washout technology:under her leadership SickKids has become a leader in infant pulmonary function testing. She has also successfully received funding for multiple research studies, as well as salary support from the CIHR, the Young Investigator Award. Dr. Theo Moraes, a clinician scientist, has a broad interest in reducing the pathology associated with respiratory viruses and was part of a group of investigators that identified a receptor for RSV, one of the most common reasons for admission to hospital in infancy. His ongoing work relates to exploiting this new knowledge and in addition he is examining ways to alter the immune response to RSV. Dr. Indra Narang continues to direct clinical care and clinical research related to sleep disordered breathing. Currently, Dr. Narang is leading a large clinical trial evaluating cardiovascular and metabolic function in obese children following treatment of obstructive sleep apnea with continuous positive airway pressure. Furthermore, Dr. Narang is collaborating with many colleagues across Canada and the USA in research studies evaluating sleep disorders in children, including the validation of a portable sleep monitor to facilitate the early diagnosis and management of children with sleep disorders. Dr Narang is also interested in the control of breathing in children. She has received external funding to evaluate the role of opioids on the control of breathing and its impact on the development of sleep apnea. Dr. Narang plays a leading role in the education and awareness of sleep disorders in children and she is the course director for the SickKids Paediatric Sleep Symposium which is an internationally recognized event. Dr. Sweezey, in collaboration with Dr. Feige Kaplan at McGill, is studying cellular and molecular aspects of the roles of novel and known glucocorticoid responsive genes in lung development. Dr. Sweezey is funded by the Canadian CF Foundation to study the effects of sex hormones on airway inflammation in CF. Dr. Reshma Amin brings expertise in both clinical research related to CF lung disease as well as clinical expertise in sleeprelated breathing disorders. She focuses her clinical and research activities on technology-dependent children and continues studies to look at new outcome measures in cystic fibrosis. This is highlighted by a recent publication in Radiology on using PET scanning as a marker of airway inflammation in CF and she currently needs a project to use multiple breath washout technology as an outcome measure in early phase trials. The paediatric cystic fibrosis clinic is the largest training program for paediatric respiratory medicine in the country Dr. Glenda Bendiak has recently completed her coursework in the MScCH (Health Practitioner Teacher Education) program at the University of Toronto. Through this, she has addressed several educational needs within the paediatric respirology training program, including the creation of a clinical encounter card for use in the ambulatory clinic rotation, and the development of curriculum maps to reflect the revised Objectives of Training, as published by the Royal College of Physicians and Surgeons of CanadaDr. Bendiak is working with collaborators from SickKids and McMaster University to validate the Health Education Impact Questionnaire in a population of adolescents with chronic illness. In addition, her poster, Association of Airway Nitric Oxide Levels with Phenotypic Characteristics in Children with Sickle Cell Disease, won Top 3 Poster at the 11th International Congress on Pediatric Pulmonology. Dr. Reshma Amin brings expertise in clinical research related to CF lung disease as well as technology- dependent children and sleep-related breathing disorders. Her recent publication (Amin et al. Radiology 2012; 264(3): ) looks at using PET-CT imaging to measure pulmonary inflammation in cystic fibrosis. She currently leads a project to use multiple breath washout as an early outcome measure in a pharmacodynamic intervention study. She is also part of a national study to measure health care utilization costs in adults and children receiving long-term mechanical ventilation. She has secured funding to assess sleep disordered breathing in the chronic kidney disease population. She was also awarded the Pediatric Subspecialty Teaching Award for Excellence in Clinical Teaching during her first year on faculty. Dr. Ratjen s main research interest areas are to conduct multi-centre trials and to develop new outcomes measures to assess CF lung disease.this includes a recent collaborative study with researcher at the Royal Brompton Hospital in London to study CFTR potentiator therapy in CF patients with normal lung function. Dr. Ratjen is leading a emerging team grant funded by CIHR bringing together basic scientists, including stem cell researchers with clinical scientists. This has recently resulted in a seminal work published in Nature Biotechnology demonstrating that skin stem cells can be reprogrammed into airway epithelium and serve as a potential renewable source assay to study CFTR directed therapy. Hartmut Grasemann continues to pursue his major research interest, which is a better understanding of the role of nitric oxide (NO) in lung diseases. Most recent publications from his laboratory show that the endogenous NO synthase inhibitor asymmetric dimethylarginine (ADMA) contributes to NO deficiency and airway obstruction in patients with cystic fibrosis (Grasemann H et al American Journal of Respiratory and Critical Care Medicine 2011: 183: pp ) and plays an important role in airway obstruction in asthma (Scott JA et al., American Journal of Respiratory and Critical Care Medicine 2011: 184: pp ). Other work done under his supervision in the Division of Respiratory Medicine, using comprehensive exhaled NO analyses, revealed abnormalities in airway NO homeostasis in patients with sickle cell disease (Radhakrishnan DK et al Journal of Pediatrics 2012: 160: pp 93-7), and in lung transplant recipients (Bendiak GN et al, Transplantation. 2011; 91:e75-7). 176 the hospital for sick children department of paediatrics Annual Report

90 In addition, members of the Division are key partners of the team that forms the Cystic Fibrosis Centre, highlighting the longstanding and ongoing excellence in both clinical care and research at SickKids in this area. The CF Centre in co-led by Christine Bear, Senior Scientist, Molecular Structure & Function, and Dr. Felix Ratjen. Newborn screening for CF was started last year and the team at SickKids was a major driving force in getting it off the ground. Dr. Ratjen acts as medical advisor to the screening group in Ottawa, and a collaborative study funded by the Canadian CF Foundation has been initiated to evaluate the clinical course of patients with an unclear diagnosis within the Newborn Screening Program. Dr. Ratjen and the CF group are involved in multiple studies in a leading (PI) role. These include multi-centre studies on hypertonic saline in CF funded by the US CF Foundation and National Institutes of Health (NIH) (themain study results were published in JAMA), a study to evaluate the efficacy of CFTR modulation using new outcome measures, and multi-centre studies on pseudomonas eradication strategies and novel approaches of bronchodilator therapy. Other studies supported by research grants, as well as generous support by individuals such as the Irwin family, are ongoing. The CF group is also involved in other clinical studies of drugs targeting the basic defect in CF. A close collaboration exists with the adult CF program at St, Michael s Hospital led by Dr.Tullis in both clinical care and many areas of research, and with the lung transplant team at Toronto General Hospital led by Dr. Keshavjee. The Division continues to be involved in the NIH-funded Childhood Asthma Management Program Continuation Study (Dr. Grasemann, Dr. Solomon, and Dr. Subbarao). There has also been a growing interest in assessing the pulmonary sequelae of sickle cell disease that are being addressed in a number of research studies (Dr. Grasemann, Dr. Narang, and Dr. Subbarao). There is a broad spectrum of both basic science and clinical research that bridges the laboratory to the clinic in many disease areas. The major research foci include clinical and epidemiological studies in patients with asthma and CF, translational studies on airway inflammation and infection in cystic fibrosis, effect of sex hormones in airway inflammation, defining the role of novel genes in lung development, studies in aerosol therapy and exercise physiology. Honors and Awards Solomon, M: Winner of the POWER Teacher Award, Department of Paediatrics, The Hospital for Sick Children, Toronto, September Publications Aaron SD, Vandemheen KL, Freitag A, Pedder L, Cameron W, Lavoie A, Paterson N, Wilcox P, Rabin H, Tullis E, Morrison N, Ratjen F. Treatment of aspergillus fumigatus in patients with cystic fibrosis: A randomized, placebo-controlled pilot study. PLoS One Journal 2012: 7 (4): pp e Al-Saleh S, Dragulescu A, Manson D, Golding F, Traubici J, Mei-Zahav M, Maclusky IB, Faughnan ME, Carpenter S, Ratjen F. Utility of contrast echocardiography for pulmonary arteriovenous malformation screening in pediatric hereditary hemorrhagic telangiectasia. Journal of Pediatrics 2011: 160 (6): pp Al-Saleh S, Riekstins A, Forrest CR, Philips JH, Narang I. Sleep related disordered breathing in children with syndromic craniosynostosis. Journal of Cranio-maxillo-facial Surgery 2011: 39 (3): pp Amin R, Charron M, Grinblat L, Shammas A, Grasemann H, Graniel K, Ciet P, Tiddens H, Ratjen F. Cystic fibrosis: Detecting changes in airway inflammation with FDG PET/CT. Radiology 2012: 264 (3): pp Amin R, Chaves A, Syed F, Moraes TJ. Technology-dependent children: The chronically ventilated child. Current Pediatric Reviews 2012: 8 (2): pp Amin R, Lam M, Dupuis A, Ratjen F. The effect of early pseudomonas aeruginosa treatment on lung function in pediatric cystic fibrosis. Pediatric Pulmonology Journal 2011: 46 (6): pp Amin R, Waters V. Antibiotic treatment for stenotrophomonas maltophilia in people with cystic fibrosis. Cochrane Database of Systematic Reviews 2012: 16 (5): pp CD Asner S, Waters V, Solomon M, Yau Y, Richardson S, Grasemann H, Gharabaghi F, Tran D. Role of respiratory viruses in pulmonary exacerbations in children with cystic fibrosis. Journal of Cystic Fibrosis 2012: 11(5): pp Bendiak GN, Kritzinger F, Dipchand Al, Ng VL, Solomon M, Grasemann H. Flow-independent exhaled nitric oxide parameters in pediatric lung and cardiac transplant recipients. Transplantation 2011: 27 (10):e75-7. Boulet LP, Canadian Thoracic Society Asthma Clinical Assembly. Canadian Thoracic Society 2012 Guideline update: Diagnosis and management of asthma in preschoolers, children and adults. Canadian Respiratory Journal 2012: 19 (2): pp Coates AL, Green M, Leung K, Chan J, Ribeiro N, Ratjen F, Charron M. A comparison of amount and speed of deposition between the PARI LC Star jet nebulizer and an investigational eflow nebulizer. Journal of Aerosol Medicine and Pulmonary Drug Delivery 2011: 24 (3): pp Cohen E, Mahant S, Dell SD, Traubici J, Ragone A, Wadhwa A, Connolly B, Weinstein M. The long-term outcomes of pediatric pleural empyema: A prospective study. Archives of Pediatric and Adolescent Medicine 2012: 3: pp 1-6. Corvol H, Cassiman J, Ratjen F. Ancestral haplotype 8.1 and lung disease severity in European cystic fibrosis patients. Journal of Cystic Fibrosis 2011: 11: pp Davies JC, Sheridan H, Lee PS, Song T, Stone A, Ratjen F. Effect of ivacaftor on lung function in subjects with CF who have the G551D-CFTR mutation and mild lung disease: A comparison of lung clearance index (LCI) vs. spirometry. Journal of Cystic Fibrosis 2012: 11 (1): pp S15. Debley J, Stanojevic S, Filbrun AG, Subbarao P. Bronchodilator responsiveness in wheezy infants and toddlers is not associated with asthma risk factors. Pediatric Pulmonology 2012: 47 (5): pp Dell SD, Cernelc-Kohan M, Hagood JS. Diffuse and interstitial lung disease and childhood rheumatologic disorders. Current Opinion in Rheumatology 2012: 24 (5): pp Duong M, Amin R, Baatje AJ, Kritzinger F, Qi Y, Meghji Z, Lou W, Grasemann H, O Byrne PM, Subbarao P. The effects of montelukast, budesonide alone, and in combination on exercise-induced bronchoconstriction. Journal of Allergy and Clinical Immunology 2012: 130 (2): pp Gonska T, Choi P, Stephenson A, Ellis L, Martin S, Solomon M, Dupuis A, Dorfman R, Zielenski J, Ooi C, Weiser W, Durie P, Tullis E. Role of cystic fibrosis transmembrane regulator (CFTR) in patients with chronic sinopulmonary disease. Chest 2012: 11 (5): pp Grasemann H, de Perrot M, Bendiak G, Cox P, van Arsdell G, Keshavjee S, Solomon M. ABO-incompatible lung transplantation in an infant. American Journal of Transplantation 2012: 12 (3): pp Grasemann H, Ratjen F. Nitric oxide and L-arginine deficiency in cystic fibrosis. Current Pharmaceutical Design 2012: 18 (5): pp Grosse-Wortmann L, Yoo SJ, Solomon M, MacGowan CK, Keshavjee S, Grasemann H. Cardiopulmonary magnetic resonance imaging in children after lung transplantation: Preliminary observations. Journal of Heart and Lung Transplantation 2011: 30 (11): pp Hancox RJ, Subbarao P, Sears MR. Relevance of birth cohorts to assessment of asthma persistence. Current Allergy and Asthma Reports 2012 : 12 (3): pp Knowles, M, Leigh M, Davis S, Dell S, Ferkol T, Olivier K, Sagel S, Rosenfeld M, Burns K, Minnix S, Armstrong M, Lori A, Hazucha M, Loges N, Olbrich H, Becker-Heck A, Schmidts M, Omran H, Zariwala M. Mutations of DNAH11 in primary ciliary dyskinesia patients with normal ciliary ultrastructure. Thorax 2012: 67 (5): pp Konstan MW, Ratjen F. Effect of dornase alfa on inflammation and lung function: Potential role in the early treatment of cystic fibrosis. Journal of Cystic Fibrosis 2012: 11: (2): pp Kritzinger FE, Al-Saleh S, Narang I. Descriptive analysis of central sleep apnea in childhood at a single centre. Paediatric Pulmonology 2011: 46 (10): pp the hospital for sick children department of paediatrics Annual Report

