SickKids Healthier Children. A Better World.™ Department of Paediatrics Annual Report 2007–08 “A child’s life is like a piece of paper on which every passerby leaves a mark.” - Chinese proverb Contents 2 Chair’s Report 6 Division of Adolescent Medicine 14 Division of Cardiology 26 Division of Clinical and Metabolic Genetics 34 Division of Clinical Pharmacology & Toxicology 46 Division of Developmental Paediatrics 54 Division of Endocrinology 62 Division of Gastroenterology, Hepatology and Nutrition 74 Division of Haematology/Oncology 90 Division of Immunology/Allergy 96 Division of Infectious Diseases 104 Division of Neonatology 116 Division of Nephrology 122 Division of Neurology 134 Division of Paediatric Emergency Medicine 140 Division of Paediatric Medicine 148 Division of Respiratory Medicine 156 Division of Rheumatology 164 Bloorview Kids Rehab 166 Transplant Centre 168 Paediatric Housestaff 170 Paediatric Grand Rounds 173 Promotions/Recognition 174 Awards 175 Resident and Fellow Awards and Achievements 178 Annual Resident and Fellow Research Day Chair’s Report 2007-08 was a very special year in the Department of Paediatrics at the The Farid Mahmud (Endocrinology), Mahendra Moharir (Neurology) and Rulan Parekh Hospital for Sick Children and University of Toronto: one, not only of security, (Nephrology). This group represents the full spectrum of job profiles: clinician but also about renewal, growth and development, innovation and recognition. scientists, investigators, teachers, educators and specialists. The department and hospital will be well served for many years to come by this highly talented group. Security was present in our funding plan which has underpinned our ability to recruit new, and retain the existing, outstanding faculty. Last year our ranks were There have been and will also be more transitions in the months and year to come. enormously enriched by the appointment of new Division Heads in Endocrinology Searches are underway in at least three divisions for new heads. At the Paediatric (Mark Palmert), Developmental Paediatrics (Darcy Fehlings), Rheumatology (Brian Executive level, Rayfel Schneider has seamlessly taken over as Associate Chair of Feldman) and Gastroenterology, Hepatology and Nutrition (Anne Griffiths), as well Medical Education and his team has already garnered some impressive awards: as David Grant as the new Director of the SickKids Transplant Centre. In addition, 2 we have been able to build by recruiting to most divisions: Aleixo Muise Adelle Atkinson (Director of Postgraduate Medical Education), the PAIRO Residency (Gastroenterology/Hepatology/Nutrition), Sarah Alexander and Angela Punnett Excellence Award for our program in Paediatrics; Stacey Bernstein (Director of (Haematology/Oncology), Anu Wadhwa (Infectious Diseases), Luc Mertens and Lars Undergraduate Medical Education), a W.T. Aikens Award for small group teaching; Gross-Wortmann (Cardiology), Sarah Bowdin (Clinical Genetics), Chris Tomlinson Karen Leslie (Adolescent Medicine), the Helen Batty Award for Faculty Development; and Linh Ly (Neonatology), Theo Moraes (Respiratory Medicine), Yaron Finkelstein, Jonathan Pirie (Emergency Medicine) and Ben Saxon (Haematology/Oncology) Sanjay Mehta and Trent Mizzi (Emergency Medicine), Zia Bizmilla (Paediatric awards for excellence in postgraduate education; and Anna Banerji (Infectious Medicine), Angela Orsino and Evdokia Anagnostou (Developmental Paediatrics), Diseases), the Educational Excellence in Community Care Award. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT These awards are, I believe, a direct result of the commitment we have made Besides the plethora of awards in education, there are also many examples as a department to excellence in education. The addition of an education scholar of achievement in research and clinical care. In 2005-06, the year for which to the department in early 2009 will further enhance our commitments, as will we have the latest figures, the department ranked second amongst clinical our ongoing support of faculty development in education. More challenges await departments in the Faculty of Medicine in the amount of external grant funding: as we expand the size of the Medical School classes and move to an even more over $41 million! Annually, we publish over 750 papers, our faculty present at distributed model of education. In the meantime, we continue to increase the size all the major meetings: plenary sessions, workshops and original research. The of our residency program and our fellowship trainees. Each year, the group coming culture of research, as has been planned, permeates all aspects of our activities. to our program seems more talented than ever before. The theme of the photos New team grants have been awarded by the CIHR recently in asthma (PJ Subbarao for this annual report is, quite appropriately, Commitment to Education. and colleagues) and obesity (Brian McCrindle, Jill Hamilton and their extensive After seven years as Associate Chair of Research, Norman Rosenblum is taking and Meredith Irwin (Haematology/Oncology), Anne Opavsky (Infectious Diseases) on a new and exciting challenge: Director of Clinician-Scientist Training Programs and Rae Yeung (Rheumatology). Berge Minassian has been recognized nationally in the Faculty of Medicine at the University of Toronto. Norm has made major and internationally for his groundbreaking work in epilepsy, and David Malkin has contributions to the development of our department’s research efforts and been feted by the Israel Cancer Research Foundation as a Man of Distinction. group), and personal awards to Bob Jankov (Neonatology), Uri Tabori, Lillian Sung deserves our enormous thanks and best wishes. Meredith Irwin (Haematology/ Oncology) will take over this role and I have no doubt will bring her enormous The Resident Research Day in May 2008 was once more a great success with energy, enthusiasm and her own stamp to the task. over 110 oral and poster presentations by our trainees. This year’s keynote speaker, David Dunger, Professor of Pediatrics from Cambridge University, U.K., We thank also Seonag Macrae, now SickKids Executive Vice-President, for so ably wonderfully illustrated the development and evolution of a career in clinical representing the Child Health Services portfolio on the Paediatric Executive from research. The winners of the resident awards are listed on page 175. 2003 to 2008, and welcome her replacement, Marilyn Monk, who is already actively engaged in numerous issues of mutual importance to the hospital and department. Clinical innovations and excellence abound. Just a few examples: Eyal Cohen and colleagues are following the legacy of Dr. Norman Saunders in pursuing Once again, the Department of Paediatrics performed superbly in the annual new models for the care of children with complex care needs; Andrew Redington Faculty of Medicine promotions process. Debra Katzman (Adolescent Medicine) and his group in Cardiology and the Labatt Family Heart Centre are breaking new is now professor, and the group of 11 listed on page 173, are all now associate ground in the Cardiac Diagnostic and Interventional Unit (CDIU) which opened last professors: this is a richly deserved recognition of their academic achievements. year; Jill Hamilton and an interdisciplinary group are probing new approaches to the management of hypothalamic obesity; Sylvain Baruchel is testing new cancer therapies in Phase I clinical trials, and the entire Oncology group participates in the relentless success of the Children’s Oncology Group, as they continuously improve the outcomes of childhood cancers. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 3 A couple of new endowed chairs were announced in the past year: Andrew Redington academic and community positions. Second, we are now in a period of growth in has been awarded the BMO Financial Group Chair in Cardiology and Earl Silverman, the health-care sector. How long can this be sustained? Third, our capabilities in Rheumatology, the Ho Family Chair in Autoimmune Diseases. On page 174, we list all three pillars of academia have long since outstripped the capacity of our physical all the winners of the departmental awards-heartiest congratulations to all. plant: things are going to be quite tight until the new buildings are completed. Fourth, our training programs are being pulled in a number of different directions, including We have initiated two new programs within the department: first, the transition the need to train more Canadians in a number of subspecialties, as well as meeting position aimed at senior trainees wishing to spend one to two more years honing our social responsibilities by helping to train paediatric subspecialists who return to their academic skills before assuming a faculty position, and second, a faculty the developing world. Finally, we need to pay increasingly close attention to clinical development program that encourages the members of our department to spend care, focussing on excellence, access, safety and innovation. periods of time away from SickKids and the University of Toronto, acquiring new skills which they can bring back to enrich their own careers as well as our activities. In closing, it continues to be an honour and joy to serve as the chair/chief of this department. I am given daily reminders, not only at home but everywhere I go in the But we are not this successful in isolation. The support of the administration at world, of just how accomplished our faculty is. Thanks to all for your wonderful work SickKids and the Decanal Office in the Faculty of Medicine is essential to all of on behalf of the department, hospital, and university, but mostly for the children our activities. Mary Jo Haddad, SickKids President and CEO, and Cathy Whiteside, and their families who entrust us with their lives. U of T Dean of Medicine, remain staunch supporters of our department. I cannot overstate our appreciation for the efforts of Mary Jo, Cathy and their teams. So is everything rosy, or are there challenges that need our attention? The past five years have seen enormous new recruitment to our faculty. The pace of growth is very unlikely to continue indefinitely; in fact, the job market may become overheated with a limit on new academic positions for highly trained individuals. There is a need for an ongoing nationwide dialogue on the training of paediatricians for both 4 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT DENIS DANEMAN, MB, BCh, FRCPC Chair of Paediatrics, University of Toronto Paediatrician-in-Chief, The Hospital for Sick Children R.S. McLaughlin Chair in Paediatrics, at The Hospital for Sick Children Thanks to all for your wonderful work on behalf of the department, hospital, and university, but mostly for the children and their families who entrust us with their lives. - Dr. Denis Daneman Dr. Miriam Kaufman DIVISION HEAD Dr. Debra K. Katzman FULL-TIME Dr. Eudice Goldberg Dr. Miriam Kaufman Dr. Karen Leslie Dr. Cathleen Steinegger 6 PART-TIME Dr. Miriam Rossi Dr. Rahul Saxena Dr. Boonying Manaboriboon Dr. Ellie Vyver Dr. Mona Basker CLINICAL FELLOWS Dr. Rosawan Areemit Dr. Abigail Harrison-Kong Dr. Nuray Kanbur ADMINISTRATIVE STAFF Sharon Dolphin Melissa Keown Burnett Wint THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Division of Adolescent Medicine OVERVIEW The goal of the Good 2 Go Transition Program is to The team continues to collaborate with programs/ The Division of Adolescent Medicine provides prepare all youth with chronic health conditions to clinics within the hospital and with community state-of-the-art, comprehensive specialty care to leave SickKids by the age of 18 with the necessary skills partners. The Transition Program offers supervision patients up to 18 years of age, focusing its clinical and knowledge to advocate for themselves, maintain and mentorship to interdisciplinary trainees in the activities in five program areas: eating disorders, health-promoting behaviours and utilize adult area of transition. transition (Good 2 Go), substance abuse, young health-care services appropriately and successfully. The Transition Program’s poster presentation, families and complex adolescent problems. The Eating Disorders Program at SickKids continues The team continues to develop and customize new The Good 2 Go Transition Program: Helping Programs resources, including colourful clinic posters and Promote Healthy Transitions from the Paediatric to to provide a variety of integrated clinical services timelines to encourage patients, families and the Adult Health-Care Systems, was awarded best (inpatient, day treatment, outpatient and consultation) practitioners to begin preparing for transition to adult poster at the Interprofessional Practice Symposium to adolescents and their families. All aspects of the care. MyHealth Passport, an initiative introduced last held at SickKids in November 2007. In addition, the program are multidisciplinary and family-based, year, is being evaluated and used in a research project Good 2 Go Transition Program was recognized by the allowing for the flexibility in the program to best meet in the Inflammatory Bowel Disease Clinic. 2007 accreditation team as one of five leading practices in the hospital. the particular needs of each individual adolescent. New initiatives have included the integration of The team has recently received funding from the SickKids multi-family group therapy (MFGT) at SickKids and Foundation to develop clinical pathways for youth, ages The Adolescent Substance Abuse Outreach Program throughout the province. MFGT was recognized by 12 to 18 years. The pathways will be used at clinic visits and the Substance Abuse Day Treatment Program the 2007 accreditation team as one of five leading throughout the hospital, ensuring that staff are offer comprehensive clinical care to adolescents practices in the hospital. The highly successful consistently aware of their role in facilitating transition. and their families with substance abuse problems. Ontario Community Outreach Program (a collaborative The team also received funding from the Kidney The spectrum of services includes both outpatient of SickKids, University Health Network and the Foundation of Canada to work collaboratively with staff services as well as more intensive, five days a week MOHLTC) continues to train health-care professionals from the University Health Network to explore the best day treatment. Patients receive health assessments around the province to assess and treat eating way to modify an existing adult self-management program and follow up that address both the medical sequelae disorders, thus allowing more patients to receive for use with youth who have chronic health conditions. of their substance use, as well as the other medical care in their own community. concerns that result from high-risk health behaviours. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 7 to reduce high-risk health behaviours by promoting The Day Treatment Program expanded its services last both at local and regional levels, and provide year to include a summer program, combining expertise in child and youth substance use and abuse healthy sexuality and healthy sexual relationships; treatment, academic and medical support to day to various committees dealing with prevention and assistance to health-care teams in recognizing the treatment patients. This summer’s program will treatment issues at the municipal, provincial, and role of the mind and body regarding various health- build upon the success of last year’s experience. national levels. Expertise in the area of substance care processes; and maintaining a presence within abuse prevention has been highlighted this year the community to advocate for the health-care The program also provides training and education through work with the City of Toronto task force needs of youth. These services are provided in the to many levels of learners from different health and working group on substance abuse prevention. Adolescent Clinic as well as in collaboration with renal, cardiac and lung transplant programs, the HIV and professions, including postgraduate Paediatrics and SLE clinics, and through inpatient consultations. Last Psychiatry, and child and youth counselling trainees. The Substance Abuse Program is building on prior New training initiatives for Adolescent Medicine collaboration with the Centre for Addiction and Mental year, a combined CAPP/SCAN (Suspected Child Abuse fellows that were implemented last year have been Health for a project that will pilot screening and and Neglect) clinic was established to collaboratively further enhanced. As well, specialized training in assessment tools for youth with substance abuse, assess and provide education and support for substance use and abuse for paediatric residents other high-risk behaviours and concurrent disorders. adolescents identified as engaging in high-risk sexual behaviours. This past year, the CAPP clinic created a has been launched with the creation of a new interdisciplinary clinic specifically addressing the The Complex Adolescent Problem Program (CAPP) new link to the Substance Abuse Treatment Program complex needs of patients in the Substance Abuse continues to focus on adolescents with chronic illness, allowing youth in the Substance Abuse Program to Program. In addition to the many outreach educational disability or complicated medical conditions. The goal receive their medical care in an interdisciplinary clinic activities of the program, staff presented at national of CAPP is to provide access to expert adolescent involving team members from both programs. CAPP and international conferences this year at the invitation health-care services to youth, both within the continues to build partnerships with local agencies of leaders in the fields of paediatric health-care and community and those already patients at SickKids. in an effort to enable youth in the downtown core congenital heart disease. Members of the staff are Objectives of the program include provision of to access health information and necessary health involved in many aspects of service development, comprehensive multidisciplinary care in an effort services otherwise unavailable to them. In addition, collaboration with the Toronto Children’s Aid Society 8 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT for youth in care to access medical and psychosocial Assessment and intervention in the areas of growth activities include the medical complications of eating services. Increasingly, the CAPP team is being and development, infant and adolescent mental health, disorders with specific emphasis on osteoporosis and consulted regarding youth with clinical conditions maltreatment, and social determinants of health brain structure and function, epidemiology of early- that are considered diagnostic dilemmas. provide the basis for research in adolescent parenting. onset eating disorders, evaluation of family-based Management of these conditions requires an The project is now completing the assessment phase treatments for adolescents with eating disorders, interdisciplinary team skilled in understanding and is entering the intervention phase with an emphasis the evaluation of the use of information technology the interplay of physical and emotional factors on maternal and infant mental health, and ultimately for health promotion with youth, treatment outcome that contribute to presenting symptoms. improving child/parent relationships. Interprofessional in adolescent substance use, HIV educational team members continue to provide resources and interventions with incarcerated youth, health values The Young Families Program (YFP) focuses on providing education to health professionals in a variety of in adolescents with chronic illnesses, the assessment health care, advocacy, support and education to settings through conference presentations and and evaluation of high risk parents and their children, high-risk adolescent parents and their children. The committee participation. the evaluation of transition of care to the adult healthcare system, and faculty development and mentoring. YFP collaborates with several agencies that provide services to a diverse group of adolescent parents, The Division of Adolescent Medicine at SickKids is including prenatal homes, health services and child the largest of its kind in Canada, with a diverse The division trains physicians (from Paediatrics, protection agencies. In particular, the YFP provides a interprofessional clinical team and staff. This past Psychiatry and Family Medicine), nurses, psychologists, consultation clinic at Jessie’s Centre for Teenagers year, two nurses in Adolescent Medicine successfully social workers, dietitians, art therapists and child and provides educational, supportive and policy passed the newly instituted nurse practitioner youth counsellors in all aspects of adolescent health. recommendations. In partnership with the SCAN team, examination and are now registered with the College the High Risk Project has been operational since of Nurses of Ontario as nurse practitioners. Members of the division continue to make significant educational contributions locally, provincially, nationally 2004 when external funding was established to standardize clinical care for adolescent parents Research activities are an integral part of the activities and internationally. Members of the division hold and their children. of the faculty and trainees of the division. The division leadership roles in the SickKids Summer Student has significantly expanded its research, addressing Program, the Centre for Faculty Development, and a multitude of adolescent health issues. Research organizing international annual scientific meetings on adolescent health. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 9 Division members play a leading role in national PUBLICATIONS and international clinical, education and research Bravender T, Bryant-Waugh R, Herzog D, Katzman D, Pinzon J, McDuff P: Sex and sexual health: A survey committees, including the Infant Mental Health Kreipe RD, Lask B, Le Grange D, Lock J, Loeb K, of Canadian youth and mothers. Paediatrics and Child Health 2008: 13(1): pp 25-30. Frappier J, Kaufman M, Baltzer F, Elliott A, Lane M, Promotion Project, Canadian Paediatric Society, Madden S, Nicholls D, O’Toole J, Pinhas L, Rome E, American Academy of Paediatrics, Society for Sokol-Burger M, Wallen U, Zucker N: workgroup for Adolescent Medicine, Canadian Association for classification of eating disorders in children and Infectious Diseases and Immunization Committee Adolescent Health, Canadian Association for Medical adolescents. Classification of child and adolescent (Bortolussi R, Moore DL, Robinson JL, Rousseau- Education and the Academy for Eating Disorders. eating disturbances. Workgroup for classification Harsany E, Samson LM (Principal Author) and of eating disorders in children and adolescents Adolescent Medicine Committee (Findlay S, Pinzon J, Over the past three years, the division has taken the (WCEDCA). International Journal of Eating Disorders Taddeo D, Katzman DK), Canadian Paediatric Society lead in pursuing paediatric subspecialty certification 2007: 40(Suppl): pp S117-S122. (CPS): HPV vaccine for children and adolescents. Paediatrics and Child Health 2007: 12(7): pp 599-603. for Adolescent Medicine through the Royal College of Physicians and Surgeons of Canada (RCPSC). Egan-Lee E, Hollenberg E, Dematteo D, Tobin S, Adolescent Medicine was endorsed at the RCPSC’s Oandasan I, Beduz A, Kwan D, Leslie K, Lee J, Jayaraman GC, Shields S, Rossi M, Haase D, Singh A, Council in May 2007, and is now the 63rd recognized Tassone M, Merkley J, Lingard L, Sinclair L, Lowe M, Predy G, Neudorf C, Ogilvie G, Rekart M, Beaudoin C, Reeves S: Catalyzing and sustaining communities Hammond G, Toye B, Wong T and the SY-Track Study of collaboration around interprofessional care: An Team: Street youth in Canada: findings from enhanced HONOURS AND AWARDS evaluation of four educational programs. Journal of surveillance of Canadian street youth, 1999-2003. Katzman DK: Eating Disorder Champion Award, 2007. Interprofessional Care 2008: 22(3): pp 317-319. Ottawa, ON: Public Health Agency of Canada, 2006. discipline of the RCPSC as a subspecialty of Paediatrics. Cat. No. HP5-15/2006 ISBN 0-662-49069-X. Leslie K: Helen Batty Award for Teaching in Faculty Development, 2008. 10 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Accessed March 5, 2007. Misra M, Prabhakaran R, Miller KK, Goldstein A, Katzman DK, Frappier JY, Goldberg E: Adolescent Leslie K: Youth substance use and abuse: challenges medicine: a new paediatric subspecialty in Canada. and strategies for identification and intervention. Mickley D, Clauss L, Lockhart P, Cord J, Herzog DB, (Invited submission). Paediatrics and Child Health Canadian Medical Association Journal 2008: 178(2): Katzman DK, Klibanski A: Weight gain and restoration 2008: 13(1): pp 12-14. pp 145-148. of menses as predictors of bone mineral density Katzman DK: Irregular menses: a warning sign of Leslie K: Harm reduction: an approach to reducing Journal of Clinical Endocrinology and Metabolism vomiting for weight control. Journal of Adolescent risky health behaviours in adolescents. Paediatrics 2008: 93: pp 1231-1237. Health 2008: 42(5): pp 429-431. and Child Health 2008: 13(1): pp 53-56. change in adolescent girls with anorexia nervosa-1. Richardson D, Glover TS, Nasmith L, Leslie K, Kirsh G, McVey G, Tweed S, Katzman DK: Misra M, Miller KK, Cord J, Prabhakaran R, Bandiera G, Paterson M, Power B, Oandason I, Frank Psychosocial profiles of young adolescent females Goldstein M, Katzman DK, Herzog DB, Klibanski A: JR: The CanMEDS train-the-trainer collaborator faculty seeking treatment for an eating disorder. Journal of Relationships between serum adipokines, insulin development program. Ottawa: The Royal College of levels and bone density in girls with anorexia nervosa. Physicians and Surgeons of Canada 2007. Adolescent Health 2007: 40(4): pp 351-356. Journal of Clinical Endocrinology and Metabolism Korszak D, Connolly B, Baron T, Katzman DK, 2007: 92(6): pp 2046-2052. Steinegger C, Katzman DK: Interviewing the adolescent with an eating disorder. Adolescent Bernstein S: Safety and efficacy of image-guided gastrostomy (G) and gastrojejunostomy (GJ) tubes Misra M, Prabhakaran R, Miller KK, Goldstein MA, Medicine State of the Art Reviews 2008: 19(1): in children and adolescents with primary psychiatric Mickley D, Clauss L, Lockhart P, Cord J, Herzog DB, pp 18-40. illness. International Journal of Eating Disorders Katzman DK, Klibanski A: Prognostic indicators of 2007: 40(7): pp 645-651. changes in bone density measures in adolescent Tallett S, Lingard L, Hellmann J, Hilliard R, Hurley J, girls with anorexia nervosa. Journal of Clinical Leslie K, Jefferies A, Mian M, Pirie J, Rosenfield J, Endocrinology and Metabolism 2008: 93(4): Schneider R, Spero L: Measuring educational pp 1292-1297. workload: a pilot study of paper-based and PDA tools. Medical Teacher 2008: 30(3): pp 296-301. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 11 FUNDING Vyver E, Steinnegger C, Katzman DK: Eating disorders Pinhas L, Katzman DK, Dimitropoulos G, Woodside B: and menstrual dysfunction in adolescents. Annals Binging and bulimia nervosa in children and adolescents. Bulimic eating disorders in children and adolescents: of the New York Academy of Sciences 2008: 1135: In: Child and Adolescent Eating Disorders (Jaffa T, Canadian Paediatric Surveillance Program (CPSP). pp 253-264. McDermott, eds.). New York: Cambridge University Pinhas L, Heinmaa M, Boachie A, Crosby R, Katzman Press, 2007. DK (Senior Investigator). Psychiatric Endowment BOOKS AND BOOK CHAPTERS Fund at SickKids ($24,000, 2007-2008). Adolescent Health Care, A Practical Guide, 5th Edition Pinhas L, Steinegger C, Katzman DK: Clinical (Neinstein LS, Gordon CM, Katzman DK, Rosen DS, assessment and physical complications. In: Development and testing of a self-management Woods ER, eds.). Philadelphia: Lippincott Williams Eating Disorders in Childhood and Adolescence, program for youth post kidney transplant: a Paediatric and Wilkins, 2007. 3rd Edition (Lask B, Bryant-Waugh R, eds.). and adult care collaboration. Kidney Foundation London: Brunner-Routledge, 2007. of Canada Grant ($90,000, 2008-2010). Adolescent Health Care LITE (Neinstein LS, Gordon CM, Katzman DK, Rosen DS, Woods ER, eds.). Steinegger C: Nutrition. In: Adolescent Medicine: Development and testing of a self-management Philadelphia: Lippincott Williams and Wilkins, 2007. The Requisites in Pediatrics (Slap GD, ed.). program for youth post kidney transplant: A paediatric Philadelphia: Mosby Inc., 2008. and adult care collaboration. Dettmer E, Cullen-Dean Katzman DK, Golden NE: Eating disorders. In: Adolescent G, D’Agostino N, Kaufman M, Lorber S, Mitchell M, Health Care: A Practical Guide, 5th Edition (Neinstein Schiff J, Pool R. RN Funding Kidney Foundation L, Gordon C, Katzman DK, Rosen D, Woods E, eds.). of Canada ($85,134, 2008). Philadelphia: Lippincott Williams and Wilkins, 2007. 12 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Effects of anorexia nervosa on peak bone mass. Creating transition clinical pathways. Kaufman M, Klibanski A, Katzman DK, Misra M, Herzog D, Cullen-Dean G, Dettmer E, Lorber S, Horricks L. Levitsky L. National Institute of Health/NIDDK Sun Life Financial ($25,000, 2008). ($3,801,660, 2003-2008). Leadership: creating communities of practice for Faculty development to support capacity building interprofessional collaboration (IPC) in Toronto. for interprofessional education. Leslie K, Reeves S, Kwan D, Leslie K, Lieff S, Nasmith L, Oandasan I. Silver I, Merkley J, Richardson D, Wagner S, Andrews Reeves S, Silver I. Ministry of Health and Long-Term M, Kwan D. Ministry of Health and Long-Term Care Care Interprofessional, Mentorship, Preceptorship, ($310,000, 2008). Leadership and Coaching Fund ($1,200,000, 2007). Family therapy and fluoxetine in the treatment of Youth with chronic health conditions: tell us how we adolescent anorexia nervosa. Agras S, Brandt H, can best teach you self-management skills. Mitchell M, Halmi K, Johnson C, Kaye W, Wilfley D, Woodside B, Rivers P, McCurdy C, D’Agostino N, Dettmer E, Pinhas L, Katzman DK. National Institute of Mental Cullen-Dean G, Lorber S, Harrison-Kong A, Kaufman M, Health ($6,250,000 2007-2012). Pool R. Funding UHN Allied Health Research Award ($1,158, 2008). THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 13 Dr. Edgar Jaeggi DIVISION HEAD Dr. Andrew Redington FULL-TIME Dr. Lee Benson Dr. Tim Bradley Dr. Rajiv Chaturvedi Dr. Anne Dipchand Dr. Mark Friedberg Dr. Fraser Golding Dr. Gil Gross 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. Elizabeth Stephenson 14 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT PART-TIME Dr. Tiscar Cavalle-Garrido Dr. Jack Colman Dr. Rejane Dillenburg Dr. Ra Han Dr. Alexandra Jevremovic Dr. Anilkumar Menon Dr. Tapas Mondal Dr. Norman Musewe Dr. Lynne Nield Dr. Erwin Oechslin Dr. Dionissios Pepelassis Dr. Rachel Wald Dr. Roxanne Walker ADJUNCT Dr. Fred Keeley (Research Scientist) CROSS-APPOINTED Dr. Desmond Bohn Dr. Tilman Humpl Dr. Afrothite Kotsakis Division of Cardiology OVERVIEW multi mode research, investigation and treatment, and tissue samples will be held for future fundamental, The Division of Cardiology was formally established in setting the scene for MRI-guided interventional translational and clinical research investigations. the 1940s and is now one of the largest and most procedures and physiologic measurements in children. The Royal College accredited Paediatric Cardiology successful in the world. Its philosophy has remained unchanged since its inception over 60 years ago: to The cardiovascular research focus within the Research Training Program is the largest in Canada and one of provide the highest quality care in an evidence-based Institute has made fundamental contributions to our the largest and most sought after in North America. environment of clinical innovation and scientific discovery. Over the past decade, the divisional activities, in understanding of the cellular biology and molecular As such, its fellows have populated cardiology groups genetics of pulmonary vascular bed, with recent throughout the world, many going on to become diversification to include the study of the fetal myocyte, leaders of our specialty. close collaboration with surgical and intensive care detailed study of the intercellular structure of the heart colleagues, have contributed to our markedly improved and vessels, electrophysiology of the developing heart The single most important development of the last preoperative diagnosis and postoperative outcomes and immunology of cardiac transplantation. While decade, however, has been the establishment of for children undergoing cardiac surgery, led the field undergoing a change in leadership, it is expected that the Labatt Family Heart Centre. Following a series in development of therapeutic catheter techniques, high quality basic and translational research, with an of important internal initiatives, the extraordinary made important contributions to our understanding of emphasis on direct clinical applicability, will continue generosity of the Labatt family has funded the fetal cardiac medicine, established one of the largest to form the bedrock of our academic program. As an redevelopment and future activities of the Heart paediatric cardiac transplantation centres worldwide, example, the Heart Centre is making a major investment Centre at SickKids. and has won international recognition for its contribution in the development of a biobank facility in which DNA 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 Dr. Joel Kirsh and patient 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’ Dr. Steven Schwartz Dr. Ben Sivarajan Dr. Shi-Joon Yoo RESEARCH ASSOCIATES La Rotta, Gustavo Dr. Jing Li CLINICAL FELLOWS Jaime Alkon Abdulmajeed Al-Otay Armando Alfaro-Ramirez Alexandra Bauch Jose Castro-Sancho Robin Clegg Matthew Crystal Yousef Etoom Deborah Fruitman Brian Grant Lars Grosse-Wortmann Damian Hutter Rubeena Khan CLINICAL FELLOWS Anders Nygren Dragos Predescu Mark Walsh Roland Weber RESEARCH FELLOWS SUBSPECIALTY RESIDENTS Christian Apitz Kentaro Asoh Jae-Young Lee Jan Møller Nielsen Lucy Roche Alejandro Floh Marko Vezmar Derek Wong DEPARTMENTAL FELLOW Ricardo Ayala-Arnez The Hospital for Sick Children 2007-08 ANNUAL REPORT 15 HONOURS AND AWARDS Mital S: Roma and Marvin Auerbach Professorship Golding F: Operational Director of Echocardiography, (Endowment) in Developmental Biology and Congenital Mori A, Li J, Liu PP, Redington AN, Coles JC: Early Cardiology, The Hospital for Sick Children. February, Heart Research, 2007. gene expression profiles during intraoperative Redington A: BMO Financial Group Chair in Cardiology, Journal of Thoracic and Cardiovascular Surgery 2007: The Hospital for Sick Children, 2008. 134(1): pp 74-81. Arab S, Konstantinov IE, Boscarino C, Cukerman E, myocardial ischemia-reperfusion in cardiac surgery. 2008. Golding F: Medical Director of Telemedicine, The Hospital for Sick Children. June, 2008. Gross G: Schwartz-Reisman Visiting SickKids Scholarship, The Hospital for Sick Children. 2008. PUBLICATIONS Atz AM, Cohen MS, Sleeper LA, McCrindle BW, Lu M, Akikusa JD, Feldman BM, Gross GJ, Silverman ED, Prakash A, Breitbart RE, Williams RV, Sang CJ, Schneider R: Sinus bradycardia following intravenous Wernovsky G: Functional status of patients with ‘pulse’ methylprednisolone. Pediatrics 2007: 119: Hamilton R: Featured Research, Canadian pp e778-e782. heterotaxy syndrome following the Fontan operation. Cardiology in the Young 2007: 17(2): pp 44-53. Cardiovascular Congress: abnormal cardiomyocyte connexin expression in arrhythmogenic right Akman HO, Sampayo JN, Ross FA, Scott JW, Wilson G, Bernasconi A, Cavalle-Garrido T, Redington AN: ventricular cardiomyopathy caused by heterozygous Benson L, Bruno C, Shanske S, Hardie DG, Dimauro S: Spontaneous intraoperative ventricular hematoma desmosomal protein mutations, 2007. Fatal infantile cardiac glycogenosis with phosphorylase in a neonate. Heart 2007: 93(8): p 898. kinase deficiency and a mutation in the gamma Kantor P: BCCA Leeds 2007 Award for the Best 2-subunit of amp-activated protein kinase. Pediatric Poster Presentation. “Sudden death of heart disease Research 2007: 62(4): pp 499-504. undiagnosed during the lifetime in children: pathology survey and review of the literature.” British Congenital Bernheimer J, Friedberg MK, Chan FP, Silverman NH: Echocardiographic diagnosis of persistent fifth aortic arch. Echocardiography 2007: 24(3): pp 258-262. Alsoufi B, Cai S, Williams WG, Coles JG, Caldarone CA, Cardiac Association (BCCA) Annual Meeting, Leeds, Redington AM, Van Arsdell GS: Improved results with Berul CI, Villafane J, Atkins DL, Cecchin F, Kirsh JA, UK, 2007. single-stage total correction of Taussig-Bing anomaly. Johns JA, Kanter RJ, Karpawich PP: Pacemaker lead European Journal of Cardiothoracic Surgery 2008: prolapse through the pulmonary valve in children. Lee J: Promoted to Associate Professor of Paediatrics, 33(2): pp 244-250. Faculty of Medicine, University of Toronto, 2008. ADMINISTRATIVE STAFF Shazia Ali (contract) Fe Berbano (contract) Theresa Braggs Sandra Brisebois Paula Capela Tracey Clatworthy (contract) Esther Delea Karen Hodgson Susan Jessop Shelley Marks Fran Matthews Shloka Mirchandaney Pacing and Clinical Electrophysiology 2007: 30: pp 1183-1189. Andrea Morris Susan Morton Eleanor Paulino Robin Smith (contract) Rooma Vaidya Denise Vanek Shirley Wang Collins KK, Rhee EK, Kirsh JA, Cannon BA, Fish F, Bharat W, Manlhiot C, McCrindle BW, Pollock-BarZiv Chaturvedi RR, Redington AN: Pulmonary regurgitation SM, Dipchand AI: The profile of renal function over in congenital heart disease. Heart 2007: 93(7): Dubin A, Van Hare GF: Cryoablation of accessory time in a cohort of paediatric heart transplant pp 880-889. pathways in the coronary sinus in young patients: a multicenter study from the paediatric and recipients. Pediatric Transplantation December 18, 2007 [Epub ahead of print]. Cherney D, Miller J, Scholey J, Bradley TJ, Slorach C, congenital electrophysiology society’s working Curtis J, Dekker M, Nasrallah R, Hebert R, Sochett E: group on cryoablation. Journal of Cardiovascular Electrophysiology 2007: 18: pp 592-597. Bograd AJ, Mital S, Schwarzenberger JC, Mosca RS, The effect of cyclooxygenase 2 inhibition on renal Quaegebeur JM, Addonizio LJ, Hsu DT, Chen JM: hemodynamic function in humans with Type 1 Twenty-year experience with heart transplantation for diabetes mellitus. Diabetes 2008: 57(3): pp 688-695. Bernstein D, Williams GE, Eisen H, Mital S, 1986-2006. American Journal of Transplantation Cherney DZ, Scholey JW, Nasrallah R, Dekker MG, Gene expression profiling distinguishes a molecular 2008: 8: pp 2001-2007. Slorach C, Bradley TJ, Hébert RL, Sochett EB, Miller JA. signature for Grade 1B mild acute cellular rejection Wohlgemuth JG, Klingler TM, Fang KC, Kobashigawa J: infants and children with restrictive cardiomyopathy: Renal hemodynamic effect of cyclooxygenase 2 in cardiac allograft recipients. Journal of Heart Bradley TJ, Karamlou T, Kulik A, Mitrovic B, inhibition in young men and women with uncomplicated Transplantation 2007: 26(12): pp 1270-1280. Vigneswaran T, Jaffer S, Glasgow PD, Williams WG, Type 1 diabetes mellitus. American Journal of Van Arsdell GS, McCrindle BW: Determinants of repair Physiology–Renal Physiology 2008: 294(6): type, reintervention, and mortality in 393 children with pp F1336-F1341. Davies RR, Russo MJ, Mital S, Martens TM, Sorabella RS, Hong KN, Gelijins AC, Moskowitz AJ, Quaegebeur JM, Mosca RS, Chen JM: Predicting survival among double outlet right ventricle. Journal of Thoracic and Collins N, Colman J, Benson L, Hansen M, Merchant N, high-risk paediatric cardiac transplant recipients: Horlick E: Successful percutaneous treatment of an analysis of the United Network Organ Sharing anomalous left coronary artery from pulmonary artery. Database. Journal of Thoracic Cardiovacular Cardiac chamber hypertrophy following hematopoietic International Journal of Cardiology 2007: 122(3): Surgery 2008: 135(2): pp 147-155. stem cell transplantation for primary immunodeficiency. e29-e31. Cardiovascular Surgery 2007: 134(4): pp 967-973. Bully SR, Benson L, Grunebaum E, Roifman CM: Dehghani P, Collins N, Benson L, Horlick E: Role of Biology of Blood and Marrow Transplantation 2008: 17: pp 229-235. Collins N, Mehta R, Benson L, Horlick E: Percutaneous routine radial artery access during aortic coarctation coronary artery fistula closure in adults: technical interventions. Catheterization and Cardiovascular Caldarone CA, Benson L, Holtby H, Li J, Redington AN, and procedural aspects. Catheterization and Interventions 2007: 70(4): pp 622-623. Van Arsdell GS: Initial experience with hybrid palliation Cardiovascular Interventions 2007: 69: pp 872-880. for neonates with single-ventricle physiology. Annals of Thoracic and Cardiovascular Surgery 2007: 84(4): pp 1294-1300. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 17 Dehghani P, Collins N, Horlick E, Benson L: Chronic Estes NA 3rd, Page R, Boyden P, Cappato R, Chiu-Man Friedberg MK, Silverman NH: The systolic to diastolic pulmonary thromboembolism in a patient with a Fontan C, Day J, Dimarco J, Ferrick A, Hamdan M, Hamilton R, duration ratio in children with hypoplastic left heart circulation: percutaneous management. Catheterization Hohnloser S, Jung W, Kanter R, Knight B, Lindsay B, syndrome: a novel doppler index of right ventricular and Cardiovascular Interventions 2007: 70: pp 893-896. Natale A, Reynolds D, Rosenbaum D, Saxon L, Shen function. Journal of the American Society of WK, Trayanova N, Wilkoff B: Hightlights of heart Echocardiography 2007: 20(6): pp 749-755. Dipchand AI, Bharat W, Manlhiot C, Safi M, Lobach NE, rhythm 2007, The annual scientific sessions of the McCrindle BW: A prospective study of dobutamine heart rhythm society, May, 2007: 4(10): pp e1-e9. Friedberg MK, Silverman NH, Dubin AM, Rosenthal DN: Mechanical dyssynchrony in children with systolic stress echocardiography for the assessment of Freedman SB, Haladyn KJ, Floh A, Kirsh JA, Taylor G, Thull- dysfunction secondary to cardiomyopathy: a doppler transplant recipients. Pediatric Transplantation 2008: Freedman J: Myocarditis: clinical findings and diagnostic tissue and vector velocity imaging study. Journal of 12: pp 570-576. evaluation. Pediatrics 2007: 120: pp 1278-1285. cardiac allograft vasculopathy in pediatric heart the American Society of Echocardiography 2007: 20(6): pp 756-763. Dong-Sheng G, Wei-Yi F, Chiu-Man C, Kirsh J, Gross G, Friedberg MK, Feinstein JA, Rosenthal DN: Noninvasive Hamilton RM: QT hysteresis in long-QT syndrome assessment of pulmonary arterial capacitance by Friedberg MK, Silverman NH, Dubin AM, Rosenthal children with exercise testing. Chinese Medical echocardiography. Journal of the American Society of DN: Right ventricular mechanical dyssynchrony in Journal 2007: 120: pp 179-82. Echocardiography 2007: 20(2): pp 186-190. children with hypoplastic left heart syndrome. Journal of American Society of Echocardiography 2007: 20(9): Dutta S, Mihailovic A, Benson L, Kantor PF, Friedberg MK, Kim N, Silverman NH: Atrioventricular Fitzgerald PG, Walton JM, Langer JC, Cameron BH: septal defect recently diagnosed by fetal pp 1073-1079. Thoracoscopic ligation versus coil occlusion for echocardiography: echocardiographic features, Gengsakul A, Harris L, Bradley TJ, Webb GD, Williams patent ductus arteriosus: a matched cohort study associated anomalies and outcomes. Congenital WG, Siu SC, Merchant N, McCrindle BW: The impact of of outcomes and cost. Surgical Endoscopy 2008: Heart Disease 2007: 2(2): pp 110-114. pulmonary valve replacement after tetralogy of Fallot Friedberg MK, Roche SL, Balasingam, Stephenson E, Cardiothoracic Surgery 2007: 32(3): pp 462-468. repair: a matched comparison. European Journal of 22(7): pp 1643-1648. Dutta S, Mihailovic A, Benson L, Kantor PF, Slorach C, Fackoury C, Kantor PF: Evaluation of Fitzgerald PG, Walton JM, Langer JC, Cameron BH: mechanical dyssynchrony in children with idiopathic Gonzalez AMD, Osorio JC, Gruber G, Homma S, Thoracoscopic ligation versus coil occlusion for dilated cardiomyopathy and associated clinical Mital S: Hypertrophy signalling during peripartum patent ductus arteriosus: a matched cohort study outcomes. American Journal of Cardiology 2008: cardiac remodelling. American Journal of Physiology – of outcomes and cost. Journal of Endovascular 101(8): pp 1191–1195. Surgery 2008: 22(7): 1643-1648. 18 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Heart and Circulation Physiology 2007: 293(5): pp H3008-H3013. Jaeggi E, Chitayat D, Golding F, Kim P, Yoo SJ. Grosse-Wortmann L, Al-Otay A, Goo HW, Macgowan Hypertension and Obesity in Youth of the Council on CK, Coles JG, Benson LN, Redington AN, Yoo SJ: Cardiovascular Disease in the Young, Council on Upstairs-downstairs hearts in siblings: prenatal Anatomical and functional evaluation of pulmonary Cardiovascular Nursing, Council on Epidemiology and diagnosis and clinical impact of a rare form of veins in children by using magnetic resonance imaging. Prevention, and Council on Nutrition, Physical Activity congenital malposition of the heart and great arteries. Journal of the American College of Cardiology 2007: and Metabolism. Circulation 2007 116(3): pp 344-357. Cardiology in the Young 2008: 18: pp 337-348. 49: pp 993-1002. Hickey EJ, Blackstone EH, Lofland GK, Yeh T, Pizarro C, Jaeggi ET, Friedberg MK. Diagnosis and management Grosse-Wortmann L, Hamilton R, Yo S-J: Massive Tchervenkov CI, Pigula F, Overman DM, Jacobs ML, of fetal bradyarrhythmias. Pacing and Clinical systemic-to-pulmonary collateral arteries in the setting Caldarone CA, McCrindle BW: Critical left ventricular Electrophysiology 2008: 31: pp S50-S53. of a cavopulmonary shunt and pulmonary venous outflow tract obstruction: the disproportionate impact of biventricular repair in borderline cases. Journal of Jaeggi E, Ryan G, Schmidt M, Chaturvedi RR. Stenting Thoracic and Cardiovascular Surgery 2007: 134(6): of the atrial septum in fetal lambs. Ultrasound in pp 1429-1436. Obstetrics and Gynecology 2007: 30: p 496. an argument that cuts both ways. Heart Rhythm Hickey EJ, McCrindle BW, Blackstone EH, Yeh T, Pigula F, Karamlou T, Gurofsky R, Bojcevski A, Williams WG, Journal 2008: 5: pp 716-717. Clarke D, Tchervenkov CI, Hawkins J, for the CHSS Caldarone CA, Van Arsdell GS, Paul T, McCrindle BW: stenosis. Cardiology in the Young 2007: 17: pp 548-550. Hamilton RM: Genetic testing for mutations or polymorphisms in sudden infant death syndrome: Pulmonary Conduit Working Group: Jugular venous Prevalence and associated risk factors for intervention valved conduit (contegra) matches allograft performance in 313 children with subaortic stenosis. Annals of WG, Smallhorn JF, McCrindle BW: Outcome and in infant truncus arteriosus repair. European Journal Thoracic Surgery 2007: 84(3): pp 900-906. growth potential of left heart structures after neonatal of Cardiovascular Surgery 2008: 33(5): pp 890-898. Han RK, Gurofsky RC, Lee KJ, Dipchand AI, Williams Kaufman BD, Auerbach S, Reddy S, Manlhiot C, Deng L, intervention for aortic valve stenosis. Journal of the American College of Cardiology 2007: 50: pp Horlick EM, McLaughlin PR, Benson LN: The adult with Prakash A, Printz BF, Gruber D, Papavassiliou D, 2406-2414. repaired coarctation of the aorta. Current Cardiology Hsu DT, Sehnertt A, Chung WK, Mital S. RAAS gene Reports 2007: 9: pp 323-30. polymorphisms influence progression of pediatric Helden L, Ross J, Dennison BA, Steinberger J, Ilina MV, Jaeggi ET, Lee K-J: Neonatal rhabdomyoma 122(5): pp 515-23. Williams CL: Primary prevention of cardiovascular causing right ventricular inflow obstruction with hypertrophic cardiomyopathy. Human Genetics 2007: Hayman LL, Meininger JC, Daniels SR, McCrindle BW, disease in nursing practice: focus on children and duct-dependent pulmonary blood flow: successful youth. A scientific statement from the American stenting of PDA. Catheterization and Cardiovascular Heart Association Committee on Atherosclerosis, Interventions 2007: 69: pp 881-885. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 19 Krahn AD, Champagne J, Healey JS, Cameron D, Li J, Zhang G, Cai S, Redington AN: Effect of inhaled McCrindle BW, Williams RV, Mital S, Clark BJ, Russell Simpson CS, Thibault B, Mangat I,Tung S, Sterns L, hydrogen sulfide on metabolic responses in anesthetized, JL, Klein G, Eisenmann JC: Physical activity levels in Birnie DH, Exner DV, Parkash R, Sivakumaran S, paralyzed and mechanically ventilated piglets. children and adolescents are reduced after the Fontan Davies T, Coutu B, Cyrstal E, Wolfe K, Verma A, Pediatric Critical Care Medicine 2008: 9(1): pp 110-112. Paredes FA, Essebag V: Outcome of the fidelis procedure, independent of exercise capacity, and are associated with lower perceived general health. Archives Stephenson EA, Sanatani S, Gow R, Connors S, Li J, Zhang G, Herridge J, Holtby H, Humpl T, of Disease in Childhood 2007: 92(6): pp 509-514. implantable cardioverter-defibrillator lead advisory: Redington AN, Van Arsdell GS: Energy expenditure a report from the Canadian Heart Rhythm Society and caloric and protein intake in infants following the Minich LL, Sleeper LA, Atz AM, McCrindle BW, Lu M, Working Group on device advisories. Heart Rhythm Norwood procedure. Pediatric Critical Care Medicine Colan SD, Printz EF, Klein GL, Sundel RP, Takahashi M, 2008: 5: pp 639-642. 2008: 9(1): pp 55-61. Li JS, Vetter VL, Newburger JW: Delayed diagnosis of Kawasaki disease: what are the risk factors? Lacro RV, Dietz HC, Wruck LM, Bradley TJ, Colan SD, Li J, Zhang G, Holtby H, Guerguerian AM, Cai S, Humpl Devereux RB, Klein GL, Li JS, Minich LL, Paridon SM, T, Caldarone CA, Redington AN, Van Arsdell GS: The Pediatrics 2007: 120(6): pp e1434-e1440. Pearson GD, Printz BF, Pyeritz RE, Radojewski E, influence of systemic hemodynamics and oxygen Moola F, Faulker GEJ, Kirsh JA, Kilburn J: Physical Roman MJ, Saul JP, Stylianou MP, Mahony L, for the transport on cerebral oxygen saturation in neonates activity and sport participation in youth with congenital Pediatric Heart Network Investigators: Rationale and after the Norwood procedure. Journal of Thoracic heart disease: perceptions of children and parents. design of a randomized clinical trial of beta-blocker Cardiovascular Surgery 2008: 135(1): pp 83-90. Adapted Physical Activity Quarterly 2008: 25: pp 49-70. therapy (atenolol) versus angiotensin II receptor blocker therapy (losartan) in individuals with Marfan McCrindle BW, Li JS, Minich LL, Colan SD, Atz AM, Nelken J, Meshkani R, Chahal N, McCrindle BW, Adeli K: syndrome. American Heart Journal 2007: 154(4): Takahashi M, Vetter VL, Gersony WM, Mitchell PD, Detection of familial defective apoB (FDB) mutations in pp 624-631. Newburger JW, for the Pediatric Heart Network hypercholesterolemic children and adolescents by Investigators: Coronary artery involvement in children denaturing high performance liquid chromatography Li J, Zhang G, Benson L, Holtby H, Cai S, Humpl T, with Kawasaki disease: risk factors from analysis of (DHPLC). Clinical Biochemistry 2008: 41(6): pp 395-399. Van Arsdell GS, Redington AN, Caldarone CA: serial normalized measurements. Circulation 2007: Comparison of the profiles of postoperative systemic 116(2): pp 174-179. Nielsen JM, Kristiansen SB, Ringgaard S, Nielsen TT, the hybrid or the Norwood procedure: a pilot study. McCrindle BW, McIntyre S, Kim C, Lin T, Adeli K: volume measurement in mice by conductance Circulation 2007: 116(11 Suppl): pp 179-187. Are patients after Kawasaki disease at increased catheter. Evaluation and optimization of calibration. risk for premature cardiovascular disease? Journal American Journal of Physiology - Heart and Circulatory of Pediatrics 2007: 151(3): pp 244-248. Physiology 2007: 293(1): H534-H540. Flyvbjerg A, Redington AN, Botker HE: Left ventricular hemodynamics and oxygen transport in neonates after 20 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Roche SL, Kaufman J, Dipchand AI, Kantor PF: Osaki M, Caldarone CA, Dipchand AI: Extended aortic Pollock-BarZiv SM, McCrindle BW, West LJ, dissection following aortic cannulation in an infant. Dipchand AI: Waiting before birth: outcomes after Hypertension after pediatric heart transplantation is Pediatric Cardiology 2008: 29: pp 232-233. fetal listing for heart transplantation. American primarily associated with immunosuppressive regimen. Journal of Transplantation 2008: 8: pp 412-418. The Journal of Heart and Lung Transplantation 2008: 5: pp 501-507. Osaki M, McCrindle BW, Van Arsdell GS, Dipchand AI: Anomalous origin of a coronary artery from the Pollock-BarZiv SM, McCrindle BW, West LJ, Manhliot C, opposite sinus of valsalva with an interarterial course: Vanderliet M, Dipchand AI: Competing outcomes after Roman KS, Fouron JC, Nii M, Smallhorn JF, clinical profile and approach to management in the neonatal and infant wait-listing for heart transplantation. Chaturvedi R, Jaeggi ET: Determinants of outcome in pediatric population. Pediatric Cardiology 2008: Journal of Heart and Lung Transplantation 2007: 26: fetal pulmonary valve stenosis or stresia with intact 29(1): pp 24-30. pp 980-985. ventricular septum. American Journal of Cardiology 2007: 99: pp 699-703. Ozawa A, Chaturvedi R, Lee KY, Benson L: Femoral Reid GJ, Siu SC, McCrindle BW, Irvine MJ, Webb GD: Saul JP, Epstein AE, Silka MJ, Berul CI, Dick M, vein hemostasis in children using a suture-mediated Sexual behaviour and reproductive concerns among device. Journal of Interventional Cardiology 2007: adolescents and young adults with congenital heart DiMarco JP, Friedman RA, Rosenthal E, Stephenson EA, 20: pp 164-167. disease. International Journal of Cardiology 2008: Vetter VL: Clinical competency statement: training 125(3): pp 332-338. pathways for implantation of cardioverter-defibrillators and cardiac resynchronization devices in pediatric Pihkala J, Yazaki S, Mehta R, Lee KJ, Chaturvedi R, Reid GJ, Webb GD, McCrindle BW, Irvine MJ, Siu SC: and congenital heart patients. Heart Rhythm Journal clinical impact of transcatheter closure of interatrial Health behaviors among adolescents and young 2008: 5(6): pp 926-933. communications after a fenestrated Fontan adults with congenital heart disease. Congenital procedure: medium-term outcomes. Catheterization Heart Disease 2008: 3(1): pp 16-25. Soslow JH, Friedberg MK, Silverman NH. Idiopathic Richmond ME, Addonizio LJ, Hsu DT, Mital SR, Mosca an indication for early delivery. Echocardiography Pollock-BarZiv SM, den Hollander N, Ngan BY, RS, Quaegebeur JM, Chen J, Lamour, JM: Cardiac 2008: April 8 [E-pub ahead of print]. Kantor P, McCrindle BW, Dipchand AI: Pediatric heart retransplantation in high-risk pediatric patients. transplantation in HLA-sensitized patients: evolving Pediatric Transplantation 2007: 11: pp 615-623. McCrindle BW, Arsdell GV, Benson LN. Feasibility and premature closure of the ductus arteriosus: and Cardiovascular Intervention 2007: 69: pp 1007-1014. Stephenson EA, Berul CI: Electrophysiologic management and assessment of intermediate-term interventions for inherited arrhythmia syndromes. outcomes in a high-risk population. Circulation 2007: Circulation 2007: 116: pp 1062-1080. 116(11 Suppl): pp 1172-1178. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 21 Suto F, Zhu W, Chan A, and Gross GJ: IKr and Wong D, Benson LN, Van Arsdell GS, Karamlou T, Dodd DA, Cabo J, Dipchand AI: Acute rejection: IKs remodelling differentially affects QT interval McCrindle BW: Balloon angioplasty is preferred natural history, risk factors, surveillance, and prolongation and dynamic adaptation to heart rate to surgery for aortic coarctation. Cardiology in the treatment. In: ISHLT Monograph Series Pediatric acceleration in bradycardic rabbits. American Journal Young 2008: 18: pp 79-88. Heart Transplantation (Canter CE, Kirklin J, eds.). Philadelphia: Elsevier, 2008. of Physiology – Heart and Circulatory Physiology 2007: 292: pp H1782-H1788. Zhu Y, Gramolini AO, Walsh MA, Zhou YQ, Slorach C, Friedberg MK, Takeuchi JK, Sun H, Hendelman RM, Friedberg MK, Silverman NH, Hanley FL, Mohan RV, Vogt KN, Manlhiot C, Van Arsdell G, Russell JL, Mital Backx PH, Redington AN, Maclennan DH, Bruneau BG: Albanese CT: Fetal cardiac intervention. In: S, McCrindle BW. Somatic growth in children with Tbx5-dependent pathway regulating diastolic function in Hemodynamics and Cardiology: Neonatology single ventricle physiology: Impact of physiologic state. congenital heart disease. Proceedings of the National Questions and Controversies (Kleinman CS and Journal of the American College of Cardiology 2007: Academy of Science USA 2008: 105(14): pp 5519-5524. Seri I, eds.). Philadelphia: Saunders/Elsevier, 2008. BOOK CHAPTERS Redington AR: Cardiopulmonary and right-left heart 50(19): pp 1876-1883. Walsh MA, Benson LN, Dipchand AI, Redington AN, Benson L: Pulmonary vein stenosis. In: Percutaneous interactions. In: Moss and Adam’s Heart Disease in Caldarone CA, Van Arsdell GS, Kantor PF: Surgical Interventions for Congenital Heart Disease (Sievert H, Infants, Children and Adolescents (Including the Fetus repair of the mitral valve in children with dilated Qureshi S, Wilson N, Hijazi Z, eds.). London: Informa and Young Adult), 7th Edition (Allen HD, Driscoll DJ, cardiomyopathy and mitral regurgitation. Annals Healthcare, 2007. Shaddy RE, Feltes TF, eds.). Philadelphia: Wolters Kluwer Lippincott Williams & Wilkins, 2008. of Thoracic Surgery 2008: 85: pp 2085-2088. Benson L, Mikailian H: Imaging modalities in the Walsh MA, Lee K-J, Chaturvedi R, Van Arsdell GS, cath lab: angiography. In: Percutaneous Interventions Webb GD, Smallhorn JF, Therrien J, Redington AN: Benson LN: Radiofrequency perforation of right for Congenital Heart Disease (Sievert H, Qureshi S, Congenital heart disease. In: Braunwald’s Heart ventricular outflow tract as a palliative strategy Wilson N, Hijazi Z, eds.). London: Informa Healthcare, Disease: A Textbook of Cardiovascular Medicine, 8th for pulmonary atresia wiht ventricular septal defect. 2007, pp 15-32. Edition (Libby P, Bonow RP, Mann DL, Zipes D, eds.). Catheterization and Cardiovascular Interventions 2007: 69: pp 1015-1020. Philadelphia: Saunders Elsevier, 2007. Dipchand AI: HSC mismatch protocol. In: Paediatric heart transplantation - en handledning for vardgivare Yoo SJ, Jaeggi E. Ultrasound evaluation of the (Gilljam T, ed.). Goteborg: Sahlgrenska fetal heart. In: Ultrasonography in Obstetrics and Universitetssjukhuset, 2008. Gynaecology, 5th Edition (Callen P, ed.) Philadelphia: Saunders Elsevier, 2008. 22 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT FUNDING Characterisation of circulating effectors and temporal Contractile adaptations to constrained heart rate A randomized, double-blind, multi-centre study of profiling of remote ischaemic preconditioning. Redington AN, conditions. Gross GJ, Redington AN. Canadian antiarrhythmic medications in the treatment of infants Callahan J, Gross G, Kharbanda R. Canadian Institutes Institutes of Health Research ($529,180 2008-2012). with supraventricular tachycardia (SAMIS Trial). of Health Research ($324,951 2006-2009). Contractile and electrophysiologic adaptations Shubhayan S, Stephenson E, Potts J, Etheridge S. to constrained heart rate conditions. Gross GJ, Heart and Stroke Foundation of Canada Canadian Institutes of Health Research Team in ($444,060 2006-2010). Childhood Obesity Research. Anderson H, Hamilton J, Redington AN. Heart and Stroke Foundation Parkin P, Pencharz P. Canadian Institutes of Health of Ontario ($209,097 2006-2008). A transatlantic network for the investigation of remote Research ($2,452,522 2008-2013). Contribution of three-dimensional echocardiography to ischemic preconditioning. Redington AN, McCrindle BW, Callahan J, Wilson G, Caldarone C, Kharbanda R, Clinical and genomic responses to remote ischemic surgery for left ventricular outflow tract obstruction Botker HE, Neilsen TT. Leducq Foundation preconditioning. Redington A, Arab S, Coles J, Holtby H, in children. Friedberg MK, Van Arsdell G, Calderone C, Liu P, Schwartz S, Van Arsdell G. National Heart, Lung, Coles J, Golding F, Bradley TJ, Gooi A. The Hospital ($6,800,000 2006-2011). Biventricular pacing in children after surgery for and Blood Institute/National Institutes of Health for Sick Children Paediatric Consultants Partnership ($2,047,004 US 2006-2011). ($9,819 2007-2008). congenital heart disease. Friedberg M, Stephenson EA. Labatt Family Heart Centre Innovations Funding Clinical and laboratory evaluation of acute rejection, Creating a fetal lamb model of hypoplastic left heart ($15,000 2007-2009). myocyte growth, repair and oxidative stress following syndrome. Chaturvedi R, Jaeggi E. Labatt Family de novo cardiac transplant: a comparison between Heart Centre ($21,000 2008). Cardiac gene bank. Mital S, Hamilton R, Coles J. tacrolimus- and cyclosporine-based immunprophylactic Labatt Heart Centre Fund ($100,000 2008-2010). regimens with mycophenolic acid therapeutic drug CTOT-C (Clinical Trials in Organ Transplantation monitoring. Dipchand AI, Pollock BarZiv S, Allain- in Children) 1U01AI077867-01: Alloantibodies in Cardiac transplantation in infancy – exploring the Rooney T, Kantor PF. Astellas Pharma Canada Inc. cardiac transplantation: Intervention, outcomes implication for the immune system. Platt J, West L, ($65,000 2006-2008). and mechanisms. Webber S, Dipchand AI, Blume E, Canter C, Naftel D, Hsu D. National Institutes of Cascalho M. National Heart, Lung, and Blood Institute/National Institutes of Health Clinial characteristics and psychological health ($7,100,942 US 2005-2010). of Canadian children with implantable cardioverter Health ($5,205,958 2008-2013). defibrillators. Stephenson EA, Conway J, Chiu-Man C, Mendlowitz S. Labatt Family Heart Centre Innovations Funding ($10,731 2008-2009). THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 23 Identifying factors involved in the prenatal diagnosis Individualized heparin and protamine management Late functional status in a cohort of survivors with of congenital heart defects requiring neonatal in infants undergoing cardiopulmonary bypass pulmonary atresia intact ventricular septum from the intervention. Dutil N, Jaeggi E. Labatt Family Heart for cardiac surgery reduces bleeding and blood Congenital Heart Surgeons Society study. Caldarone C. transfusion requirements. Guenwald C, Chan A, Children’s Heart Foundation ($89,190 US 2007-2008). Centre ($18,600 2008-2009). Van Arsdell G, Holtby H. Heart and Stroke Effects of remote ischemic preconditioning on clinical Foundation of Canada ($108,566 2006-2008). Molecular expression and physiology in developing atrioventricular nodal cells. Hamilton RM, Gross GJ, and genomic responses to open heart surgery in children. McCrindle BW, Redington A. Canadian Inflammation and atherosclerosis in paediatric Silverman ED. Canadian Institutes of Health Insititutes of Health Research ($100,000 2007-2008). rheumatic disease. Bradley TJ, Beyene J, Schneider R, Research ($370,500 2006-2009). Effects of right ventricular intraventricular mechanical Centre - Innovation Fund ($12,000 2007-2008). Feldman B, Silverman ED. Labatt Family Heart Optimizing health among children with congenital heart defects through rehabilitation focused on physical dyssynchrony on right and left ventricular dysfunction activity determinants. McCrindle BW, Russell JL, in tetralogy of Fallot. Friedberg M, Redington A, Inflammation and atherosclerosis in paediatric Yoo S-J, Stephenson E, Russell J, Kantor P, Roche L. rheumatic disease. Bradley TJ, Beyene J, Schneider R, Dettmer E, Longmuir P. Heart and Stroke Foundation National Grants Program, SickKids Foundation, Feldman B, Silverman ED. Heart and Stroke of Canada ($208,928 2007; $616,465 2008-2011). and Canadian Institutes of Health Research Foundation of Ontario ($125,448 2007-2009). Outcome of paediatric patients with cardiomyopathy: ($108,766 2007-2009). Intercalated disc potein expression in arrhythmogenic a multicentre review of paediatric patients listed for Genetics of heart disease in children. Mital S. right ventricular cardiomyopathy. Hamilton RM, transplant in the paediatric Heart Transplant Study Labatt Family Heart Centre Innovation Fund Chauhan V, Scherer S. Heart and Sroke Group. Dipchand AI, Hsu D, Naftel D. Children’s ($200,000 per year 2007-2010). Foundation of Ontario ($72,100 2008-2010). Cardiomyopathy Foundation ($21,321 2007-2008). HLA sensitization: prevalence and impact of Intrauterine growth retardation associated with and Perioperative use of thymoglobulin in paediatric heart alloantibodies in paediatric heart transplant recipients. without placental insufficiency: impact on myocardial transplant recipients. Dipchand AI, Pollock-BarZiv SM, Dipchand AI, Pollock-Barziv S, McCrindle BM. The function and on arterial compliance. Jaeggi ET, Allain-Rooney T, Kantor PF. Genzyme Canada ($5,000 Hospital for Sick Children Paediatric Consultants Bradley TJ, Keating SJ, Kingdom JCP. Heart and 2006-2007). Partnership ($10,000 2007 - 2008). Stroke Foundation of Ontario ($77,255 2006-2008). 