91 Loeve M, Hop WC, de Bruijne M, Van Hal PT, Robinson P, Aitken ML, Dodd JD, Tiddens HA, on behalf of the Computed Tomography Cystic Fibrosis Survival Study Group. Chest computed tomography scores are predictive of survival in CF patients awaiting lung transplantation. American Journal of Respiratory and Critical Care Medicine 2012: 185 (10): pp Lougheed MD, Lemiere C, Ducharme FM, Licskai C, Dell SD, Rowe BH, FitzGerald JM, Leigh R, Watson W, MacLean JE, Solomon M, Corey M, Selvadurai H. Cystic fibrosis newborn screening does not delay the identification of cystic fibrosis in children with negative results. Journal of Cystic Fibrosis 2011: 10 (5): pp McIlwaine M, Agnew JL, Alraie N, Lands L, Ratjen F, Milner R, Davidson AGF. Canadian National Airway Clearance Study: Positive expiratory pressure mask versus high frequency chest wall oscillation. Journal of Cystic Fibrosis 2012: 11 (1): pp S23. Moraes TJ, Lin GH, Wen T, Watts T. Incorporation of 4-1BB ligand into an adenovirus vaccine vector increases the number of functional antigen-specific CD8 T cells and enhances the duration of protection against influenza induced respiratory disease. Vaccine 2011: 29 (37): pp Morris SK, Subbarao P, Science M, Parkin P, Yau Y, Allen UD, Tran D. A retrospective cross-sectional study of risk factors and clinical spectrum of children admitted to hospital with pandemic H1N1 influenza as compared to influenza A. British Medical Journal Open 2012: 11 (2): pp 2. Naito Y, Beres A, Lapidus-Krol E, Ratjen F, Langer JC. Does earlier lobectomy result in better long-term pulmonary function in children with congenital lung anomalies? A prospective study. Journal of Pediatric Surgery 2012: 47 (5): pp Narang I, Bush A. Early origins of chronic obstructive pulmonary disease. Seminars in Fetal Neonatal Medicine 2012: 17(2): pp Narang I. Sleep fragments: Intermittent oxygen desaturations: When do they matter? American Thoracic Society 2012: Available online only: sleep/sleep-fragment/pages/2012-january.php Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, Drevinek P, Griese M, McKone EF, Wainwright CE, Konstan MW, Moss R, Ratjen F, Sermet-Gaudelus I, Rowe SM, Dong Q, Rodriguez S, Yen K, Ordonez C, Elborn JS, VX Study Group. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. New England Journal of Medicine 2011: 365: pp Ratjen F, Durham T, Navratil T, Schaberg A, Accurso FJ, Wainwright C, Barnes M, Moss RB, the TIGER-2 Study Investigator Group. Long term effects of denufosol tetrasodium in patients with cystic fibrosis. Journal of Cystic Fibrosis 2012: Jun 8 (Epub ahead of print). Ratjen F, Grasemann H. New therapies in cystic fibrosis. Current Pharmaceutical Design 2012: 18 (5): pp Ratjen F, McColley SA. Update in cystic fibrosis American Journal of Respiratory and Critical Care Medicine 2012: 185 (9): pp Ratjen F, Rosenfeld M, Brumback L, Daniel S, Rowbotham R, Kronmal R, Davis S. Efficacy of hypertonic saline in infants and young children: The ISIS study. Journal of Cystic Fibrosis 2012: 11 (1): pp S50. Ratjen F, Saiman L, Mayer-Hamblett N, Lands LC, Kloster M, Thompson V, Emmett P, Marshall B, Accurso F, Sagel S, Anstead M. Effect of azithromycin on systemic markers of inflammation in cystic fibrosis patients uninfected with pseudomonas aeruginosa. Chest 2012: May 17 (Epub ahead of print). Rosenfeld M, Ratjen F, Brumback L, Daniel S, Rowbotham R, McNamara S, Johnson R, Kronmal R, Davis SD, ISIS Study Group. Inhaled hypertonic saline in infants and children younger than 6 years with cystic fibrosis: The ISIS randomized controlled trial. Journal of the American Medical Association 2012: 307 (21): pp Rossant J, Wong A, Bear C, Chin S, Pasceri P, Thompson T, Ling-Jun H, Ratjen F, Ellis J. Directed differentiation of human pluripotent stem cells into mature airway epithelia expressing functional CFTR protein. Journal of Nature Biotechnology 2012: May 17. (Epub ahead of print). Saiman L, Mayer-Hamblett N, Anstead M, Lands L, Kloster M, Goss C, Rose L, Burns J, Marshall B, Ratjen F. Open label, follow-up study of azithromycin in pediatric patients with CF uninfected with pseudomonas aeruginosa. Pediatric Pulmonology 2012: 47 (7): pp Sweezey N. 50 years ago in the Journal of Pediatrics: Observations on skin resistance to electricity and sweat chloride content: A preliminary report. Journal of Pediatrics 2012: 160 (5): pp 750. Tayyari F, Marchant D, Moraes TJ, Duan W, Mastrangelo P, Hegele RG. Identification of a nucleolin as a cellular receptor for human respiratory syncytial virus. Nature Medicine 2011: 17 (9): pp Ungar WJ, Boydell K, Dell S, Feldman BM, Marshall D, Willan A, Wright J. A parent-child dyad approach to the assessment of health status and health-related quality of life in children with asthma. Pharmaco Economics Journal 2012: 30 (8): pp Warren TA, Yau Y, Ratjen F, Tullis E, Waters V. Serum galactomannan in cystic fibrosis patients colonized with aspergillus species. Medical Mycology Journal 2012: 50 (6): pp Waters V, Atenafu EG, Salazar JG, Lu A, Yau Y, Matukas L, Tullis E, Ratjen F. Chronic stenotrophomonas maltophillia infection and exacerbation outcomes in cystic fibrosis. Journal of Cystic Fibrosis 2011: 11: pp Waters V, Stanojevic S, Atenafu EG, Lu A, Yau Y, Tullis E, Ratjen F. Effect of pulmonary exacerbations on long-term lung function decline in cystic fibrosis. European Respiratory Journal 2012: 40: pp Wu AC, Tantisira K, Li L, Schuemann B, Weiss ST, Fuhlbrigge AL, Childhood Asthma Management Program Research Group. Predictors of symptoms are different from predictors of severe exacerbations from asthma in children. Chest 2011: 140 (1): pp Yang CL, To T, Foty R, Stieb D, Dell S. Validating a questionnaire diagnosis of asthma in children using health claims data. BioMed Central Pulmonary Medicine 2011: 11(1): pp 52. Books and Chapters Amin R, Ratjen F. Treatment of airway mucociliary clearance and airway hydration. In: Cystic Fibrosis Disease. (Gartner S, Salcedo A, Girón G, García Novo M, eds.). S&L, Madrid, Spain 2012: pp Chernick V, Bush A, Boat T, Deterding R, Ratjen F, Willmott R. In: Kendigs and Chernick s Disorders of the Respiratory Tract in Children, Eighth Edition. (Wilmot R, et al, eds.). Elseveir Saunders, Philadelphia, PA Dell SD, Schneider R. Pulmonary involvement in the systemic inflammatory diseases of childhood. In: Kendigs and Chernick s Disorders of the Respiratory Tract in Children, Eighth Edition. (Wilmot R, et al, eds.). Elseveir Saunders, Philadelphia, PA Grasemann H, Ratjen F, Dockter G. Pneumologie. In: Duale Reihe Pädiatrie. (Gortner L, Meyer S, Sitzmann FC, eds.). Frankfurt, Germany 2012: pp Grants and Funding A multi-stage population based study to determine the association between air pollution and asthma. Dell S. Health Canada. $76,442. Sep to Mar A phase 2, randomized, double-blind placebo controlled cross over study to evaluate LCI in subject with cystic fibrosis, the G551D mutation & FEV > 90% predicted (Vertex 106). Ratjen F. Vertex Pharmaceuticals. $70, CAD. Jan to Jan A randomized, double-blind, placebo-controlled parallel-group trial to confirm the efficacy after 12 weeks and the safety of tiotropim 5 μg administered once daily via the Respimat d evice in patients with cystic fibrosis. Ratjen F. Boehringer Ingelheim. $45,023 CAD. Feb to Feb Canadian birth cohort; Indoor air and the development of asthma and allergy (CHILD Study). Subbarao PJ. AllerGen NCE Inc. $357,400 CAD. Oct to Mar the hospital for sick children department of paediatrics Annual Report

92 Canadian birth cohort; Indoor air and the development of asthma and allergy (CHILD) study. Subbarao PJ, Dell S, Sears M. Canadian Institutes of Health Research. $12,000,000 CAD. Dec to Mar Canadian birth cohort; Indoor air and the development of asthma and allergy (CHILD) study, Toronto Site. Subbarao PJ. SickKids Foundation: Private donations from Bothwell Accurate and Golden Thread Charitable Foundation. $1,100,000 CAD. Aug to Oct Childhood Asthma Management Program (CAMP) Continuation study. CAMP-CS3: The Hospital for Sick Children Clinical Centre for Study of a Childhood Asthma Management Program Continuation Study. Grasemann H, Subbarao PJ, Solomon M. National Institutes of Health, National Heart, Lung and Blood Institute. John Hopkins University Centre for Clinical Trials. $370,633 CAD. Aug to Sep Collaboration between the CHILD Study and the START Study. Subbarao PJ, AllerGen NCE Inc. $50,000 CAD. Jun to Jun Correlation of Air Quality Health Index (AQHI) and chronic disease morbidities. To T, Licskai C, Dell S, Stanojevic S, Foty R. Ontario Ministry of Environment. $50,000 CAD. Apr to Mar Cystic Fibrosis Foundation Therapeutics Inc. Inhaled Saline for Infants Study (ISIS-002). Ratjen F, Subbarao P, Solomon M. National Institutes of Health Research. $1,794,674 US. Apr to Dec Emerging team grant: From genes to proteins, cells, tissues and patients. Ratjen F, Grasemann H, Amin R. Canadian Institutes of Health Research. $2,401,449 CAD. Dec to Nov Enhancing CD8 T cell immune response to RSV. Moraes T. Start up equipment fund, The Hospital for Sick Children Research Institute. $24,000 CAD. Aug to Jul Enhancing CD8 T cell immune responses to RSV. Moraes T. Dean s Fund New Staff Competition, University of Toronto. $10,000 CAD. Mar to Mar Enhancing CD8 T cell responses to RSV (Respiratory Syncytial Virus). Moraes T. Canadian Institutes of Health Research/The Hospital for Sick Children Research Institute. $300,000 CAD. Apr to Mar Ensuring effective newborn screening: The case of cystic fibrosis. Miller F, Ratjen F. Canadian Institute of Health Research. $41,292 CAD. (Total Grant Amount Awarded $636,506 CAD). Sep to Aug Functional analyses of two novel, developmentally-regulated glucocorticoid-responsive genes in fetal lung. Kaplan F, Sweezey N. Canadian Institutes of Health Research. $155,297 CAD per annum. Apr to Mar Genetic diseases of mucociliary clearance consortium. Knowles M, Dell S. University of North Carolina/National Institutes of Health. $6,709,685 CAD. Aug to Jul Glucocorticoid receptor and glucocorticoid-responsive genes in developing lung. Sweezey N, Kaplan F. Ontario Thoracic Society. $48,972 CAD per annum. Apr to Mar High impact strategies towards overweight reduction in youth (HISTORY) team grant. Narang I, McCrindle B, Hamilton J. Canadian Institutes of Health Research. $2,322,370 CAD. Apr to Mar Lab technician, salary support. The Lynne and Arnold Irwin Foundation. $56,842 CAD. Jan to Dec Modulation by sex hormones of inflammation and susceptibility to pseudomonas aeruginosa in cystic fibrosis airways. Sweezey N. Cystic Fibrosis Canada. $90,000 CAD per annum. Apr to Mar Modulation of pulmonary L-arginine metabolism by endogenous NOS inhibitors in cystic fibrosis. Grasemann H. Cystic Fibrosis Canada. $170,000 CAD. Apr to Mar New investigator award in area of clinical research. Subbarao PJ. Canadian Institutes of Health Research. $300,000 CAD. Apr to Mar Opioid-induced respiratory depression in children. Narang I. Ontario Thoracic Society. $47,961 CAD. Apr to Mar Pathogenesis of serious infections with influenza: Role and mechanisms of microvascular leak. Moraes T. Physicians Services Incorporated Foundation. $170,000 CAD. Jun to Jun Pediatric Consultants Grant. Amin R. SickKids Hospital. $9 520 CAD. May to May Platform for Outcomes Research and Translation in Asthma and allergy (PORTAL). Lynd L, Ungar W, Marra CA, Dell S, Fitzgerald JM, Clarke A, Elliott S. Allergen. $300,000. Jul 2009 to Jun 2014 Randomized, double-blind, placebo-controlled, cross-over multi-center study to assess the efficacy and safety of inhaled Tobramycin nebuliser solution (TOBI ) for the treatment of early infections of P. aeruginosa in cystic fibrosis subjects aged from 3 months to less than 7 years. Ratjen F. Novartis. $6750 CAD per patient. Apr to Mar Respiratory syncytial virus in the development of infant wheezing disorders: A bench to bedside approach to gene-virus interactions. Subbarao PJ. AllerGen NCE Inc. $176,800 CAD. Jul to Mar Respiratory syncytial virus in the development of infant wheezing disorders: A bench to bedside approach to gene virus interactions. Moraes T. AllerGen NCE Inc. $176,800 CAD. July to Mar Serum biomarkers as predictors of bronchiolitis obliterans in children receiving allogeneic hematopoietic stem cell transplantation. Gassas A, Palaniyar N, Doyle J, Schecter- Finkelstein T, Sung L, Dell S. POGO. $14,995 CAD. Jun to May Start up equipment and research support. Amin R. The Hospital for Sick Children/ Department of Pediatrics. $25,000 CAD. Jan to Jan Start up equipment and research support. Amin R. SickKids Hospital Research Institute. $77,000 CAD. Jan to Jan Start-up equipment / start-up operating. Moraes T. SickKids Foundation / The Hospital for Sick Children Research Institute. $432,000 CAD. Aug to Jul The characteristics and determinants of quality of life in invasively and non-invasively ventilated pediatric patients: New investigator grant. Amin R. Health Utilities Inc. $8,000 CAD. Dec to Nov Transplant Centre grant. Grasemann H, Solomon M. University of Health Network. $36,000 CAD. Sep to Aug Validation and use of evidence-based asthma care performance indicators in Ontario (VALUE-API). To T, Dell S, Gershon A, Lougheed D, Tamari I. Ontario Thoracic Society. $49,000 CAD. Aug to Jul the hospital for sick children department of paediatrics Annual Report

93 RHEUMATOLOGY overview Division Head Dr. Brian Feldman Full-Time Dr. Susanne Benseler Dr. Ronald Laxer Dr. Deborah Levy Dr. Shirley Tse Dr. Rae Yeung Part-Time Dr. Bonnie Cameron Dr. Rayfel Schneider Dr. Earl Silverman Dr. Lynn Spiegel Cross-Appointees Dr. Nicholas Blanchette Research Fellows and Associates Dr. Daniela Ardelean, Research Fellow Dr. Julie Barsalou, Research Fellow Dr. Michelle Batthish, Research Fellow Dr. Lily Lim, Research Fellow Dr. Nadia Luca, Research Fellow Dr. Mira van Veenendaal, Research Fellow Alicia Hilderley, Clinical Research Project Coordinator Alison Tian, Clinical Research Project Assistant Audrey Abad, Clinical Research Project Manager Haddas Grosbein, Clinical Research Project Coordinator Dan Ignas, Clinical Research Project Assistant Shazia Ali, Database Manager Lawrence Ng, Clinical Research Project Coordinator Marjorie McLimont, Clinical Research Project Manager Reena Kainth, Clinical Research Project Coordinator Shehla Sheikh, Research Coordinator Trang Duong, Research Associate Clinical Fellows Dr. Abdulrahman Al-Rasheed Dr. Marinka Twilt Dr. Senq Lee Dr. Peter Gowdie Subspecialty Residents Dr. Alisa Rachlis Dr. Gordon Soon Dr. Michelle Batthish Administrative Staff Michelle Blimkie Lynn Brown Olga Bogas Denise Ferreira Tania Viseu The Division of Rheumatology provides comprehensive care for children with rheumatic diseases. The ambulatory clinics have approximately 5,500 patient visits a year at The Hospital for Sick Children. In addition to the general rheumatology clinics, there are unique multidisciplinary subspecialty clinics for children with autoinflammatory diseases, dermatomyositis, inflammatory brain disease, morphea, systemic JIA, systematic and neonatal lupus erythematosus, spondylitis, and vasculitis. Many children have benefited from treatments in the medical day care unit, where children receive joint injections, therapeutic infusions or undergo investigations that would normally require in-patient admission. Rheumatology provides clinical care and undertakes research studies in systemic inflammatory disorders and inflammatory diseases of the bones and joints. Our program offers a comprehensive range of services for the evaluation and treatment of children with the vast range of paediatric rheumatic and inflammatory diseases. Rheumatology provides clinical care and undertakes research studies in systemic inflammatory disorders and inflammatory diseases of the bones and joints. The Division of Rheumatology has one of the largest paediatric rheumatology fellowship training programs in North America and is fully accredited by the Royal College of Physicians and Surgeons of Canada. This academic year, our fellows came from Canada, the Netherlands, Australia and Saudi Arabia. Our graduates have been successful in obtaining academic placements around the world and trainees have been productive in the research arena. Research foci include the development of a trial design for the study of rare diseases, outcome, prognosis and treatment studies in juvenile idiopathic arthritis, systemic lupus erythematosus, neonatal lupus, scleroderma, dermatomyositis, vasculitis and CNS vasculitis. Basic research focuses on the immunopathogenesis of Kawasaki disease using an animal model. department of paediatrics Annual Report

94 Honors and Awards Tse, S: Winner of POWER Teaching Award, University of Toronto, The Department of Paediatrics, Toronto, Publications Alvira CM, Guignabert C, Kim YM, Wang L, Duong T, Yeung RSM, Li DY, Mecham RP, Rabinovitch M. Inhibition of transforming growth factor-beta worsens elastin degradation in a murine model of Kawasaki disease. American Journal of Pathology 2011: 178 (3): pp Banks L, Lyn YT, Chahal N, Manlhiot C, Yeung RSM, Mc- Crindle BW. Factors associated with low moderate-to-vigorous physical activity levels in pediatric patients with Kawasaki disease. Clinical Pediatrics 2012: 51(9): pp Bernard TJ, Manco-Johnson MJ, Lo W, Mackay MT, Ganesan V, Deveber G, Goldenberg NA, Armstrong-Wells J, Dowling MM, Roach ES, Tripputi M, Fullerton HJ, Furie KL, Benseler SM, Jordan LC, Kirton A, Ichord R. Towards a consensus-based classification of childhood arterial ischemic stroke. Stroke 2012: 43(2): pp Blankier S, McCrindle BM, Ito S, Yeung RSM. The role of statins in regulating the immune response in a model of Kawasaki Disease. Clinical and Experimental Immunology 2011: 164 (2): pp Brunner H, Mina R, Pilkington C, Beresford M, Reiff A, Levy DM, Tucker L, Eberhard A, Ravelli A, Schanberg L, Saad-Magalhaes C, Higgins G, Onel K, Singer N, von Scheven E, Itert L, Klein-Gitelman M, Punaro M, Ying Y, Giannini E. Preliminary criteria for global flares in childhood-onset systemic lupus erythematosus. Arthritis Care Research 2011: 63 (9): pp Burnett HF, Regier DA, Feldman BM, Miller FA, Ungar WJ. Parents preferences for drug treatments in juvenile idiopathic arthritis: A discrete choice experiment. Arthritis Care Research (Hoboken) 2012: 64(9): pp Butbul Aviel Y, Stremler R, Benseler SM, Cameron B, Laxer RM, Ota S, Schneider R, Spiegel L, Stinson JN, Tse SM, Feldman BM. Sleep and fatigue and the relationship to pain, disease activity and quality of life in juvenile idiopathic arthritis and juvenile dermatomyositis. Rheumatology (Oxford) 2011: 50(11): pp Cellucci T, Benseler SM. Posterior reversible encephalopathy syndrome: Increasing recognition of an important clinical entity in young patients with systemic lupus erythematosus. Journal Rheumatology 2011: 38(8): pp Cellucci T, Shroff M, Tse SML. Grisel s syndrome: A pain in the neck for rheumatologists. Paediatrics and Child Health 2012: 17(5): pp Cellucci T, Tyrrell PN, Pullenayegum E, Benseler SM. von Willebrand factor antigen-- A possible biomarker of disease activity in childhood central nervous system vasculitis? Rheumatology (Oxford). 2012: June (Epub ahead of print). Cellucci T, Tyrrell PN, Sheikh S, Benseler SM. Childhood primary angiitis of the central nervous system: Identifying disease trajectories and early risk factors for persistently higher disease activity. Arthritis and Rheumatology 2012: 64(5): pp Chahal N, Somji Z, Manlhiot C, Clarizia N, Ashley J, Yeung RSM, McCrindle B. Rate, associated factors and outcomes of recurrence of Kawasaki disease in Ontario, Canada. Pediatrics International 2012: 54(3): pp Coffey M, Laxer RM. Quality and safety in pediatric complex care. Current Pediatric Reviews 2012: 8(2): pp Diamond IR, de Silva NT, Tomlinson GA, Pencharz PB, Feldman BM, Moore AM, Ling SC, Wales PW. The role of parenteral lipids in the development of advanced intestinal failure-associated liver disease in infants: A multiple-variable analysis. Journal Parenteral Enteral Nutrition 2011: 35(5): pp Diamond IR, Pencharz P, Feldman B, Ling S, Moore A, Wales P. Novel lipid based approaches to pediatric intestinal failure associated liver disease. Archives of Pediatrics and Adolescent Medicine 2012: 166 (5): pp Fain E, Mannion M, Pope E, Young D, Laxer RM, Cron R. Brain cavernomas associated with en coup de sabre linear scleroderma: Two case reports. Pediatric Rheumatology Online 2011: 9: pp 18. Feldman BM, Berger K, Bohn R, Carcao M, Fischer K, Gringeri A, Hoots K, Mantovani L, Willan AR, Schramm W. Haemophilia prophylaxis: How we can justify the costs? Hemophilia 2012: 18(5): pp Gowdie PJ, Tse SM. Juvenile idiopathic arthritis. Pediatric Clinics of North America 2012: 39(2): pp Groen W, van der Net J, Bos K, Abad A, Bergstrom BM, Blanchette VS, Feldman BM, Funk S, Helders P, Hilliard P, Manco-Johnson M, Petrini P, Zourikian N, Fischer K. Joint health and functional ability in children with haemophilia who receive intensive replacement therapy. Haemophilia 2011: 17(5): pp Huber AM, Robinson AB, Reed AM, Abramson L, Bout-Tabaku S, Carrasco R, Curran M, Feldman BM, Gewanter H, Griffin T, Haines K, Hoeltzel MF, Isgro J, Kahn P, Lang B, Lawler P, Shaham B, Schmeling H, Scuccimarri R, Shishov M, Stringer E, Wohrley J, Ilowite NT, Wallace C, Juvenile Dermatomyositis Subcommittee of the Childhood Arthritis and Rheumatology Research Alliance. Consensus treatments for moderate juvenile dermatomyositis: Beyond the first two months. Results of the second Childhood Arthritis and Rheumatology Research Alliance Consensus Conference. Arthritis Care Research 2012: 64(4): pp Hui-Yuen J, Imundo L, Avitabile C, Kahn P, Eichenfield A, Levy DM. Early versus later onset childhood-onset systemic lupus erythematosus: Clinical features, treatment and outcome. Lupus 2011: 20(9): pp Jacob CO, Eisenstein M, Dinauer MC, Ming W, Liu Q, John S, Quismorio FP Jr, Reiff A, Myones BL, Kaufman KM, McCurdy D, Harley JB, Silverman E, Kimberly RP, Vyse TJ, Gaffney PM, Moser KL, Klein-Gitelman M, Wagner-Weiner L, Langefeld CD, Armstrong DL, Zidovetzki R. Lupus-associated causal mutation in neutrophil cytosolic factor 2 (NCF2) brings unique insights to the structure and function of NADPH oxidase. Proceedings of the National Academy of Sciences of the United States of America (PNAS) 2012: 109(2): pp E Johnson SR, Granton JT, Tomlinson GA, Grosbein HA, Le T, Lee P, Seary ME, Hawker GA, Feldman BM. Warfarin in systemic sclerosis-associated and idiopathic pulmonary arterial hypertension. A bayesian approach to evaluating treatment for uncommon disease. Journal of Rheumatology 2012: 39(2): pp Kahn PJ, Yazici Y, Argilla M, Srichai M, Levy DM. Asymptomatic giant coronary aneurysm in an adolescent with Behcet s syndrome. Pediatric Rheumatology Online Journal 2012: 10: pp 2. Katchamart W, Faulkner A, Feldman BM, Tomlinson G, Bombardier C. PubMed had a higher sensitivity than Ovid-MEDLINE in the search for systematic reviews. Journal of Clinical Epidemiology 2011: 64(7): pp Khor CC, Davila S, Breunis WB, Lee YC, Shimizu C, Wright V, Yeung RSM, Tan DE, Sim KS, Wang JJ, Wong TY, Pang J, Mitchell P, Cimaz R, Dahdah N, Cheung YF, Huang GY, Yang W, Park IS, Lee JK, Wu JY, Levin M, Burns JC, Burgner D, Kuijpers TW, Hibberd ML on behalf of the Hong Kong- Shanghai, Korean, Taiwan, U.S. and International Kawasaki Disease Genetics Consortiums. Genome-wide association study identifies FCGR2A as a susceptibility locus for Kawasaki disease. Nature Genetics 2011: 43(12): pp Koitschev A, Gramlich K, Hansmann S, Benseler S, Plontke SK, Koitschev C, Koetter I, Kuemmerle-Deschner JB. Progressive familial hearing loss in Muckle-Wells syndrome. Acta Otolaryngology 2012: 132(7): pp Kuemmerle-Deschner JB, Lohse P, Koetter I, Dannecker GE, Reess F, Ummenhofer K, Koch S, Tzaribachev N, Bialkowski A, Benseler SM. NLRP3 E311K mutation in a large family with Muckle-Wells syndrome-description of a heterogeneous phenotype and response to treatment. Arthritis Research Therapy 2011: 13(6): pp 196. Lam CG, Manlhiot C, Pullenayegum EM, Tyrrell PN, Feldman BM. Efficacy of intravenous immunoglobulin therapy in juvenile dermatomyositis. Annals of Rheumatic Diseases 2011: 70(12): pp the hospital for sick children department of paediatrics Annual Report