24 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Prevention, detection and management of Temporal profiling and circulating effectors of remote Validation of accelerometry as a measure of physical thromboembolic complications after paediatric cardiac ischaemic preconditioning. Redington A, Callahan J. activity and inactivity in children with chronic disease. surgery: toward evidence-based clinical protocols. Canadian Institutes of Health Research ($284,000 Feldman B, Stephens S, Takken T, Wright V, Tremblay M, Manlhiot C, Gruenwald C, Brandao L, Chan AK, 2005-2008). Eslinger D, Beyene J, Walker-Schneiderman J, Biggar W, Longmuir P, Vandernet J. Canadian Institutes of Schwartz S, Holtby H, Sivarajan B, Van Arsdell G, Caldarone C, Clarizia N, Crawford-Lean L. Labatt The effect of remote ischemic preconditioning on Family Heart Centre Innovations Fund ($29,210 2008). clinical and genetic responses to open heart surgery Right ventricular versus modified blalock-taussig shunt Health Research ($65,000 2007-2008). in children. Redington A. Canadian Institutes of Ventricular function and arterial stiffness in the Health Research ($97,360 2007-2008). evaluation of paediatric heart failure. Kantor PF, Bradley TJ, Roche SL, Slorach C. The Hospital in infants with single ventricle defect undergoing staged reconstruction. McCrindle BM, Site PI, in a The effectiveness of low fat breast milk for the for Sick Children Paediatric Consultants multicentre networked collaboration. National Heart, treatment of chylothorax in infants following Partnership ($7,200 2007-2008). Lung, and Blood Institute/National Institutes of cardiothoracic surgery: a pilot study. Farmer S, Russell Health ($300,000 2005-2008). JL, O’Connor D. Labatt Heart Centre Innovation Fund Ventricular repolarization in complete heart block. ($24,000 2007). Gross GJ. Heart and Stroke Foundation of Ontario ($208,406 2004-2007). Role of chronic inflammation and systemic lupus erythematosus-related factors in the development The role of traditional and non-traditional risks in the of premature atherosclerosis in paediatric SLE. development of atherosclerosis in paediatric systemic Silverman ED, Bargman JM, Beyene J, Bradley TJ. Heart lupus erythematosus. Silverman E, Adeli K, Bargman J. remodelling. Gross GJ. Heart and Stroke Foundation and Stroke Foundation of Ontario ($275,000 2006-2009). Heart and Stroke Foundation of Ontario ($163,454 of Ontario ($268,447 2007-2010). Ventricular repolarization in bradycardic electrical 2003-2008). Very high resolution vascular imaging in children RSV immunoprophylaxis in children with congenital heart disease. Lee K-J, Cranis M, Allen U, James A, The SAMIS Trial: multicentre study of antiarrhythmic with normal and abnormal vascular development O’Brien K, Simmons B, Dennis M, Bracht M, O’Doherty B. medications for treatment of infants with supraventricular and maturation. Sarkola T, Jaeggi E, Redington A. Abbott Laboratories ($71,482 2006-2007). tachycardia. Sanatani, S, Stephenson EA, McCrindle BM, Societatis Medicorum Fennicae (£20,000 salary Potts J, Etheridge S. Heart and Stroke Foundation of support for Dr. Sarkola) Instrumentarium Foundation British Columbia and Yukon ($440,060 2006-2010). (£50,000); The Finnish Cultural Foundation £20,000) RSV immunoprophylaxis in children with congenital heart disease. Lee K-J, Cranis M, Allen U, James A, (2008-2009). O’Brien K, Simmons B, Dennis M, Bracht M, O’Doherty B. Abbott Industries ($82,217 2007-2008). THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 25 Dr. Rosanna Weksberg DIVISION HEAD Dr. Rosanna Weksberg FULL-TIME Dr. David Chitayat Dr. Annette Feigenbaum Dr. Roberto Mendoza Dr. Julian Raiman Dr. Andreas Schulze Dr. Grace Yoon PART-TIME Dr. Karen Chong Dr. Joe Clarke Dr. Leona Fishman Dr. Stephen Meyn Dr. Ahmad Teebi CLINICAL FELLOWS Dr. Fatma Al-Jasmi Dr. Fathiya Al-Murshedi Dr. Christopher Barnett Dr. Brian Chung Dr. Ellen Crushell Dr. Arie Koifman Dr. Gustavo Maegawa Dr. Sandhya Parkash RESIDENTS Dr. Hanna Faghfoury Dr. Jane Gillis Dr. Elaine Goh Dr. Sharan Goobie Division of Clinical and Metabolical Genetics OVERVIEW newborn period. SickKids is one of five regional established to honour Dr. Opitz, the founding The Division of Clinical and Metabolic Genetics newborn screening centres and is the largest in the editor-in-chief of the American Journal of Medical provides excellent clinical care, and supports a variety province. Dr. Andreas Schulze was recently appointed Genetics. The awardee is selected for a significant of clinical, research, educational and administrative the Medical Director of the Newborn Screening research paper published in the previous year in the programs. An important new endeavour is the Ontario Program. The program at SickKids is co-ordinated with American Journal of Medical Genetics in the areas of Genetics Secretariat (OGS). The Secretariat was the Division of Clinical and Metabolic Genetics and is congenital anomalies, mechanisms of morphogenesis generated by a Department of Paediatrics invitation supported by an interdisciplinary team. and malformations, or developmental biology. The support of this initiative by CHS and the Hospital Research endeavors in the division are a vital part of The seventh annual Starbucks Clinical Research to support province-wide subspecialty initiatives. Executive has been instrumental in its success. our program and currently focus on the translation of Summer Students Competition was awarded to The OGS is chaired by Christopher Carew from new genomic information for patients and their families. Laura White for a study on bone marrow transplantation SickKids and has representation from all the clinical The use of microarrays for genetic testing continues to in Mucopolysaccaridosis type I (Hurler Disease). She was supervised by Dr. Julian Raiman. genetics programs and genetic diagnostic laboratories increase.The large influx of genomic data for many of across Ontario. The mandate of OGS is to develop a our patients has impacted our pattern of practice. collaborative and integrated approach to negotiations There are many new challenges in the interpretation of with the Ontario Ministry of Health and Long-Term entirely novel data in the clinical setting. developed and participated in numerous educational activities within the hospital. It has important links to Care for resourcing genetic services. The DNA Resource Centre Program has continued to The Division of Clinical and Metabolic Genetics has Dr. Grace Yoon received the John M. Opitz Young the Research Institute and the Department of Paediatric Investigator Award in the fall of 2007. This award was Laboratory Medicine. The division directs fellowship provide up-to-date information regarding genetic testing available nationally and internationally, and ensures that all patients at SickKids have access to cutting-edge genetic testing. Drs. Roberto Mendoza, David Chitayat and team In 2006, the MOHLTC embarked on major changes to newborn screening. Twenty-six additional disorders (endocrine, genetic metabolic, hemoglobinopathies and cystic fibrosis) are now screened for in the RESIDENTS Dr. Shailly Jain Dr. Linda Kim Dr. Joyce So Dr. Mohammad Yaghoobi ADMINISTRATIVE STAFF Jennifer Costa Michael Delaney Susan Dukhedlin-Lalla Laurie Furtado Kamalina Gupta Denese Henry Andrea Jonker Melanie Lameront Tina McConkey Natalie Sargeant Deb Taylor Rozmin Visram and residency training programs in genetic medicine Briggs TA, Abdel-Salam GM, Balicki M, Baxter P, Demuth I, Bradshaw PS, Lindner A, Anders M, as well as the MSc Program in Genetic Counselling. Bertini E, Bishop N, Browne BH, Chitayat D, Heinrich S, Kallenbach J, Schmelz K, Digweed M, Chong WK, Eid MM, Halliday W, Hughes I, Meyn MS, Concannon P: Endogenous hSM1B/Apollo HONOURS AND AWARDS Klusmann-Koy A, Kurian M, Nischal KK, Rice GI, interacts with TRF2 and stimulates ATM in response Yoon G: John M. Opitz Young Investigator Award, 2007. Stephenson JB, Surtees R, Talbot JF, Tehrani NN, to ionizing radiation. DNA Repair 2008: May 9 Tolmie JL, Toomes C, van der Knaap MS, Crow YJ: [Epub ahead of print]. PUBLICATIONS Cerebroretinal microangiopathy with calcifications Auray-Blais C, Cyr D, Ntwari A, West ML, and cysts (CRMCC). American Journal of Medical Footitt EJ, Sinha MD, Raiman JA, Dhawan A, Cox-Brinkman J, Bichet DG, Germain DP, Genetics, Part A 2008: 146(2): pp 182-190. Moganasundram S, Champion MP: Mitochondrial Clarke JTR, Drouin R: Urinary globotriaosylceramide Brunetti-Pierri N, Doty SB, Hicks J, Phan K, and review. British Journal of Anaethesia 2008: excretion correlates with the genotype in children Mendoza-Londono R, Blazo M, Tran A, Carter S, 100(4): pp 436-441. disorders and general anaesthesia: a case series Laframboise R, Melançon SB, Stockley T, and adults with Fabry disease. Molecular Genetics Lewis R, Plon S, Phillips W, Smith EO, Ellis K, Lee, B: and Metabolism 2008: 93: pp 331-340. Generalized metabolic bone disease in Neurofibromatosis Greer KJ, Kirkpatrick SJ, Weksberg R, Pauli RM: type 1. Molecular Genetics and Metabolism 2008: Beckwith-Wiedemann syndrome in adults: 94(1): pp 105-111. observations from one family and recommendations Bähring S, Kann M, Neuenfeld Y, Gong M, Chitayat D, for care. American Journal of Medical Genetics. Toka HR, Toka O, Plessis G, Maass P, Rauch A, Aydin A, Luft FC: Inversion region for hypertension and Clarke JTR, West M, Bultas J, Schiffmann R: The Part A 2008: 146A(13): pp 1707-1712. brachydactyly on chromosome 12p features multiple pharmacology of multiple regimens of agalsidase splicing and noncoding RNA. Hypertension 2008: alfa enzyme replacement therapy for Fabry disease. Hadzi N, Vara R, Raiman J, Mieli-Vergani G: 51(2): pp 426-431. Genetic Medicine 2007: 9: pp 504-509. Old versus new antiepileptic drugs: The SANAD study. Lancet 2007: 370(9584): p 315. Beauchamp NJ, Dalton A, Ramaswami U, Niinikoski H, Mention K, Kenny P, Kolho KL, Raiman J, Walter J, Treacy E, Tanner S, Sharrard M: Glycogen storage disease type IX: high variability in clinical phenotype. Molecular Genetics and Metabolism 2007: 92(1-2): pp 88-99. 28 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Jaeggi E, Chitayat D, Golding F, Kim P, Yoo SJ: Lindner M, Abdoh G, Fang-Hoffmann J, Shabeck N, McCready ME, Carson NL, Chakraborty P, Clarke JT, Prenatal diagnosis of topsy-turvy heart. Cardiology Al-Sayrafi M, Al-Janahi M, Ho S, Abdelrahman MO, Callahan JW, Skomorowski MA, Chan AK, Bamforth F, in the Young 2008: 18(3): pp 337-342. Ben-Omran T, Bener A, Schulze A, Al-Rifai H, Al-Thani G, Casey R, Rupar CA, Geraghty MT: Development of a Hoffmann GF: Implementation of extended neonatal clinical assay for detection of GAA mutations and Kepron C, Blumenthal A, Chitayat D, Cutz E, screening and a metabolic unit in the State of Qatar: characterization of the GAA mutation spectrum in a Superti-Furga A, Keating S. An autophagic vacuolar developing and optimizing strategies in co-operation Canadian cohort of individuals with glycogen storage myopathy-like disorder presenting as non-immune with the Neonatal Screening Center in Heidelberg. disease, type II. Molecular Genetics and Metabolism hydrops in a female fetus. Pediatric and Developmental Journal of Inherited Metabolic Disease 2007: 30(4): 2007: 92: pp 325-335. Pathology 2008: February 25 [Epub ahead of print]. pp 522-529. Konen O, Armstrong D, Clarke H, Padfield N, Marshall CR, Noor A, Vincent JB, Lionel AC, Feuk L, Napierala D, Scott DA, Sibai T, Casas K, Wilson P, Weksberg R, Blaser S: C1-2 vertebral anomalies Skaug J, Shago M, Moessner R, Pinto D, McAuliffee F, Lee J, Muneer R, Leonard JC, Ramji FG, Lachman R, in 22q11.2 microdeletion syndrome. Pediatric Chitayat D, Halliday W, Keating S, Shah V, Li S, Stankiewicz P, Lee B, Mulvihill JJ: De novo Radiology 2008: 38(7): pp 766-771. Fink M, Nevo O, Ryan G, Shannon P, Blaser S: three-way chromosome translocation 46, XY, t Rhombencephalosynapsis: prenatal imaging and (4;6;21)(p 16; p 21.1; q21) in a male with Levy HL, Milanowski A, Chakrapani A, Cleary M, autopsy findings. Ultrasound in Obstetrics and cleidocranial dysplasia. American Journal of Medical Lee P, Trefz FK, Whitley CB, Feillet F, Feigenbaum AS, Gynaecology 2008: 3(5): pp 542-548. Genetics 2008: 146(4): pp 453-8. Purandare SM, Mendoza-Londono R, Yatsenko SA, Bebchuk JD, Christ-Schmidt H, Dorenbaum A, Sapropterin Research Group: Efficacy of sapropterin Marshall C, Noor A, Vincent J, Lionel A, Feuk L, Ren Y, Thiruvahindrapduram B, Fiebig A, Schreiber S, dihydrochloride (tetrahydrobiopterin, 6R-BH4) for Skaug J, Shago M, Moessner R, Pinto D, Ren Y, Friedman J, Ketelaars CE, Vos YJ, Ficicioglu C, reduction of phenylalanine concentration in patients Thiruvahindrapduram B, Fiebig A, Schreiber S, Kirkpatrick S, Nicolson R, Sloman L, Summers A, with phenylketonuria: a phase III randomised Friedman J, Ketelaars CEJ, Vos YJ, Ficicioglu C, Gibbons CA, Teebi A, Chitayat D, Weksberg R, placebo-controlled study. Lancet 2007: 370(9586): Kirkpatrick S, Nicholson R, Sloman L, Summers A, Thompson A, Vardy C, Crosbie V, Luscombe S, pp 504-510. Gibbons C, Teebi A, Chitayat D, Weksberg R, Baatjes R, Zwaigenbaum L, Roberts W, Fernandez B, Thompson A, Vardy C, Crosbie V, Luscombe S, Szatmari P, Scherer SW: Structural variation of Baatjes R, Zwaigenbaum L, Roberts W, Fernandez B, chromosomes in autism spectrum disorder. American Szatmari P, Scherer S: Structural variation of Journal of Human Genetics 2008: 82(2): pp 477-488. chromosomes in autism spectrum disorder. American Journal of Human Genetics 2008: 82(2): pp 477-488. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 29 Spitzer RF, Wherrett D, Chitayat D, Colgan T, Dodge JE, Robinson AJ, Blaser S, Toi A, Chitayat D, Halliday W, Sermer D, Quercia N, Chong K, Chitayat D: Acrofacial Pantazi S, Gundogan M, Laughlin S, Ryan G: The fetal dysostosis syndrome type Rodriguez: prenatal diagnosis Salle JL, Allen L: Maternal luteoma of pregnancy cerebellar vermis: assessment for abnormal development and autopsy findings. American Journal of Medical presenting with virilization of the female infant. by ultrasonography and magnetic resonance imaging. Genetics. Part A 2007: 143(24): pp 3286-3289. Journal of Obstetrics and Gynaecology Canada 2007: 29(10): pp 835-840. Ultrasound Quarterly 2007: 23(3): pp 211-223. Smith AC, Shuman C, Chitayat D, Steele L, Ray PN, Rela M, Battula N, Madanur M, Mieli-Vergani G, Bourgeois J, Weksberg R: Severe presentation of Stachon AC, Baskin B, Smith AC, Shugar A, Dhawan A, Champion M, Raiman J, Heaton N: Auxiliary Beckwith-Wiedemann syndrome associated with high Cytrynbaum C, Fishman L, Mendoza-Londono R, liver transplantation for propionic academia. American levels of constitutional paternal uniparental disomy for Klatt R, Tebbi A, Ray PN, Weksberg R: Molecular Journal of Transplantation 2007: 7(9): pp 2200-2203. chromosome 11p15. American Journal of Medical diagnosis of 22q11.2 deletion and duplication by Genetics. Part A 2007: 143(24): pp 3010-3015. multiplex ligation dependent probe amplification. American Journal of Medical Genetics. Part A 2007: Revencu N, Boon LM, Mulliken JB, Enjolras O, Crodisco MR, Burrows PE, Clapuyt P, Hammer F, Smith AC, Choufani S, Ferreira JC, and Weksberg R: Dubois J, Baselga E, Brancati F, Carder R, Quintal JM, Growth regulation, imprinted genes and chromosome Dallapiccola B, Fischer G, Frieden IJ, Garzon M, Harper J, 11p15.5. Pediatric Research 2007: 61(5 Pt 2): Johnson-Patel J, Labreze C, Martorell L, Paltiel HJ, Pohl pp 43R-47R. 143(23): pp 2924-2930. Van der Knaap MS, Linnankivi T, Paetau A, Feigenbaum A, Wakusawa K, Haginoya K, Kohler W, Henneke M, Dinopoulos A, Grattan-Smith P, Brockmann K, A, Prendiville J, Rohrbach M, Clarke JTR: Treatment of lysosomal storage disorders: progress with enzyme Somech R, Somers GR, Chitayat D, Grunebaum E, Schiffmann R, Blaser S: Hypomyelination with atrophy replacement therapy. Drugs 2007: 67: pp 2697-2716. Atkinson A, Kolomietz E, Roifman CM: Fatal lung of the basal ganglia and cerebellum. Follow-up and fibrosis associated with immunodeficiency and pathology. Neurology 2007: 69: pp 166-171. Quere I, Siegel DH, Valente EM, Van Hagen A, Van gonadal dysgenesis in 46XX sisters – a new syndrome. Hest L, Vaux KK, Vicente A, Weibel L, Chitayat D, American Journal of Medical Genetics. Part A 2008: Verbruggen KT, Sijens PE, Schulze A, Lunsing RJ, Vikkula M: Parkes Weber syndrome, vein of Galen 146(1): pp 8-14. Jakobs C, Salomons GS, van Spronsen FJ. Successful aneurysmal malformation, and other fast-flow vascular treatment of a guanidinoacetate methyltransferase anomalies are caused by RASA1 mutations. Human deficient patient: findings with relevance to treatment Mutations 2008: 29(7): pp 959-965. strategy and pathophysiology. Molecular Genetics and Metabolism 2007: 91(3): pp 294-296. 30 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Watts P, Kumar N, Ganesh A, Sastry P, Pilz D, Levin AV, Yoon G, Westmacott R, MacMillan L, Quercia N, Hughes S, Weksberg R, Moldovan L: Use of Chitayat D: Chorioretinal dysplasia, hydranencephaly, Koutsou P, Georghiou A, Christodoulou K, Banwell B: quantitative PCR for the detection of genomic and intracranial calcifications: pseudo-TORCH or a Complete deletion of the aprataxin gene: ataxia with microdeletions or microduplications. In: PCR new syndrome? Eye 2008: 22(5): pp 730-733. oculomotor apraxia type 1 with severe phenotype and Methods Express (Hughes S, Moody A, eds.). cognitive deficit. Journal of Neurology, Neurosurgery Scion Publishing Ltd., United Kingdom, 2007. Weksberg R, Shuman C, Wilkins-Haug L, Mann M, and Psychiatry 2008: 79(2): pp 234-236. Stewart D, Croughan M, Rakowsky C, Leader A, Hall J, Mendoza-Londono R, Ardelean D, Goobie S: Friedman JM, Simpson JL, Holmes L, Infante-Rivard C: Zampino G, Pantaleoni F, Carta C, Cobellis G, Vasta I, Genetics: In: The Hospital for Sick Children Workshop report: evaluation of genetic and epigenetic Neri C, Pogna E, De Feo E, Delogu A, Sarkozy A, Atzeri F, Handbook of Paediatrics. Elsevier, Canada, 2007. risks associated with assisted reproductive Selicorni A, Rauen K, Cytrynbaum C, Weksberg R, technologies and infertility. Fertility and Sterility 2007: Dallapiccola B, Ballabio A, Gelb B, Neri G, Tartaglia M: Schulze A, Battini R: Pre-symptomatic treatment 88(1): pp 27-31. Diversity, parental germline origin, and phenotypic of Creatine biosynthesis defects. In: Creatine and spectrum of de nova HRAS missense changes in Costello creatine Kinase in Health and Disease (Salomons syndrome. Human Mutation 2007: 28(3): pp 265-272. GS, Wyss M, eds.). Springer, Dordrecht, 2007. Werner T, Treiss I, Kohlmueller D, Mehlem P, Teich M, Longin E, Gerstner T, Koenig SA, Schulze A: Effects of valproate on acylcarnitines in children with epilepsy BOOKS AND BOOK CHAPTERS Wyss M, Braissant O, Pischel I, Salomons GS, using ESI-MS/MS. Epilepsia 2007: 48(1): pp 72-76. Chitayat D: The clinical approach to the newborn with Schulze A, Stockler S, Wallimann T: Creatine and multiple abnormalities. In: Residents Handbook of creatine kinase in health and disease - a bright future Yoon G, Rosenberg J, Blaser S, Rauen KA: Neonatology, 3rd Edition (Kirpalani H, Moore A, ahead? In: Creatine and Creatine Kinase in Health Neurological complications of the cardio-facio- Perlman M, eds.). B.C. Decker Inc., Hamilton, Ontario, and Disease (Salomons GS, Wyss M, eds.). Springer, cutaneous syndrome (CFC). Developmental Medicine Canada, 2007. Dordrecht, 2007. and Child Neurology 2007: 49(12): pp 894–899. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 31 FUNDING Canadian Molecular Cytogenetics Platform. Gene expression of neuronal progenitors in a Analysis of imprinting control in a tumour predisposing Friedman JM, Chitayat D, Meyn S, Weksberg R. 1q22-linked familial subtype of schizophrenia. region on human chromosome 11p15. Weksberg R, Canadian Foundation for Innovation ($4,512,925 McCurdy R, Weksberg R. Canadian Psychiatric Squire J. Canadian Institutes of Health Research 2005-2010) Research Foundation (CPRF) ($80,000 2005-2007) Collaborative Graduate Training Program in Molecular Genome-wide DNA methylation profiling in autism. Autism: Searching for epigenetics errors. Weksberg R. Medicine (training grant) Canadian Institutes of Weksberg R, Roberts W, Scherer S. Canadian SickKids Foundation New Ideas Grant ($50,000 Health Research ($300,000 2002-2008) Institutes of Health Research ($649,518 2008-2011) Dynamics of human double-strand break repair. Hunter Outcome Survey. Clarke, J. Shire Human ($359,565 2004-2007) 2007-2008) A retrospective natural history study of ASM deficiency Meyn S. Canadian Institutes of Health Research Genetic Therapies ($560 per patient ID entry (Niemann-Pick Disease Types A and B). Clarke J. ($123,000 2004-2008) 2007-2009) Genzyme Corporation ($26,995 2005-2008) Canadian Fabry Disease Initiative (combined adult Fabry Outcome Survey. Clarke J. Shire Human Genetic Iduronate-2-sulfatase enzyme replacement therapy in Therapies ($110,000 2007-2009) mucopolysaccharidosis II (MPS II). Clarke J. Shire Human Genetics Therapies ($111,497 2005-2008) and paediatric patients). Sirrs S, Casey R, Clarke J, Bichet D, West M. Canadian federal and provincial Formation and evaluation of a comprehensive governments, private stakeholders ($2,967,450 computer-based teaching module concerning the 2006-2009) Integrative genomics for health research. Scherer S, recognition, diagnosis, and treatment of MPS II (Hunter Weksberg R. Canadian Foundation for Innovation disease). Clarke J, Al-Jasmi F, Moldovan L. Shire ($3,600,262 2004-2008) Human Genetics Therapies ($149,491 2007- 2008) 32 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Investigation of intrauterine growth restriction by gene Pregnancy and development outcomes. Weksberg R, expression and DNA methylation profiling. Weksberg R, Goobie S, Kim L, Roberts W, Croughan M. The Chitayat D, Kingdom J, Keating S. Canadian Institutes Physicians Services Incorporated Foundation of Health Research ($383,883 2008-2011) ($20,000 2007-2008) MPS I and other lysosomal diseases registry. Clarke J. Rare Diseases Clinical Research Centre for urea Genzyme Corporation ($224,901 2006-2009) cycle disorders. Clarke J, Feigenbaum A. Canadian Institutes of Health Research ($25,000 2006-2008) Potential predictor of schizophrenia in a genetic high-risk sample: Ontario project. Chow E, Bassett A, Selective killing of ALT-dependent malignant cells by Weksberg R, Zipursky R, Mikulis D. Ontario Mental inhibition of the Fanconi anaemia pathway: a pilot Health Foundation, Bill Jefferies Schizophrenia study. Meyn S. The Hospital for Sick Children Endowment Fund ($148,188 2002-2008) Research Institute Seed Grant ($50,000 2008-2009) Urea Cycle Diseases Consortium: longitudinal study of urea cycle disorders. Feigenbaum A. Mendel Tuchman/Mark Batshaw USA ($7,000 2007-2008) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 33 Dr. Gideon Koren Dr. Irena Nulman DIVISION HEAD Dr. Shinya Ito Dr. Irena Nulman Dr. Margaret Thompson SCIENTIFIC STAFF Dr. Patricia Harper FULL-TIME Dr. Gideon Koren Dr. Christine Newman PART-TIME Dr. David Juurlink CROSS-APPOINTEES/ CLINICAL Dr. Christopher Parshuram Dr. Denis Scolnik Dr. Dr. Dr. Dr. Dr. Dr. Neil Shear Ran Goldman Sheila Jacobson Sylvain Baruchel Marco Sivilotti Prashant Joshi CROSS-APPOINTEES/ SCIENTIFIC Dr. Lidia Derewlany Dr. Tom Einarson Dr. Anna Taddio Dr. Bonnie Stevens CLINICAL FELLOWS Dr. Nobuko Taguchi Dr. Radinka Boskovic Dr. Yaron Finkelstein Dr. Tali Finkelstein Dr. Facundo Garcia Bournissen Division of Clinical Pharmacology & Toxicology OVERVIEW The division is one of the two major training sites of PUBLICATIONS The Division of Clinical Pharmacology & Toxicology the Royal College-accredited Clinical Pharmacology Avner M, Finkelstein Y, Hackam D, Koren G: focusses on a wide range of drug-related issues in Residency Program of the University of Toronto. Our Establishing causality in pediatric adverse drug paediatrics. The division is one of the largest and one medical residency training program spearheaded the reactions: use of the Naranjo probability scale. of the most active paediatric pharmacology programs recent change of the specialty residency training Paediatric Drugs 2007: 9: pp 267-270. internationally. Our major clinical activities include structure to add Emergency Medicine to the existing a consultation service, the Motherisk program, the four entry disciplines, which include Paediatrics. This Bar-Oz B, Einarson T, Einarson A, Boskovic R, Ontario Regional Poison Information Centre, and the change enables us to train residents with emergency O’Brien L, Malm H, Berard A, Koren G: Paroxetine DART (Drug Allergy, Reactions and Toxicity) clinic in medicine background in the clinical toxicology stream and congenital malformation: meta-analysis and collaboration with the Division of Immunology/Allergy of our education program. consideration of potential confounding factors. which provides specialized outpatient follow up and Clinical Therapeutics 2007: 29: pp 918-926. management for those with various drug/toxin-related Our research programs focus on drug metabolizing problems. The two toxicology information centres enzymes and their regulation, drug transporters, Bennett HA, Einarson A, Taddio A, Koren G, Einarson TR: receive annually about 35,000 (Motherisk) and placental and mammary gland drug transport and Depression during pregnancy: overview of clinical 75,000 (Poison Information Centre) inquiries which metabolism, and reproductive toxicology and factors. Clinical Drug Investigation 2007: 24: pp 157-179. have reached the limit of the response capacity of teratology, including fetal alcohol syndrome research. the systems. The Motherisk program, directed by The breadth and depth of these research programs Berard A, Azoulay L, Koren G, Blais L, Perreault S, Dr. Gideon Koren, continues disseminating new are shown in the publication and funding records. Oraichi D: Isotretinoin, pregnancies, abortions and breastfeeding babies. The Ontario Regional Poison HONOURS AND AWARDS Journal of Clinical Pharmacology 2007: 63: pp 196-205. Information Centre led by Dr. Margaret Thompson Koren G: Spirit of the Community Humanitarian Award, has now become a hub for medical toxicology training, Toronto, 2007. Bozzo P, Koren G, Nava-Ocampo AA, Einarson A: The internationally. The Palliative and Bereavement Care Koren G: Canadian Society for Clinical Pharmacology, a comparison between depressed women treated with Service directed by Dr. Christine Newman has recently Education Award, 2007. antidepressants and non-depressed women. Clinical knowledge in the field of drug safety for fetus and birth defects: a population-based perspective. British attracting many residents and fellows nationally and incidence of nausea and vomiting of pregnancy (NVP): become a part of the division and provides expertise specifically on drug therapy for terminally ill patients. and Investigative Medicine 2007: 29: pp 347-350. Koren G: Schwartz-Riesman Visiting Professorship, Tel Aviv University, 2007. CLINICAL FELLOWS Dr. Parvaneh Yazdanibrojeni Dr. Miho Inoue Dr. Toshihiro Tanaka Dr. Saskia de-Wildt Dr. Dan Rhodes Dr. Dr. Dr. Dr. Arthur Staroselsky Alla Osadchy Aleksey Kazmin Chagit Klieger-Grossman RESEARCH FELLOWS Dr. Alejandro A. Nava-Ocampo Dr. Shin-ichiro Takahashi Dr. Nada Djokanovic Dr. Naziha Mahmoud RESEARCH ASSOCIATE Katarina Aleksa RESIDENT Dr. Lisa Thurgur ADMINISTRATIVE STAFF Niki Balamatsis Hila Halshtok Leah Moscato Sarah Bondy Donna Tedesco Chambers C, Koren G, Tutuncu ZN, Johnson D, de Wildt SN, Koren G: Authors’ reply. Paediatric Einarson TR, Koren G, Einarson A: Problems with Jones KL: Are new agents used to treat rheumatoid Anaesthesia 2008: 18: pp 273-274. maternal antidepressant treatment and neonatal outcomes study. Archives of General Psychiatry arthritis safe to take during pregnancy? Organization of Teratology Information Specialists (OTIS) study. Dubnov-Raz G, Finkelstein Y, Koren G: Omega-3 fatty Canadian Family Physician 2007: 53: pp 409-412. acid supplementation during pregnancy: For mother, baby, or neither? Canadian Family Physician 2007: 53: Chen N, Aleksa K, Woodland C, Rieder M, Koren G: pp 817-818. 2007: 64: p 866. Einarson TR, Navioz Y, Maltepe C, Einarson A, Koren G: Existence and severity of nausea and vomiting in pregnancy (NVP) with different partners. Journal of N-Acetylcysteine prevents ifosfamide-induced Obstetrics and Gynaecology 2007: 27: pp 360-362. nephrotoxicity in rats. British Journal of Dugoua JJ, Perri D, Seely D, Mills E, Koren G: Pharmacology 2008: 153: pp 1364-1372. Safety and efficacy of blue cohosh (Caulophyllum thalictroides) during pregnancy and lactation. Etwel FA, Rieder MJ, Bend JR, Koren G: Chen N, Aleksa K, Woodland C, Rieder M, Canadian Journal of Clinical Pharmacology 2008: A surveillance method for the early identification Koren G: Prevention of ifosfamide nephrotoxicity 15: pp e66-e73. of idiosyncratic adverse drug reactions. Drug Safety 2008: 31: pp 169-180. by N-acetylcysteine: Clinical pharmacokinetic considerations. Canadian Journal of Clinical Pharmacology 2007: 14: pp 246-250. Chen N, Aleksa K, Woodland C, Rieder M, Koren G: Dugoua JJ, Seely D, Perri D, Koren G, Mills E: Safety and efficacy of chastetree (vitex agnus-castus) Feig DS, Briggs GG, Koren G: Oral antidiabetic agents during pregnancy and lactation. Canadian Journal in pregnancy and lactation: a paradigm shift? Annals of Clinical Pharmacology 2008: 15: pp e74-e79. of Pharmacotherapy 2007: 41: pp 1174-1180. The effect of N-acetylcysteine on ifosfamide-induced nephrotoxicity: In vitro studies in renal tubular cells. Dugoua JJ, Seely D, Perri D, Mills E, Koren G: Safety Finkelstein Y, Rezvani M, Garcia-Bournissen F, Translucent Research 2007: 150: pp 51-57. and efficacy of cranberry (vaccinium macrocarpon) Nurmohamed L, Koren G: Inactive pharmaceutical during pregnancy and lactation. Canadian Journal ingredients: implications for pregnancy. Canadian De Rosa MF, Ackerley C, Clarke D, Wang B, Ito S, of Clinical Pharmacology 2008: 15: pp e80-e86. Journal of Clinical Pharmacology 2007: 14: pp e17-e28. Lingwood C. Inhibition of multidrug resistance 1 (MDR1) by adamantylGb3, a globotraosylceramide analog. Einarson A, Koren G: Prescrire des antidépresseurs Journal of Biology Chemistry 2008: 283: pp 4501-4511. aux femmes enceintes: que doivent faire les médecins de famille? Canadian Family Physician 2007: 53: pp 1423-1425. 