95 Laxer RM, Sherry DD. Pediatric rheumatology. Pediatric Clinics of North America 2012: 59 (2): pp xiii-xiv. Lee SJ, Silverman ED, Bargman JM. The role of antimalarials agents in the treatment of in SLE and lupus nephritis. Nature Reviews Nephrology 2011: 7(12): pp Levy DM, Kamphuis S. Systemic lupus erythematosus in children and adolescents. Pediatric Clinics of North America 2012: 59(2): pp Levy LM, Peschken CA, Tucker LB, Chédeville G, Huber AM, Pope JE, Silverman ED and the Canadian Network for Improved Outcomes in SLE (CaNIOS) Investigators. The 1000 Canadian faces in lupus: Influence of ethnicity on disease in the pediatric cohort. Arthritis Care Research 2012 June (Epub ahead of print). Lim SH, Benseler SM, Tyrrell PN, Charron M, Harvey E, Hebert D, Silverman ED. Low bone mineral density is present in newly diagnosed paediatric systemic lupus erythematosus patients. Annals of Rheumatic Disease 2011: 70(11): pp Lim LS, Benseler SM, Tyrrell PN, Harvey E, Herbert D, Charron M, Silverman ED. Predicting longitudinal trajectory of bone mineral density in paediatric systemic lupus erythematosus patients. Annals of Rheumatic Disease 2012: March (Epub ahead of print). Luca N, Daengsuwan T, Dalmau J, Jones K, deveber G, Kobayashi J, Laxer RM, Benseler SM. Anti-N-methyl-Daspartate receptor encephalitis: A newly recognized inflammatory brain disease in children. Arthritis Rheumatology 2011: 63(8): pp Luca NJC, Soon GS, Tse SML. Clinician s corner: Acute chorea. Paediatrics and Child Health 2011: 16(10): pp Luca N, Yeung RSM. Epidemiology and management of Kawasaki disease. Pediatric Drugs 2012: 72(8): pp Manlhiot C, Christie E, McCrindle BW, Rosenberg H, Chahal N, Yeung RSM. Complete and incomplete Kawasaki disease: Two sides of the same coin. European Journal of Pediatrics 2012: 171(4): pp Millar K, Manlhiot C, Yeung RSM, Somji Z, McCrindle BW. Corticosteroid administration for patients with coronary artery aneurysms after Kawasaki disease may be associated with impaired regression. International Journal of Cardiology 2012: 154(1): pp Miles BS, Anderson P, Agostino A, Golomb MR, Achonu C, Blanchette V, Feldman BM, McLimont M, Revel-Vilk S, Stain A, Barnes MA. Effect of intracranial bleeds on the neurocognitive, academic, behavioral, and adaptive functioning of boys with hemophilia. Haemophilia 2012: 18(2): pp Mina R, von Scheven E, Ardoin SP, Eberhard BA, Punaro M, Ilowite N, Hsu J, Klein-Gitelman M, Moorthy LN, Muscal E, Radhakrishna SM, Wagner-Weiner L, Adams M, Blier P, Buckley L, Chalom E, Chédeville G, Eichenfield A, Fish N, Henrickson M, Hersh AO, Hollister R, Jones O, Jung L, Levy D, Lopez- Benitez J, McCurdy D, Miettunen PM, Quintero-Del Rio AI, Rothman D, Rullo O, Ruth N, Schanberg LE, Silverman E, Singer NG, Soep J, Syed R, Vogler LB, Yalcindag A, Ldirim- Toruner C, Wallace CA, Brunner HI. Consensus treatment plans for induction therapy of newly-diagnosed proliferative lupus nephritis in juvenile systemic lupus erythematosus. Arthritis Care and Research 2012: 64(3): pp Mirsky L, Chakkittakandiyil A, Laxer RM, O Brien C, Pope E. Relapse after systemic treatment in pediatric morphea. British Journal of Dermatology 2012: 166(2): pp Moran CJ, Walters TD, Guo CH, Kugathasan S, Klein C, Turner D, Wolters VM, Bandsma RH, Mouzaki M, Zachos M, NEOPICS, Langer JC, Cutz E, Benseler SM, Roifman CM, Silverberg MS, Griffiths AM, Snapper SB, Muise AM. IL-10R polymorphisms are associated with very-early-onset ulcerative colitis. Inflammatory Bowel Disease 2012: May (Epub ahead of print). Morishita K, Li SC, Muscal E, Spalding S, Guzman J, Uribe A, Abramson L, Baszis K, Benseler S, Bowyer S, Campillo S, Chira P, Hersh AO, Higgins G, Eberhard A, Ede K, Imundo L, Jung L, Kim S, Kingsbury DJ, Klein-Gitelman M, Lawson EF, Lovell DJ, Mason T, McCurdy D, Nanda K, Nassi L, O Neil KM, Rabinovich E, Pope E, Doria AS, Theriault M, Mohanta A, Laxer RM. Topical imiquimod 5% cream for pediatric plaque morphea: A prospective, multiple-baseline, open-label pilot study. Dermatology 2011: 223(4): pp Ramsey SE, Reiff A, Rosenkranz M, Schikler K, Stevens A, Wahezi D, Cabral DA, ARChiVe Investigators Network. Assessing the performance of the Birmingham Vasculitis Activity Score at diagnosis for children with antineutrophil cytoplasmic antibody-associated vasculitis in A Registry for Childhood Vasculitis (ARChiVe). Journal of Rheumatology 2012: 39(5): pp Sleeper LA, Minich LL, McCrindle BM, Li JS, Mason W, Colan SD, Atz AM, Printz BF, Baker A, Vetter VL, Newburger JW. Evaluation of Kawasaki disease risk Scoring systems for intravenous immunoglobulin resistance. Pediatric Heart Network Investigators. Journal of Pediatrics 2011: 158(5): pp e3. Stinson JN, Connelly M, Jibb LA, Schanberg LE, Walco G, Spiegel LR, Tse SML, Chalom EC, Chira P, Rapoff M. Developing a standardized approach to the assessment of pain in children and youth presenting to pediatric rheumatology providers: A Delphi survey and consensus conference process followed by feasibility testing. Pediatric Rheumatology Journal (Online) 2012: 10: pp 7. Stinson JN, Feldman BM, Duffy CM, Huber AM, Tucker LB, McGrath PJ, Tse SM, Hetherington R, Spiegel LR, Campillo S, Benseler SM, Gill N, White ME, Baker N, Vijenthira A. Jointly managing arthritis: Information needs of children with juvenile idiopathic arthritis (JIA) and their parents. Journal of Child Health Care 2012: 16(2): pp Tse SML, Burgos-Vargas R, Colbert RA. Juvenile spondyloarthritis treatment recommendations. American Journal of the Medical Sciences 2012: 343(5): pp Tse S, Laxer RM. New advances in juvenile spondyloarthritis. Nature Reviews Rheumatology 2012: 8(5): pp Twilt M, Benseler SM, Cabral D. Granulomatosis with polyangiitis in childhood. Current Rheumatology Reports 2012: Jan 29. Twilt M, Benseler SM. The spectrum of CNS vasculitis in children and adults. National Review in Rheumatology 2011: 8 (2): pp: Twilt M, Laxer RM: Clinical care of children with sterile bone inflammation. Current Opinion in Rheumatology 2011: 23(5): pp Uribe AG, Huber AM, Kim S, O Neil KM, Wahezi DM, Abramson L, Baszis K, Benseler SM, Bowyer SL, Campillo S, Chira P, Hersh AO, Higgins GC, Eberhard A, Ede K, Imundo LF, Jung L, Kingsbury DJ, Klein-Gitelman M, Lawson EF, Li SC, Lovell DJ, Mason T, McCurdy D, Muscal E, Nassi L, Rabinovich E, Reiff A, Rosenkranz M, Schikler KN, Singer NG, Spalding S, Stevens AM, Cabral DA, A Registry for Children with Vasculitis e-entry (ARChiVe) Network. Increased sensitivity of the European Medicines Agency algorithm for classification of childhood granulomatosis with polyangiitis. Journal of Rheumatology 2012: 39(8): pp Uribe AG, Huber AM, Kim S, O Neil KM, Wahezi DM, Abramson L, Baszis K, Benseler SM, Bowyer SL, Campillo S, Chira P, Hersh AO, Higgins GC, Eberhard A, Ede K, Imundo LF, Jung L, Kingsbury DJ, Klein-Gitelman M, Lawson EF, Li SC, Lovell DJ, Mason T, McCurdy D, Muscal E, Nassi L, Rabinovich E, Reiff A, Sabri K, Saurenmann RK, Silverman ED, Levin AV. Catarac and juvenile arthritis. Opthalmology 2011: 118 (12): pp Wallace C, Giannini E, Huang B, Itert L, Rupeto N, CARRA, Pediatric Rheumatology Collaborative Study Group, Paediatric Rheumatology Trials Organization. American College of Rheumatology provisional criteria for defining clinical inactive disease in select categories of juvenile idiopathic arthritis. Arthritis Care and Research 2011: 63 (7): pp Webb BD, Barrera M, Beyene J, Carcao M, Daneman D, Elliott I, Gong GWK, Halperin IJ, Lord S, Melville H, Narayanan UG, Ota S, Solomon M, Sung L, Young NL, Zachos M, Feldman BM. Determinants of quality of life in children with chronic somatic disease: Pilot data from the GapS Questionnaire. Quality of Life Research 2012: March (Epub ahead of print). Williams T, Aranow C, Ross G, Barsdorf A, Imundo L, Eichenfield A, Kahn P, Diamond B, Levy DM. Neurocognitive impairment in childhood-onset systemic lupus erythematosus: Measurement issues in diagnosis. Arthritis Care Research 2011: 63(8): pp the hospital for sick children department of paediatrics Annual Report

96 Wittkowski H, Kuemmerle-Deschner JB, Austermann J, Holzinger D, Goldbach-Mansky R, Gramlich K, Lohse P, Jung T, Roth J, Benseler SM, Foell D. MRP8 and MRP14, phagocyte-specific danger signals, are sensitive biomarkers of disease activity in cryopyrin-associated periodic syndromes. Annals of Rheumatic Disease 2011: 70(12): pp Yu A, Stephens D, Feldman BF, Parkin PC, Kahr WHA, Brandão LR, Shouldice M, Levin AV. The role of prothrombotic factors in the ocular manifestations of abusive and non-abusive head trauma: A feasibility study. Child Abuse and Neglect 2012: 36(4): pp Books and Chapters Dell S, Schneider R. Pulmonary involvement in the systemic diseases of childhood. In: Disorders of the Respiratory Tract in Children, Eighth Edition (Wilmott R, Chernick V, Bush A, Deterding R, Boat T, Ratjen F, eds.). Elsevier, Philadelphia, PA Laxer RM, Pope EM, O Brien C. Localized scleroderma in a child. In: Case Studies in Systemic Sclerosis. (Silver R, Denton C, eds.). Springer 2011: pp Yeung RSM. Vasculitis: Kawasaki disease. In: Textbook of Clinical Pediatrics, Second Edition (Elzouki AY, Harfi HA, Nazer H, Stapleton FB, Oh W, Whitley RJ, eds.). 2012: pp Grants and Funding A one-year open-label, multicenter trial to assess efficacy, safety and tolerability of Canakinumab (ACZ885) and the efficacy and safety of childhood vaccinations in patients aged 4 years or younger with Cryopyrin Associated Periodic Syndromes (CAPS). Laxer RM, Schneider R, Tse S. Novartis Pharmaceuticals Incorporated. $42,646 CAD. Apr 2011 to Apr 2013 Autoimmunity in sisters of SLE patients (SisSLE): Mapping auto-immune phenotypes in multiplex families. Gregeren P, Diamond B, Levy DM. NIH-NIAID R01A $485,422 CAD to 2013 Biologic outcome predictors in juvenile idiopathic arthrits. Rosenberg A, Muhajarine N, Scherer S, Whiting SJ, Duffy C, Baxter-Jones, ADG, Oen K, Turvey S, Yeung RSM, Silverman ED, Gordon JR, Pahwa P, Von Baeyer CL, Bernatsky S, Boire G, Busch A, Cabral D, Cameron B, Campillo S, Dancey P, Davison S, D Eon M, Doria A, Dorval J, Duffy K, Ellsworth J, Feldman B, Houghton K, Huber A, Johnston N, Koren G, Kulyk W, Lang B, Lawson J, Laxer, RM, LeBlanc C, Malleson P, McDuffie H, Miettunen P, Nair B, Olszynski W, Petroz G, Petty R, Ramsay S, Reed M, Schneider R, Sibley J, Spiegel L, Stinson J, Succimmari R, Taylor-Gjevre R, Tse S, Tucker L, Wrogemann J. Canadian Institutes of Health Research. $1,250,000 CAD. Jul 2006 to Jun 2013 Biomarker discovery and targets of pathology in congenital heart block. Hamilton R, Jaeggi E, Silverman E. March of Dimes. $482,163 CAD. Dec 2011 to Nov 2014 Biomarker discovery and mechanisms of fetal pathology in congenital heart block. Hamilton R, Standberg L, Silverman E, Jaeggi E, Gramolini A, Ackerley C. Canadian Institutes of Health Research. $1,031,989 CAD. Oct 2012 to Sep 2017 Canadian Child Health Clinical Scientist Program (CCHCSP). Levy DM. Career Enhancement Program Award. $10,000 CAD to 2012 Cancer risk in pediatric-onset rheumatic disease. Bernatsky S, Silverman E, Clarke A, Duffy C, Rosenberg A. Canadian Institutes of Health Research. $77,745 CAD to 2011 Cerebral venous hemodynamics in pediatric multiple sclerosis. Banwell B, Macgowan C, Laughlin S, Shroff M, Sled J, Yeung RSM, Benseler S, Traubici J, Moharir M, Arnold D, Narayanan S, Bar-Or A, Marrie RA. Multiple Sclerosis Society of Canada. $196,579 CAD to 2012 Childhood onset SLE: Determining long-term outcomes. Levy DM. Physicians Services Incorporated. $158,000 CAD to 2013 Chronic childhood vasculitis: Characterizing the individual rare diseases to improve patient outcome. Cabral D, Benseler SM, Foell D, Hancock R, Colin R. Canadian Institutes of Health Research. $2,500,000 CAD to 2017 CIHR Team in linking exercise, physical activity and pathophysiology in childhood arthritis: A Canadian Collaborative Team. Duffy C, Tucker L, Baxter-Jones A, Houghton K, Macdonald H, McKay H, Oen K, Rosenberg A, Roth J, Ward L, Yeung R, Benseler S, Feldman D, Guzmán J, Stringer E, Boire G, Cabral D, Cameron B, Campillo S, Chédeville G, Cheung A, Dancey P, Ellsworth J, Huber A, Johnson N, Karunanayake C, Lang B, Laxer R, Miettunen P, Pullenayegum E, Ramsey S, Schneider R, Scuccimarri R, Silverman E, Spiegel L, Schmeling H, Tse S, Turvey S, Watanabe Duffy K. Canadian Institutes of Health Research. $2,500,000 CAD. Oct 2010 to Sep 2015 Comparing the burden of illness of hemophilia in the developing and the developed world: The Sao Paulo-Toronto Hemophilia Study. Feldman BM, Blanchette V. Baxter Healthcare Corporation. $219,637 CAD. Feb 2010 to Feb 2013 Creating a novel mentorship program for junior faculty at a pediatric academic health sciences centre The role of a modified developmental evaluation approach. Weiss S, Park K, Martimianakis M, Schneider R. The Hospital for Sick Children, Paediatric Consultants Education Research Grants Committee. $4,820 CAD Determinants of risks and outcomes in inflammatory arthritis. Siminovitch K, Yeung RSM, Bernatsky S, Bombardier C, Boillais L, MacPherson A, Oen K. Canadian Institutes of Health Research. $2,000,000 CAD to 2012 Determining collaborative practice behavior following the introduction of an advanced clinician practitioner in arthritis care across diverse clinical settings in Ontario. Lundon K, Shupak R, Lineker S, Schneider R, Passalent L, Sinclair L, Kennedy C. Canadian Initiative for Outcomes in Rheumatology Care. $49,898 CAD to 2011 Development of a novel strategy to teach the Pediatric MSK Examination: Adolescents patient partners with Juvenile Idiopathic Arthritis (JIA), Phase 1: Exploring teens perception and motivation to participate. Morin M, Mylopoulos M, Schneider R, Bell M, Dubrowski A, Bourse en pédagogie médicale, CHU Saint-Justine. PSI Foundation Medical Education Research and Development Grant. $16,000 CAD. Oct 2011 to Jan 2013 Effects of creatine supplementation on muscle pathophysiology in children with dermatomyositis. Stephens S, Feldman BM, Wells GD, Tein I, Schulze A. Rare Disease Foundation and the BC Children s Hospital Foundation. $3,500 CAD to 2013 Effects of pubertal hormone changes on SLE. O Neil K, Levy DM. Lupus Foundation of America. $60,000 CAD to 2012 Faculty and resident perceptions about teaching and learning the Paediatric Musculoskeletal Examination: An exploratory study. Schneider R, Hutchinson J. The Hospital for Sick Children, Pediatric Consultants Education Research Grants Committee. $4,925 CAD. Jul 2011 to Jun 2012 Hemophilia dose escalation primary prophylaxis (CHPS-3) observationsal extension study. Feldman BM, Blanchette V, Rivard GE, Israels S, Chan A, Lillilcrap D, Willan A, Einarson T, Babyn P, Doria A, Wintle R. Bayer Inc. $583,671 CAD to 2012 How genes determine SLE phenotype and outcome. Silverman ED, Kamphuis S, Beyene J, Scherer S. Alliance for Lupus Research. $350,000 CAD to 2012 Inflammation and atherosclerosis in pediatric rheumatic disease. Bradley T, Silverman ED, Schneider R, Feldman B, Beyene J. The Heart and Stroke Foundation of Canada. $191,533 CAD to 2011 Jointly managing JIA online: An internet-based psychoeducational program for children with Juvenile Idiopathic Arthritis (JIA) and their parents. Stinson J, McGrath P, Palermo T, Spiegel L, Tse S, Tucker L, Victor J. Canadian Institutes of Health Research. $435,889 CAD the hospital for sick children department of paediatrics Annual Report

97 Kawasaki disease: On our way to unraveling the pathogenesis. Yeung RSM. Canadian Institutes of Health Research. $425,214 CAD to 2011 Kawasaki disease: On our way to unraveling the pathogenesis. Yeung RSM, McCrindle B, Siminovitch K. Canadian Institutes of Health Research. $798,779 CAD to 2016 Local temperature as a marker of disease activity and progression in patients with morphea. Pope E, Laxer RM. Rare Disease Foundation. $3500 CAD. May 2012 to May 2013 M trial: A double-blinded, placebo-controlled, multicenter study of the efficacy and safety of the Adalimumab in pediatric patients with enthesitis related arthritis. Burgos-Vargas R, Tse SM. Abbott International. $174,792 CAD to 2015 Malignancy in pediatric-onset SLE. Bernatsky S, Silverman ED. The Leukemia and Lymphoma Society of Canada. $75,545 CAD to 2012 Neurocognitive function in childhood-onset systemic lupus erythematosus (csle): A 36-month prospective study. Levy DM (Mentors: Silverman E, Diamond B). NIH/NIAMS- K23AR A2. $648,540 CAD to 2012 System level evaluation of the Advanced Clinician Practitioner in Arthritis Care (ACPAC) program graduates in Ontario. Lundon K, Shupak R, Soever L, Kennedy C, Schneider R, Lineker S. Ontario Ministry of Health. $160,010 CAD to 2011 Superantigens in autoimmunity. Yeung RSM. Canadian Institutes of Health Research. $461,325 CAD to 2012 Teens taking charge: An internet-based self-management program for adolescents with arthritis: A randomized controlled trial. Stinson J, Campillo S, Dancey P, Duffy C, Ellsworth J, Feldman B, Fuss S, Hetherington R, Huber A, Johnson N, McGrath P, Oen K, Rosenberg A, Spiegel L, Tse S, Tucker L, Victor C. Canadian Institutes of Health Research. $442,140 CAD. Oct 2011 to Sep 2014 Tocilizumab in polyarticular JIA. Silverman ED, Schneider R, Benseler S. Roche Pharmaceuticals. $195,000 CAD to 2013 Towards a better understanding of Kawasaki disease. Yeung RSM, Benseler S, Feldman B, Duffy C, Rosenberg A. The Heart and Stroke Foundation of Canada. $239,158 CAD to 2011 Towards a better understanding of Kawasaki disease. Yeung RSM, McCrindle B, Siminovitch K. Canadian Institutes of Health Research. $792,717 CAD to 2016 Understanding Childhood Arthritis Network (UCAN): Towards a harmonized approach to the conduct of research in childhood arthritis worldwide. Yeung RSM. Canadian Arthritis Network, Sustained Impact Platform Program Supplemental Funding. $30,000 CAD to 2013 Validation of the Children s International Mucositis Evaluation Scale. Sung L, Feldman BM. Canadian Institutes of Health Research. $457,898 CAD. Apr 2010 to Mar 2012 Whole body MRI in juvenile spondyloarthropathy. Tse SM, Rachlis A, Babyn P, Stimec J, Mueller E, Anderson M, Whitney K, Marcuz J, Delaurier A. Spondyloarthritis Research Consortium of Canada. $9,784 CAD Widening the therapeutic window in acute ischemic stroke with blood-brain-barrier permeability and its relationship with corresponding blood markers: Implications for short and long-term outcomes. Kassner A, Yeung RSM. Heart and Stroke Foundation of Canada. $136,490 CAD to 2013 Pediatric Rheumatology Optimized Platforms for Experimental Research (PROPER Project). Rosenberg A, Yeung RSM. Canadian Arthritis Network Platforms Grant (CAN-RIPP). $60,000 CAD to 2011 Periodic fever syndrome in Canada: Canadian Pediatric Society Prevalence Study. Dancey P, Benseler S, Junker A, Laxer RM, Miettunen P, Turner L, Gattorno M. Canadian Pediatric Society, Novartis Pharmaceutical Incorporated. $58,000 CAD to 2014 Pharmacogenetics of juvenile idiopathic arthritis A genome wide association study on the efficacy and toxicity of methotrexate therapy. Schmeling H, Feldman BM, Siminovitch K, Benseler S. Canadian Arthritis Network. $240,000 CAD. Apr 2010 to Mar 2012 Towards developing MR imaging guidelines and outcome measures in juvenile idiopathic arthritis: A special interest pediatric research group. Doria A, Van Rossum M, Benseler SM, Miller E, Rosenberg A, Roth J, Stinson J, Tse S, Yeung R. Canadian Institutes of Health Research. $24,954 CAD to 2013 Towards a harmonized approach to the conduct of research in childhood arthritis worldwide. Yeung RSM, Feldman B, Wedderburn L, Prakken B. Province of Ontario Ministry of Research and Innovation, International Strategic Opportunities Program. $450,000 CAD to 2013 Towards a wireless pain diary support system for youth with juvenile idiopathic arthritis. Stinson J, Benseler S, Feldman B, Laxer R, Luca N, Spiegel L. Canadian Institutes of Health Research. $15,000 CAD. Oct 2010 to Jan the hospital for sick children department of paediatrics Annual Report

98 TRANSPLANT AND REGENERATIVE MEDICINE CENTRE overview Medical Director Dr. David Grant Executive Director Judy Van Clieaf Transplant Fellows 2011 Dr. Marsha Gooden, Renal Transplant Dr. Hannah Piper, GIFT Program Dr. Ian Chen, Adolescent Medicine Dr. Suren Thavagnanam, Lung Transplant Dr. Steven Greenway, Heart Transplant Administrative Staff Christine Garner, Administrative Coordinator Kathryn Breckbill, Project Coordinator Christopher Battiston, Data Analyst Centre Members The Clinical Teams include members of the Departments of Nephrology, Gastroenterology, Hepatology & Nutrition, Cardiology, Respiratory Medicine, Psychiatry, Psychology, General Surgery and Infectious Diseases. The success of the transplant programs can also be attributed to strong contributions by interdisciplinary team members from occupational and physiotherapy, nutrition, social work and pharmacy. We have become one of the busiest paediatric transplant centres in North America and a world leader in innovative care. Centre Overview and Clinical Activity SickKids made history in 1969 by performing Canada s first paediatric kidney transplantation. We have become one of the busiest paediatric transplant centres in North America and a world leader in innovative care, improving the health of children by integrating care, research and teaching. The Centre provides the best in complex and specialized care through scientific and clinical research, sharing knowledge and expertise; and advocacy for children who need transplant care. Our transplant and intestinal rehabilitation case volumes have grown to within the top five to 10 per cent of centres in North America. Our outcomes match or exceed the best programs. This past year, our Centre completed a record number of transplants; 73 in total including 29 transplants by the Kidney and Liver Transplant Programs which were both unusually busy. As innovators in transplantation, during this past year our lung transplant program completed the first infant lung transplant in Canada and the first ABO-incompatible lung transplant in the world. department of paediatrics Annual Report

99 In January 2012, The Transplant Centre was re-branded as the Transplant and Regenerative Medicine Centre (TRMC). Our immediate mission is to enhance the quality of life of paediatric transplant recipients through innovative research, education and care. To do this, we aim to enhance long-term graft function and quality of life by using stem cells and other novel therapies to 1) transplant organs without maintenance immune suppression and 2) improve the function of deceased donor organs. Our longer term vision is to harness the unique regenerative capacity of paediatric tissues to repair organs, making transplants unnecessary. Research Highlights Research and new knowledge dissemination are major priorities for the Centre. Centre members are the principal or co-investigators for CIHR and/or disease-specific philanthropic extramural awards to conduct basic, translational and clinical research. All of our clinical transplant programs are actively recruiting patients to NIH-sponsored clinical trials. Currently there are over 40 active REB-approved research projects underway. In the past year, members published upwards of 87 publications around the issues of transplantation and graft management, end-stage organ diseases, inflammation or repair and intestinal failure and gut repair. A selection of TRMC publications are provided at the end of this report. Successful fundraising by the Centre has substantially enhanced research. We are currently provided with more than $400,000 for multi-year research support. In our Research Committee chaired by Dr. Binita Kamath facilitated four grant competitions to support clinical innovations and provide seedfunding for high-risk / high-gain innovative scientific research. In addition, we are stewarding targeted research funding from the Liam s Light Foundation and The Gift Gala to support multidisciplinary research on liver, kidney and intestinal organ failure. An example of innovative research is a study recently completed by The Group for the Improvement of Intestinal Function and Treatment (GIFT) led by Dr. Paul Wales. Liver failure is the most common cause of death in intestinal failure patients due to chronic parenteral nutrition, sepsis and the inability to eat. This past year, Dr. Wales led and completed a Canadian, multicentre randomized trial of a novel lipid emulsion formulation for intravenous nutrition (SMOFlipid). This study examines the efficacy of an ω3fa (Omega-3 fatty acid) containing balanced lipid emulsion in the prevention of progression of parenteral nutrition-associated liver disease (PNALD) in infants with Intestinal Failure/Short Bowel Syndrome (SBS) and early liver dysfunction. Preliminary data suggest that administration of this new nutritional produce will dramatically reduce the incidence of fatal liver disease in this highly vulnerable paediatric population. Patient recruitment has finished and the study outcomes are now being analyzed for reporting and publication. Education Highlights Five elearning Modules: Second Year Paediatric ilearn Modules SickKids is responsible for training the next generation of physicians who will care for paediatric transplant recipients. A working group led by Dr. Yaron Avitzur and Dr. Paul Kantor has developed five e-learning modules to educate trainees about paediatric transplantation. This initiative has enhanced awareness of transplant-related issues and will improve the efficacy of self-education of second-year paediatric residents. These modules will be presented in national and international academic meetings as examples of the TRMC s innovations in medical care and education. Centre Advocacy High School Outreach Initiative The TRMC Strategic Initiatives Working Group Chaired by Dr. Vicky Ng launched a unique, teen-focused educational outreach program to educate youth about organ donation. This innovative pilot project partnered the three University of Toronto transplant programs and Trillium Gift of Life Network (TGLN) to educate high school students about the lifesaving impact of organ and tissue donation and transplantation. For this pilot project nearly 100 health-care providers from SickKids, St. Michael s Hospital and University Health Network volunteered and attended training workshops. Between March and June 2012, a health-care provider, a transplant recipient and a TGLN representative visited 35 high schools from the Toronto Catholic District School Board and Toronto District School Board, presenting to over 6,000 high school students at 54 class presentations and assemblies. Plans are underway for expansion of this partnership into the school year. The project highlights the ongoing efforts of the TRMC for patient advocacy through collaborations with partners at TGLN and Toronto Transplant Institute. Selected Honors and Awards Anthony, A: ISHLT Nursing and Social Sciences Excellence in Research Award. Conway, J: ISHLT Transplant Registry Junior Faculty Award; IPTA Young Investigator Award. Courtney-Martin, G: 2011 CST Associate Member s Research Grant. Mouzaki, M: 2011 Travel Award for AASLD Single Topic Symposium on Pediatric Liver Transplantation. Vanderlaan, R: SickKids Resident Research Day Award; IPTA Distinguished Abstract Award. Yuen, D: Awarded the inaugural Canadian Society of Transplantation Fellowship for his research on signaling axes regulating cell migration with Dr. Lisa Robinson. Selected Publications Amir A, Shapiro R, Shulman LM, Mor E, Steinberg R, Fleishhacker H, Bergerin R, Avitzur Y. BK virus infection and its effect on renal function in pediatric liver transplant recipients: A cross sectional, longitudinal, prospective study. Transplantation 2011: 92: pp Bilavski E, Dagan A, Yarden-Bilavski H, Davidovits M, Shapiro R, Weintraub N, Amir J, Avitzur Y. Adrenal insufficiency during physiological stress in children after kidney or liver transplantation. Pediatric Transplantation 2011: 15: pp Conway J, Dipchand AI. Transplantation as a therapy for pediatric cardiomyopathies: When to list and risk factors for death while waiting. Progress in Pediatric Cardiology 2011: 32: pp Conway J, Manlhiot C, Allain-Rooney T, McCrindle BW, Lau W, Dipchand AI. Development of donor-specific isohemagglutinins following pediatric ABO-incompatible heart transplantation. American Journal of Transplantation 2012: Jan 10. Frieling M, Williams A, Al Shareef T, Kala G, Teh JC, Langlois V, Allen U, Hebert D, Robinson LA. Novel influenza (H1N1) infection in pediatric renal transplant recipients: A single-center experience. Pediatric Transplantation 2012: 16: pp George JF, Taylor DO, Blume ED, Kirklin JK, Naftel DC, Brown RN, Dipchand AI, McGiffin DC, Pamboukian S, Tallaj J. Minimizing infection and rejection death: Clues acquired from 19 years of multi-institutional cardiac transplantation data. Journal of Heart and Lung Transplantation 2011: 30(2): pp Gijsen V, Mital S, van Schaik RH, Soldin OP, Soldin SJ, van der Heiden IP, Nulman I, Koren G, de Wildt SN. Age and CYP3A5 genotype affect tacrolimus dosing requirements after transplant in pediatric heart recipients. Journal Heart Lung Transplant 2011: 30(12): pp Grasemann H, de Perrot M, Bendiak GN, Cox P, van Arsdell GS, Keshavjee S. Solomon M. ABO-incompatible lung transplantation in an infant. American Journal of Transplantation 2012: 12(3): pp Gupta AA, Gerstle JT, Ng V, Wong A, Fecteau A, Malogolowkin MH, Meyers RL, Grant D, Grant RM. Critical review of controversial issues in the management of advanced pediatric liver tumours. Pediatric Blood Cancer 2011: 56(7): pp Nightingale S, McEwan-Jackson FD, Hawker GA, Macarthur C, Khambalia AZ, Lo L, Fecteau A, Ng VL. Corticosteroid exposure is not associated with long-term bone mineral density in pediatric liver transplantation. Journal Pediatric Gastroenterology and Nutrition 2011: 53(3): pp Superina R, Magee JC, Brandt ML, Healey PJ, Tiao G, Ryckman F, Karrer FM, Kishore I, Fecteau A, West K, Burns RC, Flake A, Lee H, Lowell J, Dillon P, Columbani P, Ricketts R, Li Y, Moore J, Wang KS and the Childhood Liver Research and Education Network (ChiLDREN). The anatomic pattern of biliary atresia identified at time of Kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival. Annals of Surgery 2011: 254(4): pp Wales PW, Kosar C, Carricato M, de Silva NT, Lang K, Avitzur Y. Ethanol lock therapy to reduce the incidence of catheter related bloodstream infections (CRBSI) in home TPN patients with intestinal failure: Preliminary experience. Journal of Pediatric Surgery 2011: 46(5): pp the hospital for sick children department of paediatrics Annual Report

100 grand rounds grand rounds schedule The Department of Paediatrics Grand Rounds carries on a time-honoured tradition of academic excellence. Every Wednesday morning at 9 a.m. the Daniels Hollywood Theatre is filled with a variety of learners including medical students, residents and fellows, medical staff, research staff as well as other SickKids staff. Grand Rounds topics cover a multitude of issues that address the broad determinants of child health as they relate to the University of Toronto s Department of Paediatrics. The Grand Rounds committee, chaired by Ron Laxer, is comprised of the following members: Eyal Cohen, Elizabeth Donner, Marvin Gans, Jamie Hutchison, Laila Nourimand, Evelyn Roszenblum, Rayfel Schneider, Lillian Sung and Neil Sweezey. The committee seeks input from members of the Department, as well as the hospital at large, to develop an exciting series of presentations for the academic year (see list below). The summer Grand Rounds series coordinated by the chief residents, allows senior trainees and subspecialty fellows to provide updates within specific specialties and sub-specialties. In , a new initiative, entitled Daneman and Friends was extremely well received. In these sessions, our Department Chair invites several staff to address a specific theme. As in past years, the chief residents rounds, entitled Cases from the Ward, remain a highlight where three to four senior paediatric residents present unique cases that they have come across. Professors Rounds are delivered by individuals who have been promoted to full Professor in the Department of Paediatrics within the previous year. Professors Rounds were covered by Dr. Jamie Hutchinson, Dr. Ann Jeffries, Dr. Patricia Parkin and Dr. Hilary Whyte. The Department is fortunate to be able to host a number of named lectures. This past year, Dr. Roger Strasser, Dean of the Northern Ontario School of Medicine, delivered the Harry Bain Lecture. SickKids own Dr. Brian Robinson gave the Jacob s Ladder Lecture; Dr. Berent Prakken from the Netherlands gave the Abe Shore Lecture; Dr. Carter Snead gave the Robert H. A. Haslam Lecture, and Dr. Shai Izraeli from Tel Aviv gave the Daniel Burton Leukemia Lecture. The Medical Education Day was a tribute to Dr. Sue Tallett for her many years of dedication and contribution to the Department of Paediatrics. A very provocative and stimulating lecture was given by Dr. Nuala Kenny from Dalhousie University. The Andrew Sass-Kortsak Award Lecture was delivered by Dr. Tuan Trang. Once again, the Department of Paediatrics Grand Rounds Committee acknowledges the tremendous help and support of a number of individuals who do the vast majority of the behind-the-scenes work, specifically Valerie Brown in Department of Paediatrics and Frank Ferrari and Sam Mendolia in the Audio Visual Department at SickKids. date name title Jul 6 Dr. Sarah Schwartz Current Issues in the Care of Children with Medical Complexity General Paediatrics Jul 13 Drs. Johan Van Limbergen and Peter Church Beyond Symptoms: Crohn s Disease Treatment in 2011 GI/Hepatology/Nutrition Jul 20 Drs. Alene Toulany and Trisha Tulloch Ending the Food Fight Before our Kids FAED Away Adolescent Medicine Jul 27 Dr. Deepali Mankad and Dr. Andrea Hoffman Neuroplasticity in the Developing Brain Developmental Paediatrics Aug 3 Dr. Al-Naimi A to Zzz of pediatric sleep Respiratory Medicine Aug 10 Drs. Gordon Soon, Senq Lee and Marinka Twilt More than just skin deep - Childhood vasculitis Rheumatology Aug 17 Drs. Janice Tijssen and Vijay Anand Critical Care Medicine Management of Acute Asthma: Owl s Blood, Chicken Soup, 1500 years of progress what about the last 15? Aug 24 Drs. Walla Al-Hertani, Shailly Jain and Ramses Badilla-Porras Clinical Genetics and Metabolics The Bedside Sherlock Holmes: Lessons from arrays, peroxisomes and blood spots Aug 31 Dr Cecilia Herbozo, Dr Enas Elgouhaery Recent Changes in Neonatal Resuscitation and Dr Edward Pugh Neonatology Sep 7 Dr. Roger Strasser Bain Lecture: Education for Child Health in the North Dean, Northern Ontario School of Medicine Sep 14 Dr. Patricia Parkin Building Academic General Pediatrics at SickKids and Beyond Division of Paediatric Medicine, Professor, University of Toronto Sep 21 Dr. Daniel Wechsler Chief, Pediatric Hematology-Oncology, Duke University Medical Center, Durham, NC Keep CALM and Carry On : The Role of CALM Translocations in Leukemogenesis 198 the hospital for sick children department of paediatrics Annual Report

101 grand rounds schedule continued date name title date name title Sep 28 Oct 5 Oct 12 Oct 19 Oct 26 Dr. Victor Blanchette Former Head, Division of Hematology/Oncology Dr. Joanne Wolfe Director, Pediatric Palliative Care, Children s Hospital Boston Division Chief, Pediatric Palliative Care Service, Department of Psychosocial Oncology and Palliative Care, Dana-Farber Cancer Institute, Boston, MA Dr. Leona Cuttler Endocrinology, Rainbow Babies and Childrens Hospital and Case Western Reserve University, Cleveland Dr. Peter Scal Explorer Clinic, Minneapolis, MN Dr. Jean-Laurent Casanova Head, Laboratory of Human Genetics of Infectious Disease, Rockefeller University, NY Childhood Immune Thrombocytopenic Purpura (ITP): What is Appropriate Care? Easing Suffering in Children with Life-Threatening Illness Controversies in the Management of Childhood Short Stature: Evidence, Practice, and Policy Building Rheumtogrow.org: An Internet Based Program for Youth with Arthritis Life-threatening infectious diseases of childhood: single-gene inborn errors of immunity? 2012 Jan 4 Jan 11 Jan 18 Jan 25 Dr. Jamie Hutchison Research Director, Critical Care Medicine, SickKids Dr. Anne Matlow, Medical Director, Infection Prevention & Control Programme; Medical Director, Patient Safety; Associate Director, University of Toronto Centre for Patient Safety Dr. Trey Coffey, Staff, Paediatric Medicine, SickKids Dr. Maria Isabel Achatz Oncogenetics, Hospital A.C. Camargo, Sao Paolo, Brazil Dr. Debbie O Connor Physiology and Experimental Medicine, SickKids Dr. Sharon Unger Pediatrics, Mount Sinai Hospital Neuroprotection in brain injury In our own back yard: Adverse events in children hospitalized in Canada TP53 gene, Li-Fraumeni Syndrome and the treaty of Tordesillas: the high frequency of a founder mutation Not enough mother s own milk? The case for donor human milk for the vulnerable infant. Nov 2 Dr. Denis Daneman and Friends (Dr. Jill Hamilton, Dr. David Malkin, Dr. Rayfel Schneider) Dept Pediatrics, SickKids Changing life s trajectory: Scratching the surface of Personalized Medicine Feb 1 Dr. Berent Prakken Pediatric Immunology, Rheumatology University Medical Centre, Utrecht, Netherlands Abe Shore Lecture: Understanding Childhood Arthritis: A continuous inspirational journey from bench to bedside Nov 9 Nov 16 Dr. Eric D. Kodish Professor and Chair, Bioethics, Lerner College of Medicine, Case, Western Reserve University, OH Dr. John Engelhardt Anatomy and Cell Biology, University of Iowa, Iowa City Context Matters: Informed Consent in Childhood Cancer Research Alterations to Stem/Progenitor Cell Niches in the Cystic Fibrosis Airway Feb 8 Feb 15 Dr. Ann Jefferies, Neonatology, Mt Sinai and SickKids Dr. Joy Lawn Director of Global Evidence and Policy, Saving Newborn Lives/Save the Children USA You Want Me to Assess Advocacy? Assessing Multiple Physician Competencies During Residency Training Global Health Day: Newborn Survival around the World: Progress and Future Priorities Nov 23 Dr. Brian Robinson Canada Research Chair in Metabolism and Nutrition; Senior Scientist, Genetics & Genome Biology Programme, Research Institute, SickKids Jacob s Ladder Lecture: How the brain regulates its energy supply: Clues from the many genetic defects affecting the pyruvate dehydrogenase complex in children Professor, Departments of Biochemistry and Paediatrics, University of Toronto Nov 30 Dr. Evaline Alessandrini Director, Quality Scholars Program in Health Care The Acute Care Model: A New Framework for Improving the Quality of Emergency Care Transformation, Cincinnati Children s Hospital Dec 7 Chair: Dr. Melissa Chan Cases from the Ward Dr. Charles Larson, Dr. Lindsey Craw, Dr. Luke Jagiello Residents, Dept Pediatrics, SickKids Dec 14 Residents and Fellows Holiday Grand Rounds Feb 22 Clinical Pathology Conference Feb 29 Dr. Melissa Chan More Cases from the Ward Chief Resident, Dept Pediatrics, SickKids Mar 7 Denis and Friends Cases at the edge.. Drs. Denis Daneman, Eyal Cohen, Paul Nathan, Indra Narang and Steve Freedman Mar 14 March Break No Rounds Mar 21 Dr. Randy Jirtle Epigenetics, Nutrition, and Disease Susceptibility Professor, Dept Radiation Oncology Duke University Medical Centre, Durham, NC Mar 28 Dr. Nuala Kenny Professor Emeritus, Dept Bioethics, Dalhousie University, NS Medical Education Day: Tribute to Sue Tallett The Possibility of Professionalism in a Globalized World 200 the hospital for sick children department of paediatrics Annual Report

102 grand rounds schedule continued awards day date name title Apr 4 Dr. Carter Snead Division Head, Neurology, SickKids Haslam Lecture: Symphony of the Brain: Dissonance in the Melody Apr 11 Dr. Phyllis A. Dennery Professor of Pediatrics, University of Pennsylvania Chief, Division of Neonatology, Werner and Gertrude Henle Barry Smith Lecture: A tale of two oxygens: How a small molecule defined Neonatology Chair in Pediatrics Children s Hospital of Philadelphia Apr 18 Dr. Hilary Whyte A transport system - the instrument of regionalized health care. Neonatology, Transport, SickKids Apr 25 Drs. Brian Feldman, Etienne Sochett, Leanne Ward, Steven Teitelbaum Steroids and Bones: Glucocorticoid Osteotoxicity in Children with Chronic Disease Rheumatology, SickKids May 2 Dr. Lillian Sung Staff Physician Decision Making in Pediatric Oncology: Evaluation and Incorporation of Patient and Parent Preferences Haematology/Oncology May 9 Dr. Steven Miller Canada Research Chair in Neonatal Neuroscience UBC, BC Children s Hospital Paediatric Update: Newborn brain injury: More than meets the eye (Videoconference from Board of Trade Centre) Incoming Head, Division of Neurology, SickKids May 16 Dr. Shai Izraeli Head, Functional Genomics and Childhood Leukemia Research Sheba Medical Center, Tel Aviv University, Israel Daniel Burton Leukemia Lecture: Pediatric syndromes as experiments of Nature: Lessons from the leukemias of Down Syndrome May 23 Dr. Mary Motz, Psychologist, Breaking the Cycle, Toronto Motherisk Lecture: Breaking the Cycle: Advances in Secondary Prevention of In Utero Alcohol and Drug Exposure May 30 Pediatric Research Day No Rounds Colony Grande Ballroom, Chestnut Conference Centre, 89 Chestnut Street Jun 6 Dr. Richard Weinshilboum Pharmacology Chair, Mayo Clinic, Rochester, MN DPLM Symposium Day: Pharmacogenomics: Beyond Biomarkers Jun 13 Winner of the 24th Annual P2X Receptors, Chronic Pain, and the Poppy Andrew Sass-Kortsak Award: Dr. Tuan Trang Jun 20 Awards Day Jun 27 Professor Andrew Bush, Pediatric Respirology, Royal Brompton & Harefield NHS Foundation Trust Antenatal and childhood origins of COPD faculty TEACHING/EDUCATION AWARDS Dr. Sheila Jacobson R.H.A. Haslam Preclerkship Teaching Award to the faculty member who has demonstrated excellence in preclerkship teaching Dr. Heather Yang and The Lionel Weinstein Clerkship Teaching Dr. Beverley Kupfert Award to the faculty member who has demonstrated excellence in clerkship teaching Dr. Jeff Weisbrot The Undergraduate Community Teaching Award in recognition of excellence in the teaching of medical students within community-based hospitals and offices Dr. Briseida Mema Harry Bain Award to a full-time member of the Department of Paediatrics who has demonstrated excellence in teaching as nominated by core paediatric residents Dr. Douglas Campbell Marvin Gerstein Award to a part-time member of the Department of Paediatrics who has demonstrated excellence in teaching as nominated by core paediatric residents Dr. Tony Barozzino Postgraduate Community Teaching Award in recognition of excellence in the teaching of paediatric residents within communitybased hospitals and offices Dr. Walid Farhat and PERLs Award (Paediatric Educational Dr. Martin Koyle Resident Lecture Series Award) to the faculty member who has demonstrated commitment to excellence in teaching at the Paediatric academic sessions Dr. Jill Hamilton Subspecialty Teaching Award to the faculty member who has demonstrated commitment to excellence in teaching the subspecialty residents and clinical fellows Dr. Wendy Roberts Continuing Medical Education Award to the faculty member who has demonstrated excellence in course coordination or teaching in a Department of Paediatrics/ University of Toronto Continuing Education sponsored event faculty CLINICAL AWARDS Dr. Katherine Verbeeten The Saunders Prize to a senior resident who has demonstrated excellence in clinical paediatrics. research AWARD core paediatrics Dr. Michael Khoury research AWARD JUNIOR TRAINEE CLINICAL SCIENCE Kathleen Nelligan research AWARD SENIOR TRAINEE CLINICAL SCIENCE Dr. Michelle Batthish Dr. Alisha Kassam Dr. Trisha Tulloch research AWARD junior TRAINEE basic SCIENCE Ziad Al Adham research AWARD senior TRAINEE basic SCIENCE Mobin Jamal PAEDIATRIC ADMINISTRATIVE AWARD Rozmin Visram In recognition of an administrative support staff member in the Department of Paediatrics who has demonstrated outstanding performance and excellence in all aspects of their role. This individual possesses outstanding communication and interpersonal skills and is motivated to perform above and beyond their daily responsibilities. This award was first awarded in Resident and Fellow Awards and Achievements TEACHING/EDUCATION AWARDS Dr. Sanjukta Basak (PGY3) Christopher Ondaatje Award to a senior Dr. Melissa Chan (PGY4) resident for excellence in clinical teaching Dr. Charles Larson (PGY4) as nominated by core paediatric residents. Dr. Ramses CanMEDS Award Subspecialty Trainee Badilla-Porras Dr. Jennifer Harrington Dr. Melanie Penner PeRLS Teaching Award 202 the hospital for sick children department of paediatrics Annual Report

103 promotions Medical Education Training & Scholarship Congratulations to the 10 members of our faculty promoted to Full (five) and Associate (also five) Professor in 2012: Full Professors Brenda Banwell Jeremy Friedman Irena Nulman Rayfel Schneider Rae Yeung Neurology Paediatric Medicine Clinical Pharmacology Rheumatology Rheumatology associate Professors Kathy Boutis Paediatric Emergency Medicine Eyal Cohen Paediatric Medicine Roberto Mendoza Clinical and Metabolic Genetics Jennifer Russel Cardiology Uri Tabori Haematology/Oncology And a huge thank you once again to Mel Freedman and all of the members of the Departmental Promotions Committee for their sterling work. Education is one of the core strengths of the Department of Paediatrics, University of Toronto. This report serves to recognize the dedication of our teachers and educators. It documents their achievements and outlines promising new directions for research, innovation, teaching and development in medical education. ORGANIZATION Educational activities in the Department of Paediatrics are led by Dr. Rayfel Schneider, the Associate Chair (Education) and the Medical Education Advisory Committee (MEAC) (see org. chart below). The MEAC works to enhance excellence in teaching and education and increase the recognition of the educational achievement of trainees and faculty. The MEAC participates in career development activities for faculty. It also serves to establish mentorship opportunities for faculty engaged in educational activities, to foster collaborations and networks with other groups interested in education and to provide advice and guidance to the Chair with regard to educational priorities. Our department works closely with the Learning Institute and the education scientists who work there. This year we welcomed Dr. Jonathan Kronick as the new Chief of Education for the Learning Institute and we look forward to both continued and new initiatives under his leadership. We also enjoy close relationships with the other education centres at the university, such as the Wilson Centre for Research in Education, the Centre for Faculty Development (CFD) and the Ontario Institute for Studies in Education (OISE). Details about some of our ongoing collaborations with these centres/groups can be found in the body of this report. Chair Denis Daneman MEAC Associate Chair (Education) Rayfel Schneider Undergraduate Education Stacey Bernstein Postgraduate Education Adelle Atkinson Subspecialty Education Program Directors Continuing Education Suzan Schneewiess Scholarship/ Research Tina Martimianakis Faculty Development Shelly Weiss 204 the hospital for sick children department of paediatrics Annual Report

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Child Life and Health appeals to me because of its with Edinburgh and because of its implied philosophy?but even so, it not Child Health has the attraction of brevity and directwholly satisfying.. Indeed I would suggest that the time has come when we ?uld exercise care in depicting the study and practice of child health as a speciality.. think not, if the total work of those engaged in the care of child life is to be presented in its true perspective to our professional colleagues and to the general public.. perspective is vital to successful paediatric care?and I think it is urgently sOt collectively we should spare no effort in making accelerated large-scale in1' provement of housing conditions the spearhead of progress in child care.. Failure to do so is to ignore global influences which will witho11 question one day have the most far-reaching repercussions on your work and mine' o'1 on the work of those who follow us; on the care of child life in this country; and the standards of child health throughout the world.

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Consultant Paediatrician, United Bristol Hospitals and Bath Clinical Area; Shaw Lecturer in Diseases of Children, University of Bristol The City of Bath has known many famous men.. j Malthus played a vital part in Darwin's theory of evolution.. He described the of populations to increase faster than their food supply.. subject, because this year is the centenary of Darwin's The Origin of Species I been stimulated to consider paediatrics from the evolutionary aspect.. f Before I go on to discuss paediatrics in terms of evolution let me first briefly the Malthusian theme into our own day.. It is estimated that in Biblical times population of the whole world was about the same as is that of the United King^ Last year the world population increased by approximately the same nufl1 c now.. It might eventually be solved by control", as it has been called.. Will these methods solve the problem, or merely postpone its solution^ j An eminent scientist recently suggested that the population problem is likely solved catastrophically.. Species or sub-species adapt themselves to their environing they adapt or perish.. I am going to apply this tentatively to paediatrics, which prtI1i however, that neither adaptability norterm for the primitive buildings in 1Vory towers?though this is hardly an apt manY us work.. We are all w^th children.. There will, of course, be new diseases.. Having seen children of school-leaving age already set of artificial teeth, and learning from my colleagues' pointed rert|arj?. complete at ?ne ex obesity in adults shortens life, I mention concentrated carbohydrates as I fa mP'e ?f the excess diseases against which man may need to be protected.. ^at one can foresee a Brave New World in which the Malthusian problem Mil exist.

Examination paediatrics wayne harris pdf free download bb84b2e1ba examination paediatrics wayne harris pdf free 181 nvidia geforce4 420 go windows 7 driver 12 ati pharmacology proctored test bank for pn 16 The CompTIA Linux+/LPIC-1 Training and Exam Preparation Guide, First Edition is a comprehensive resource designed and written with one fundamental goal in mind: teach […]

Examination paediatrics wayne harris pdf free download. bb84b2e1ba examination paediatrics wayne harris pdf free 181 nvidia geforce4 420 go windows 7 driver 12 ati pharmacology proctored test bank for pn 16. The CompTIA Linux+/LPIC-1 Training and Exam Preparation Guide, First Edition is a comprehensive resource designed and written with one fundamental goal in mind: teach Linux in an easy and practical. A practical study guide to help candidates pass clinical examinations in paediatrics, particularly at postgraduate level.. To find more books about examination paediatrics wayne harris, you can use related keywords : Examination Paediatrics Wayne Harris Pdf, examination paediatrics wayne harris, Examination Paediatrics Wayne Harris Free Download, Examination Paediatrics Wayne Harris Pdf Free Download, wayne harris paediatrics, Examination Pediatrics Wayne Harris. Examination Paediatrics is an essential guide for medical trainees undertaking paediatric clinical examinations.. Aligned with current practice and examination format, this new edition provides guid…. Click Download or Read Online button to get examination-paediatrics book now.. This book is written for candidates preparing for the Fellowship Examination of the Royal Australasian College of. Examination Paediatrics, 4th Edition By (author): Wayne Harris MBBS MRCP(UK) FRACP A practical study guide to help candidates pass clinical examinations in paediatrics, particularly at postgraduate level Examination Paediatrics, 4th Edition is written for…. Examination Paediatrics 5th Edition

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Nelson Essentials Of Pediatrics E Book written by Karen Marcdante and has been published by Elsevier Health Sciences this book supported file pdf, txt, epub, kindle and other format this book has been release on 2014-02-25 with Medical categories.. The resource of choice for pediatric residencies, clerkships, and exams, Nelson Essentials of Pediatrics continues to provide a focused overview of the core knowledge in pediatrics.. Succinct, targeted coverage of normal childhood growth and development, as well as the diagnosis, management, and prevention of common pediatric diseases and disorders, make this an ideal medical reference book for students, pediatric residents, nurse practitioners, and physician assistants.. Nelson Essentials Of Pediatrics E Book written by O P Misra and has been published by Elsevier Health Sciences this book supported file pdf, txt, epub, kindle and other format this book has been release on 2016-09-16 with Medical categories.. Succinct, targeted coverage of normal childhood growth and development, as well as the diagnosis, management, and prevention of common pediatric diseases and disorders, make this an ideal medical reference book for students, pediatric residents, nurse practitioners, and physician assistants.. Nelson Textbook Of Pediatrics E Book written by Robert M. Kliegman and has been published by Elsevier Health Sciences this book supported file pdf, txt, epub, kindle and other format this book has been release on 2015-04-17 with Medical categories.. Embracing the new advances in science as well as the time-honored art of pediatric practice, this classic reference provides the essential information that practitioners and other care providers involved in pediatric health care throughout the world need to understand to effectively address the enormous range of biologic, psychologic, and social problems that our children and youth may face.. Nelson Essentials Of Pediatrics written by Karen Marcdante and has been published by Elsevier this book supported file pdf, txt, epub, kindle and other format this book has been release on 2022-03-07 with Medical categories.. Wong S Essentials Of Pediatric Nursing E Book written by Marilyn J. Hockenberry and has been published by Elsevier Health Sciences this book supported file pdf, txt, epub, kindle and other format this book has been release on 2021-03-05 with Medical categories.. As the #1 title in the pediatric nursing market for over 40 years, Wong's Essentials of Pediatric Nursing, 11th Edition continues to leverage its trademark developmental approach as it equips readers with the very latest research and guidelines for treating children today.. This new eleventh edition also features Next Generation NCLEX®-style case studies and questions, a new chapter covering all systems cancers, additional case studies within the nursing care plans, and updated and expanded evidence-based content throughout to best reflect the latest standards and trends impacting pediatric nursing care today.. Nelson Essentials Of Pediatrics written by Karen Marcdante and has been published by Elsevier Health Sciences this book supported file pdf, txt, epub, kindle and other format this book has been release on 2010-03-31 with Medical categories.. Considered the resource of choice for pediatric residencies, clerkships, and exams, Nelson Essentials of Pediatrics provides comprehensive, yet concise and accessible guidance on normal childhood growth and development, as well as the diagnosis, management, and prevention of common pediatric diseases and disorders.. Wong S Essentials Of Pediatric Nursing E Book written by David Wilson and has been published by Elsevier Health Sciences this book supported file pdf, txt, epub, kindle and other format this book has been release on 2013-12-27 with Medical categories.. Family focus includes a separate chapter on the role of the family in child health, family content throughout the text, and Family-Centered Care boxes that highlight information on patient teaching, home care, and incorporating the family in the child's care.

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DNS. • Puffer (10x) - 5µl • dNTP mix (2,5 mM) - 5µl • MgCl2 - (25mM )- 5µl • Enzim - 0,5 µl • S primer (10pmol/µl) - 1µl • AS Primer (10pmol/µl) - 1µl • DNS - 1µl • H2O - 50 µl-ig. Real Time PCR Real-time PCR során a reakció során keletkező fluoreszcenciát detektáljuk.. • Enzim mix • S primer • AS Primer • DNS • H2O. Real Time PCR Hibridizációs próba (FRET) Az egyik próba a 3’ végén egy donor a másik az 5’ végén egy akceptor fluorochrommal jelölt.. Primer Melting Temperature (Tm): primers with melting temperatures in the range of 52-58 °C generally produce the best results.

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