36 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Goh YI, Bollano E, Einarson TR, Koren G: Prenatal Finkelstein Y, Wahl MS, Bentur Y, Schechter T, Garcia-Bournissen F, Finkelstein Y, Rezvani M, Koren G: Garty BZ, Erickson TB, Chodick G, Cahana A, Exposure to alcohol-containing medications during multivitamin supplementation and rates of pediatric Mounstephen WJ, Koren G, Aks SE: Universal versus pregnancy. Canadian Family Physician 2007: 52: cancers: a meta-analysis. Clinical Pharmacology selective iron supplementation for infants and the pp 1067-1068. and Therapeutics 2007: 81: pp 685-691. a comparative study of two populations. Annals of Garcia-Bournissen F, Rokach B, Karaskov T, Koren G: Goh YI, Koren G: Folic acid in pregnancy and fetal Pharmacotherapy 2007: 41: pp 414-419. Cocaine detection in maternal and neonatal hair: outcomes. Journal of Obstetrics and Gynaecology implications to fetal toxicology. Therapeutic Drug 2008: 28: pp 3-13. risk of unintentional poisoning in young children: Florescu A, Ferrence R, Einarson TR, Selby P, Kramer M, Monitoring 2007: 29: pp 71-76. Goldman RD, Ong M, Wolpi J, Doyle J, Parshuram C, Woodruff S, Grossman L, Rankin A, Jacqz-Aigrain E, Koren G: Reference values for hair cotinine as a Garcia-Bournissen F, Shrim A, Koren G: Exposure Koren G: Pharmacological risk factors for biomarker of active and passive smoking in women to sibutramine during pregnancy. Canadian Family amphotericin B nephrotoxicity in children. Journal of reproductive age, pregnant women, children, and Physician 2007: 53: pp 229-230. of Clinical Pharmacology 2007: 47: pp 1049-1054. Gearing RE, Selkirk EK, Koren G, Leslie M, Motz M, Goldstein LH, Dolinsky G, Greenberg R, Schaefer C, Zelazo LB, McNeill T, Lozier FA: Perspectives of Cohen-Kerem R, Diav-Citrin O, Malm H, Reuvers- neonates: systematic review and meta-analysis. Therapeutic Drug Monitoring 2007: 29: pp 437-446. Forman R, Gill S, Moretti M, Tulandi T, Koren G, mothers with substance use problems on father Lodewijks ME, Rost van Tonningen-van Driel MM, Casper R: Fetal safety of letrozole and clomiphene involvement. Canadian Journal of Clinical Arnon J, Ornoy A, Clementi M, DiGianantonio E, Koren G, citrate for ovulation induction. Journal of Obstetrics Pharmacology 2008: 15: pp e99-e107. Braunstein R, Berkovitch M: Pregnancy outcome of women exposed to azathioprine during pregnancy. and Gynaecology Canada 2007: 29: pp 668-671. Gedeon C, Anger G, Piquette-Miller M, Koren G: Birth defects research, Part A. Clinical and Molecular Garcia-Bournissen F, Asrar F, Verjee Z, Karaskov T, Breast cancer resistance protein: mediating the Teratology 2007: 79: pp 696-701. Koren G: Contamination of hair with 3,4-methylene trans-placental transfer of glyburide across dioxymethamphetamine (ecstasy) in two young girls the human placenta. Placenta 2008: 29: pp 39-43. from a “meth lab.” Clinical Pediatrics (Philadelphia) 2008: 47: pp 186-188. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 37 Hancock RL, Koren G, Einarson A, Ungar WJ: The Kirkwood A, Harris C, Timar N, Koren G: Is gentamicin Koren G, Maltepe C: Preventing recurrence of severe effectiveness of Teratology Information Services (TIS). ototoxic to the fetus? Journal of Obstetrics and morning sickness. Canadian Family Physician 2007: Reproductive Toxicology 2007: 23: pp 125-132. Gynaecology Canada 2007: 29: pp 140-145. 52: pp 1545-1546. Huber AM, Tomlinson GA, Koren G, Feldman BM: Koren G, Chen N, Aleksa K: Drug-induced Koren G, Nulman I: Critical appraisal rounds: a new Amitriptyline to relieve pain in juvenile idiopathic nephrotoxciity in children: pharmacologically based training model for clinical pharmacologists. Canadian arthritis: a pilot study using Bayesian meta-analysis prevention of long-term impairment. Paediatric Journal of Clinical Pharmacology 2007: 14: pp e81-85. of multiple N-of-1 clinical trials. Journal of Drugs 2007: 9: pp 139-142. Koren G, Pairaideau N: Compliance with prenatal Rheumatology 2007: 34: pp 1125-1132. Koren G, Kramer M, Mattison D: The Summer vitamins. Patients with morning sickness sometimes Humphreys C, Garcia-Bournissen F, Ito S, Koren G: Institutes in Maternal-Fetal Pharmacology: building find it difficult. Canadian Family Physician 2007: 52: Expsoure to attention deficit hyperactivity disorder critical mass in a neglected area. Journal of Obstetrics pp 1392-1393. medications during pregnancy. Canadian Family and Gynaecology Canada 2007: 29: pp 544-545. Koren G: Changes in drug handling during pregnancy: Physician 2007: 53: pp 1153-1155. Koren G, MacLeod S, Davis D: Drugs in pregnancy: what it might mean for your patients. Canadian Family Physician 2007: 52: pp 1214-1215. Kalra S, Einarson A, Karaskov T, Van Uum S, Koren G: acknowledging challenges–finding solutions. Canadian The relationship between stress and hair cortisol in Journal of Clinical Pharmacology 2007: healthy pregnant women. Clinical Investigative 14: pp e2-e4. Koren G: Complementary and alternative medicine for children–time to enter the evidence-based era. Medicine 2007: 30: pp 103-107. Canadian Journal of Clinical Pharmacology 2007: 14: p e103. Liu GX, Zhou J, Koren G: Single-channel properties Dr. Shinya Ito and team of 1 (K, slow1) and I (K,slow2) in mouse ventricular myocytes. Pflugers Archives 2008: 456(3): pp 541-547. 38 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Kosuge K, Chuang A, Uematsu S, Tan KP, Ko BCB, O’Brien L, Schachtschneider AM, Koren G, Walker JH, Sauve B, Koren G, Walsh G, Tokmakejian S, Ohashi K, Ito S: Discovery of osmo-sensitive Einarson A: Longitudinal study of depression, anxiety, Van Uum SH: Measurement of cortisol in human transcriptional regulation of human cytochrome P450 irritability, and stress in pregnancy following evidence- hair as a biomarker of systemic exposure. Clinical 3As by the tonicity-responsive enhancer binding based counseling on the use of antidepressants. Investigative Medicine 2007: 30: pp 183-191. protein (nuclear factor of activated T cells 5). Journal of Psychiatry Practice 2007: 13: pp 33-39. Schechter T, Finkelstein Y, Doyle J, Verjee Z, Moretti M, Molecular Pharmacology 2007: 72: pp 826-837. Parshuram CS, To T, Seto W, Trope A, Koren G, Koren G, Dupuis LL: Pharmacokinetic disposition and Lau AC, Duong TT, Ito S, Yeung RSM: Matrix Laupacis A: Systematic evaluation of errors occurring clinical outcomes in infants and children receiving metalloproteinase-9 activity leads to coronary during the preparation of intravenous medication. intravenous busulfan for allogeneic hematopoietic aneurysms in an animal model of Kawasaki disease. Canadian Medical Association Journal 2008: 178: pp 42-48. stem cell transplantation. Biology Blood Marrow Transplant 2007: 13: pp 307-314. Arthritis and Rheumatism 2008: 58: pp 854-863. Pereg D, Koren G, Lishner M: The treatment of Madadi P, Koren G, Cairns J, Chitayat D, Gaedigk A, Hodgkin’s and non-Hodgkin’s lymphoma in pregnancy. Seely D, Dugoua JJ, Perri D, Mills E, Koren G: Safety Leeder JS, Teitelbaum R, Karaskov T, Aleksa K: Safety Haematologica 2007: 92: pp 1230-1237. and efficacy of panax ginseng during pregnancy and lactation. Canadian Journal of Clinical Pharmacology of codeine during breastfeeding: fatal morphine poisoning in the breastfed neonate of a mother Piwko C, Ungar WJ, Einarson TR, Wolpin J, Koren G: prescribed codeine. Canadian Family Physician 2007: The weekly cost of nausea and vomiting of pregnancy 53: pp 33-35. for women calling the Toronto Motherisk Program. 2008: 15: pp e87-e94. Shor S, Koren G, Nulman I: Teratogenicity of Current Medical Research Opinion 2007: 23: pp 833-840. lamotrigine. Canadian Family Physician 2007: 53: pp 1007-1009. Moretti ME, Verjee Z, Ito S, Koren G: Breastfeeding during maternal use of azathioprine. Annals of Ray JG, Vermeulen MJ, Koren G: Taking ACE inhibitors Pharmacotherapy 2007: 40: pp 2269-2272. during early pregnancy: is it safe? Canadian Family Shrim A, Garcia-Bournissen F, Koren G: Pharmaceutical agents and pregnancy in urology Physician 2007: 53: pp 1439-1440. practice. Urology Clinical North America 2007: Nava-Ocampo AA, Koren G: Human teratogens and evidence-based teratogen risk counseling: Rezvani M, Finkelstein Y, Verjee Z, Railton C, Koren G: the Motherisk approach. Clinical Obstetrics and Generalized seizures following topical lidocaine Gynaecology 2007: 50: pp 123-131. administration during circumcision: establishing 34: pp 27-33. causation. Paediatric Drugs 2007: 9: pp 125-127. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 39 Shrim A, Garcia-Bournissen F, Maxwell C, Farine D, Sung L, Tomlinson GA, Greenberg ML, Koren G, Judd P, Zhu M, Gach AA, Liu G, Xu X, Lim CC, Zhang J, Mao L, Koren G: Favorable pregnancy outcome following Ota S, Feldman BM: Serial controlled N-of-1 trials of Chuprun JK, Koch WJ, Liao R, Koren G, Blaxall BC, Trastuzumab (Herceptin) use during pregnancy–case topical vitamin E as prophylaxis for chemotherapy- Mende U: Enhanced calcium cycling and contractile report and updated literature review. Reproductive induced oral mucositis in paediatric patients. function in transgenic hearts expressing constitutively Toxicology 2007: 23: pp 611-613. European Journal of Cancer 2007: 43: pp 1269-1275. active G (alpha) protein. American Journal of Physiology–Heart and Circulatory Physiology 2008: Shrim A, Garcia-Bournissen F, Maxwell C, Farine D, Sutander M, Garcia-Bournissen F, Koren G: Koren G: Trastuzumab treatment for breast cancer Hypothyroidism in pregnancy. Journal of Obstetrics during pregnancy. Canadian Family Physician 2008: and Gynaecology Canada 2007: 29: pp 354-356. 54: pp 31-32. 294(3): pp H1335-H1347. BOOKS AND BOOK CHAPTERS Addis A, Koren G: Safety of fluoxetine during the first Taguchi N, Mian M, Shouldice M, Karaskov T, Gareri J, trimester of pregnancy: a meta-analytical review of Sivojelezova A, Koren G, Einarson A: Glucosamine use Nulman I, Verjee ZH, Koren G: Chronic cocaine epidemiological studies. In: The Evidence-Based A-to-Z in pregnancy: an evaluation of pregnancy outcome. exposure in a toddler revealed by hair test. Clinical Clinician’s Pocket Guide, Part II, Section 4. McGraw Journal of Women’s Health 2007: 16: pp 345-348. Pediatrics 2007: 46: pp 272-275. Hill, New York, 2007. Stade B, Ungar WJ, Stevens B, Beyen J, Koren G: Tan KP, Yang M, Ito S: Activation of Nrf2 by toxic Addis A, Moretti EM, Syed AF, Einarson RT, Koren G: Cost of fetal alcohol syndrome disorder in Canada. bile acids provokes adaptive defense responses to Fetal effects of cocaine: an updated meta-analysis. Canadian Family Physician 2007: 53: pp 1303-1304. enhance cell survival at the emergence of oxidative In: The Evidence-Based, A-to-Z Clinician’s Pocket stress. Molecular Pharmacology 2007: 72: pp 1380-1390. Guide, Part II, Section 3. McGraw Hill, New York, 2007. American Gastroenterological Association Institute Yamada J, Stevens B, de Silva N, Gibbins S, Beyene J, Bar-Oz B, Hackman R, Einarson RT, Koren G: Staroselsky A, Garcia-Bournissen F, Koren G: medical position statement on the use of Taddio A, Newman C, Koren G: Hair cortisol as a Pregnancy outcome after cyclosporine therapy during gastrointestinal medication in pregnancy. potential biologic marker of chronic stress in hospitalized pregnancy: a meta-analysis. In: The Evidence-Based, Gastroenterology 2007: 132: pp 824-825. neonates. Neonatology 2007: 92: pp 42-49. A-to-Z Clinician’s Pocket Guide, Part II, Section 4. McGraw Hill, New York, 2007. Staroselsky A, Nava-Ocampo AA, Vohra S, Koren G: Hemorrhoids in pregnancy. Canadian Family Physician 2008: 54: pp 189-190. 40 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Bar-Oz B, Moretti EM, Mareels G, Van Tittelboom T, Bentur Y, Koren G: The common occupational Dolovich RL, Addis A, Vaillancourt JMR, Power JDB, Koren G: Reporting bias in retrospective exposures encountered by pregnant women. In: Koren G, Einarson RT: Benzodiazepine use in ascertainment of drug-induced embryopathy. In: The The Evidence-Based, A-to-Z Clinician’s Pocket Guide, pregnancy and major malformations or oral cleft: Evidence-Based, A-to-Z Clinician’s Pocket Guide, Part Part I. McGraw Hill, New York, 2007. meta-analysis of cohort and case-control studies. In: The Evidence-Based, A-to-Z Clinician’s Pocket Guide, II, Section 1. McGraw Hill, New York, 2007. Bonari L, Pinto N, Ahn E, Einarson A, Steiner M, Koren G: Part II, Section 4. McGraw Hill, New York, 2007. Ben David S, Einarson RT, Ben David Y, Nulman I, Perinatal risks of untreated depression during Pastuszak A, Koren G: The safety of nitrofurantoin pregnancy. In: The Evidence-Based, A-to-Z Clinician’s Einarson RT, Koren G, Mattice D, Schechter-Tsafriri C: during the first trimester of pregnancy: a meta- Pocket Guide, Part I. McGraw Hill, New York, 2007. Maternal spermicide use and adverse reproductive Pocket Guide, Part II, Section 4. McGraw Hill, New Burtin P, Taddio A, Ariburnu O, Einarson RT, Koren G: A-to-Z Clinician’s Pocket Guide, Part II, Section 4. York, 2007. Safety of metronidazole in pregnancy: a meta-analysis. McGraw Hill, New York, 2007. Bennett AH, Einarson A, Taddio A, Koren G, Einarson Guide, Part II, Section 4. McGraw Hill, New York, 2007. outcome: A meta-analysis. In: The Evidence-Based, analysis. In: The Evidence-Based, A-to-Z Clinician’s In: The Evidence-Based, A-to-Z Clinician’s Pocket Einarson RT, Leeder JS, Koren G: A method for meta-analysis of epidemiological studies. In: The RT: Prevalence of depression during pregnancy: systematic review. In: The Evidence-Based, A-to-Z Cohen-Kerem R, Railton C, Oren D, Lishner M, Evidence-Based, A-to-Z Clinician’s Pocket Guide, Clinician’s Pocket Guide, Part II, Section 2. McGraw Koren G: Pregnancy outcome following non-obstetric Part II, Section 1. McGraw Hill, New York, 2007. Hill, New York, 2007. surgical intervention. In: The Evidence-Based, Bentur Y, Koren G: Occupational exposures known A-to-Z Clinician’s Pocket Guide, Part II, Section 2. Fernandes O, Sabharwal M, Smiley T, Pastuszak A, McGraw Hill, New York, 2007. Koren G, Einarson T: Moderate to heavy caffeine consumption during pregnancy and relationship to to be human reproductive poisons. In: The EvidenceBased, A-to-Z Clinician’s Pocket Guide, Part I. Diav-Citrin O, Koren G: Direct drug toxicity to the fetus. spontaneous abortion and abnormal fetal growth: McGraw Hill, New York, 2007. In: The Evidence-Based, A-to-Z Clinician’s Pocket a meta-analysis. In: The Evidence-Based, A-to-Z Guide, Part I. McGraw Hill, New York, 2007. Clinician’s Pocket Guide, Part II, Section 3. McGraw Hill, New York, 2007. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 41 Fried S, Kozer E, Nulman I, Einarson RT, Koren G: Gilbert C, Valois M, Koren G: Pregnancy outcome after Hard LM, Einarson RT, Koren G: The role of Malformation rates in children of women with first-trimester exposure to metformin: a meta-analysis. acetaldehyde in pregnancy outcome after prenatal untreated epilepsy: a meta-analysis. In: The In: The Evidence-Based, A-to-Z Clinician’s Pocket alcohol exposure. In: The Evidence-Based, A-to-Z Evidence-Based, A-to-Z Clinician’s Pocket Guide, Guide, Part II, Section 4. McGraw Hill, New York, 2007. Clinician’s Pocket Guide, Part II, Section 3. McGraw Hill, New York, 2007. Part II, Section 2. McGraw Hill, New York, 2007. Goh YI, Bollano E, Einarson RT, Koren G: Prenatal Gareri NJ, Klein J, Koren G: Determination of prenatal multivitamin supplementation and rates of congenital Hemels EHM, Einarson A, Koren G, Lanctot LK, exposures: In: The Evidence-Based, A-to-Z Clinician’s anomalies: a meta-analysis. In: The Evidence-Based, Einarson RT: Antidepressant use during pregnancy Pocket Guide, Part I. McGraw Hill, New York, 2007. A-to-Z Clinician’s Pocket Guide, Part II, Section 4. and the rates of spontaneous abortions: a meta- McGraw Hill, New York, 2007. analysis. In: The Evidence-Based, A-to-Z Clinician’s disposition in the maternal-fetal unit. In: The Evidence- Goh YI, Koren G: Folic acid and congenital birth defects: York, 2007. Based, A-to-Z Clinician’s Pocket Guide, Part II. McGraw a review. In: The Evidence-Based, A-to-Z Clinician’s Hill, New York, 2007. Pocket Guide, Part I. McGraw Hill, New York, 2007. Pocket Guide, Part II, Section 4. McGraw Hill, New Gedeon C, Koren G: Gestational changes in drug Koren G, Nava-Ocampo AA, Moretti EM, Sussman R, Nulman I: Major malformations with valproic acid. In: Gilbert C, Mazzotta P, Loebstein R, Koren G: Fetal Gutzin J, Kozer E, Magee AL, Feig SD, Koren G: The Evidence-Based, A-to-Z Clinician’s Pocket Guide, safety of drugs used in the treatment of allergic The safety of oral hypoglycemic agents in the first Part II, Section 4. McGraw Hill, New York, 2007. rhinitis: a critical review. In: The Evidence-Based, trimester of pregnancy: a meta-analysis. In: The A-to-Z Clinician’s Pocket Guide, Part II, Section 4. Evidence-Based, A-to-Z Clinician’s Pocket Guide, Koren G: Bias against the null hypothesis in McGraw Hill, New York, 2007. Part II, Section 4. McGraw Hill, New York, 2007. maternal-fetal pharmacology and toxicology. In: The Evidence-Based, A-to-Z Clinician’s Pocket Guide, Part II, Section 1. McGraw Hill, New York, 2007. 42 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT McMartin IK, Chu M, Kopecky E, Einarson RT, Koren G: Koren G: Drugs in pregnancy: acknowledging Magee AL, Elran E, Bull BS, Logan A, Koren G: Risks challenges–finding solutions. In: The Evidence-Based, and benefits of β-receptor blockers for pregnancy Pregnancy outcome following maternal organic solvent A-to-Z Clinician’s Pocket Guide, Part I. McGraw Hill, hypertension: overview of the randomized trials. In: exposure: a meta-analysis of epidemiologic studies. New York, 2007. The Evidence-Based, A-to-Z Clinician’s Pocket Guide, In: The Evidence-Based, A-to-Z Clinician’s Pocket Part II, Section 4. McGraw Hill, New York, 2007. Guide, Part II, Section 3. McGraw Hill, New York, 2007. In: The Evidence-Based, A-to-Z Clinician’s Pocket Magee AL, Mazzotta P, Koren G: Evidence-based view Moretti EM, Bar-Oz B, Fried S, Koren G: Maternal Guide, Part I. McGraw Hill, New York, 2007. of safety and effectiveness of pharmacologic therapy hyperthermia and the risk for neural tube defects in for nausea and vomiting during pregnancy (NVP). In: offspring: systematic review and meta-analysis. In: Koren G: Risk of varicella infection during late pregnancy. The Evidence-Based, A-to-Z Clinician’s Pocket Guide, The Evidence-Based, A-to-Z Clinician’s Pocket Guide, In: The Evidence-Based, A-to-Z Clinician’s Pocket Part II, Section 4. McGraw Hill, New York, 2007. Part II, Section 2. McGraw Hill, New York, 2007. Koren G: Medication in pregnancy and breastfeeding. Guide, Part II, Section 2. McGraw Hill, New York, 2007. Makarechian N, Agro K, Devline J, Trepanier E, Nava-Ocampo AA, Talaie H, Koren G: Systematic Kozer E, Nikfar S, Costei A, Boskovic R, Nulman I, Koren G, Einarson RT: Association between review of randomized controlled trials and cohort Koren G: Aspirin consumption during the first moderate alcohol consumption during pregnancy and studies on pregnancy outcome following antiretroviral trimester of pregnancy and congenital anomalies: spontaneous abortion, stillbirth and premature birth: treatment in pregnancy. In: The Evidence-Based, a meta-analysis. In: The Evidence-Based, A-to-Z A meta-analysis. In: The Evidence-Based, A-to-Z A-to-Z Clinician’s Pocket Guide, Part II, Section 4. Clinician’s Pocket Guide, Part II, Section 4. Clinician’s Pocket Guide, Part II, Section 3. McGraw McGraw Hill, New York, 2007. McGraw Hill, New York, 2007. Hill, New York, 2007. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 43 Nikfar S, Abdollahi M, Moretti EM, Magee AL, Koren G: Nulman I, Sussman R, Farber N, Chitayat D, Koren G: Raman-Wilms L, Tseng Lin-in A, Wighardt S, Einarson RT, Use of proton pump inhibitors during pregnancy and Fetal malformations associated with drugs and Koren G: Fetal genital effects of first-trimester sex rates of major malformations: a meta-analysis. In: The chemicals: visualization by sonography/fetal MRI. In: hormone exposure: a meta-analysis. In: The Evidence-Based, A-to-Z Clinician’s Pocket Guide, Part The Evidence-Based, A-to-Z Clinician’s Pocket Guide, Evidence-Based, A-to-Z Clinician’s Pocket Guide, II, Section 4. McGraw Hill, New York, 2007. Part I. McGraw Hill, New York, 2007. Part II, Section 4. McGraw Hill, New York, 2007. Seto A, Einarson RT, Koren G: Evaluation of Nulman I, Izmaylov Y, Staroselsky A, Koren G: Park-Wyllie L, Mazotta P, Pastuszak A, Moretti EM, Teratogenic drugs and chemicals in humans. In: The Beique L, Hunnisett A, Friesen HM, Jacobson S, brompheniramine safety in pregnancy. In: The Evidence-Based, A-to-Z Clinician’s Pocket Guide, Part I. Kasapinovic S, Chang D, Diav-Citrin O, Chitayat D, Evidence-Based, A-to-Z Clinician’s Pocket Guide, Nulman I, Einarson RT, Koren G: Birth defects after Part II, Section 4. McGraw Hill, New York, 2007. McGraw Hill, New York, 2007. maternal exposure to corticosteroids: prospective Seto A, Einarson RT, Koren G: Pregnancy outcome Nulman I, Knittel-Kerem D, Koren G: Fetal Alcohol cohort study and meta-analysis of epidemiological Spectrum Disorder: the central nervous system studies. In: The Evidence-Based, A-to-Z Clinician’s following first trimester exposure to antihistamines: tragedy. In: The Evidence-Based, A-to-Z Clinician’s Pocket Guide, Part II, Section 4. McGraw Hill, New a meta-analysis. In: The Evidence-Based, A-to-Z Pocket Guide, Part I. McGraw Hill, New York, 2007. York, 2007. Clinician’s Pocket Guide, Part II, Section 4. McGraw Nulman I, Koren G: Epilepsy and pregnancy. Polygenis D, Wharton S, Malmberg C, Sherman N, In: The Evidence-Based, A-to-Z Clinician’s Pocket Kennedy D, Koren G, Einarson RT: Moderate alcohol Sivojelezova A, Gallo M, Bollano E, Boom H, Koren G: Hill, New York, 2007. consumption during pregnancy and the incidence The use of natural health products in pregnancy and of fetal malformations: a meta-analysis. In: The lactation. In: The Evidence-Based, A-to-Z Clinician’s Nulman I, Laslo D, Fried S, Uleryk E, Lishner M, Evidence-Based, A-to-Z Clinician’s Pocket Guide, Pocket Guide, Part I. McGraw Hill, New York, 2007. Koren G: Neurodevelopment of children exposed Part II, Section 3. McGraw Hill, New York, 2007. Guide, Part I. McGraw Hill, New York, 2007. in utero to treatment of maternal malignancy. In: The Evidence-Based, A-to-Z Clinician’s Pocket Guide, Part II, Section 4. McGraw Hill, New York, 2007. 44 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT von Dadelszen P, Ornstein MP, Bull SB, Logan AG, Genotype-specific approaches in therapeutics in Novel transcriptional control of human CYP3A. Koren G, Magee AL: Fall in mean arterial pressure children. Carlton B, Hayden M, Ito S. University of Ito S. Canadian Institute of Health Research and fetal growth restriction in pregnancy hypertension: British Columbia ($105,000 2005-2007) ($340,540 2007-2010) a meta-analysis. In: The Evidence-Based, A-to-Z Clinician’s Pocket Guide, Part II, Section 2. McGraw Hair analysis for cortisol and testosterone. Van Um S, Pharmacogenetics of adverse drug reactions in Hill, New York, 2007. Koren G. The Physicians Services Incorporated children. Carlton B, Hayden M, Ito S, Koren G. Foundation ($130,000 2006-2008) Genome Canada ($350,000 2005-08) Determinants of renal handling of drugs during Improving prenatal vitamins to women. Koren G. The role of placenta in fetal toxicology. Koren G. development. Koren G. Canadian Institutes of Health Duchesnay Incorporated ($400,000 2004-2007) FUNDING Canadian Institutes of Health Research ($584,309 2007-2011) Research ($583,800 2003-2008) Neurodevelopment after fetal exposure to supradose Drugs and human milk. Ito S. Canadian Institutes of diclectin. Koren G, Nulman I. Duchesnay Incorporated Health Research ($376,323 2007-2010) ($480,000 2005-2008) Drugs and human milk. Ito S. Canadian Institutes of New Emerging Team (NET) grant. Koren G, Brien J. Health Research ($645,998 2002-2007) Canadian Institutes for Health Research ($1,250,000 2002-2007) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 45 Dr. Darcy Fehlings DIVISION HEAD Dr. Darcy Fehlings FULL-TIME Dr. Douglas Biggar Dr. Elizabeth Jimenez Dr. Dr. Dr. Dr. Dr. Dr. Tamarah Kagan-Kushnir Alvin Loh Laura McAdam Anne Kawamura Golda Milo-Manson Angela Orsino Dr. Wendy Roberts Dr. Jay Rosenfield Dr. Peter Rumney PART-TIME AND/OR COMMUNITY PHYSICIANS Dr. Janine Flanagan Dr. Cynthia Goldfarb Dr. Mark Handley-Derry Dr. Nicola Jones-Stokreef Dr. Peggy Kirkpatrick Dr. Donal O’Leary Dr. Anne Richards Dr. Clive Schwartz Dr. David Shawn Dr. Diann Superina CROSS-APPOINTEES/ SCIENTIFIC STAFF Dr. Colin Macarthur Dr. Patricia McKeever Division of Developmental Paediatrics OVERVIEW The division members actively lead and participate in PUBLICATIONS The Division of Developmental Paediatrics is situated applied clinical research in child development and Angus J, Paszat LF, McKeever P, Trebilcock A, Shivji F, at Bloorview Kids Rehab. The division consists of rehabilitation, in association with the Bloorview Edwards B: Pathways to breast cancer diagnosis 11 full-time physicians as well as a strong community Research Institute led by Dr. Colin MacArthur. Two and treatment: exploring the social relations of section of developmental paediatricians. The main exciting projects initiated this year are the CIHR-funded diagnostic delay. Texto and Contexto Enfermagem clinical focus of the division is on childhood research evaluating the long-term effectiveness of 2007: 16(4): pp 591-598. developmental disorders such as cerebral palsy, botulinum toxin injections in children with cerebral and autism spectrum disorders. In addition, there palsy, and the identification of Toronto as a research Au H, Harrison M, Ahmet A, Orsino A, Beck C, Tallett S, is a strong rehabilitation component with the acquired site in the North American Autism Treatment Network. Gans M, Birken C: Residents as health advocates: year, the division welcomed Dr. Angela Orsino, a HONOURS AND AWARDS a child advocacy initiative at the University of Toronto. developmental paediatrician whose clinical expertise Fehlings D: Inaugural Holder, Bloorview Children’s Paediatrics and Child Health 2007: 12(7): pp 567-571. brain injury and neuromuscular programs. This focusses on children with autism spectrum disorders. the development, implementation and evaluation of Hospital Foundation/University of Toronto Chair in Developmental Paediatrics, 2007. Within the division, there is a strong commitment Bonati MT, Russo S, Finelli P, Valsecchi MR, Cogliati F, Cavalleri F, Roberts W, Elia M, Larizza L: Evaluation of to education. Under the leadership of Dr. Golda Macarthur C: Inaugural Holder, Bloorview Children’s Milo-Manson as program director, the fully accredited Hospital Foundation/University of Toronto Chair in autism traits in Angelmans syndrome: a resource to unfold autism genes. Neurogenetics 2007: 8(3): subspecialty residency program in Developmental Paediatric Rehabilitation, 2007. pp 169-178. also a strong commitment to training paediatric McKeever P: Inaugural Holder, Bloorview Kids Carnevale FA, Macdonald ME, Bluebond-Langner M, and psychiatry residents and medical students Foundation/University of Toronto Childhood McKeever P: Using participant observation in pediatric in applied child development. Chair in Disability Studies, 2007. Paediatrics is the largest in Canada. There is health care settings: ethical challenges and solutions. Journal of Child Health Care 2008: 12(1): pp 18-32. An exciting event for the division was the creation in Roberts W: Division of Developmental 2007 of the Bloorview Children’s Hospital Foundation/ Paediatrics Faculty Teaching Award, 2008. University of Toronto Endowed Chair in Developmental Paediatrics. Dr. Darcy Fehlings, Division Head, is the Schwartz C: Dr. R H A Haslam Pre-Clerkship Teaching inaugural holder of this leadership chair. Award for Excellence in Pre-Clerkship Teaching, 2008. FELLOWS Dr. Ghassan Abu Kuwaik Dr. Kassia Johnson Dr. Sharon Smile Dr. Lyn Sonnenberg ADMINISTRATIVE STAFF AT BLOORVIEW KIDS REHAB Merlene Chin Deborah Horner Joanne Jenah Romana Karnetova ADMINISTRATIVE STAFF Maureen Hodge Barbara Zimnowodzki Epstein I, Stevens S, McKeever P, Baruchel S: Using a puppet to elicit talk with children. Nursing Inquiry 2008: 15(1): pp 49-56. Huertas-Ceballos, Logan S, Bennett C, Macarthur C: Gibbins S, Stevens S, McGrath P, Yamada J, Beyene J, Harrop A, Brant R, Ghali W, Macarthur C: Injury Breau L, Camfield C, Finley A, Franck L, Johnston C, mortality rates in native and non-native children: Psychosocial interventions for recurrent abdominal Howlett A, McKeever P, O’Brien K, Ohlsson A: a population-based study. Public Health Reports pain (RAP) and irritable bowel syndrome (IBS) in Comparison of pain responses in infants of different 2007: 122: pp 339-346. childhood. Cochrane Database Systems Review 2008: 1: CD003014. gestational ages. Neonatology 2008: 93: pp 10-18. Hewitt A, Macarthur C, Raina P: The role of evidence Gibson B, McKeever P: Men on the margin: in public health policy: an example of linkage and Kingsnorth S, Healy H, Macarthur C: Preparing for a Bourdieusian examination of living into adulthood exchange in the prevention of scald burns. Health adulthood: a systematic review of life skill programs with muscular dystrophy. Social Science and Care Policy 2007: 3: pp 59-66. for youth with physical disabilities. Journal of Adolescent Health 2007: 41: pp 323-332. Medicine 2007: 65(3): pp 505-517. Hudak P, McKeever P, Wright JG: Unstable embodiments: a phenomenological interpretation of Kolski HK, Hawkins C, Zatz M, de Flavia P, Biggar WD, technology, and place: social and ethical implications patient satisfaction with treatment outcome. Journal Alman B, Vajsar J: Diagnosis of limb-girdle muscular of long-term dependency on medical devices. Ethics, of Medical Humanities 2007: 28(1): pp: 1041-3545. dystrophy 2A by immunohistochemical techniques. Gibson B, Upshur R, Young N, McKeever P: Disability, Neuropathology 2008: 28(3): pp 264-268. Place and Environment 2007: 10(1): pp 7-28. Huertas-Ceballos, Logan S, Bennett C, Macarthur C: Guerriere D, Wong A, Croxford R, Leong V, McKeever P, Dietary interventions for recurrent abdominal pain Moessner R, Marshall CR, Sutcliffe JS, Skaug J, Coyte P: Costs and determinants of privately-financed (RAP) and irritable bowel syndrome (IBS) in childhood. Pinto D, Vincent J, Zwaigenbaum L, Fernandez B, home-based health care in Ontario, Canada. Health Cochrane Database Systems Review 2008: 1: Roberts W, Szatmari P, Scherer SW: SHANK3 and Social Care in the Community 2008: 16(2): CD003019. mutations to autism spectrum disorder. American Journal of Human Genetics 2007: 81(6): pp 1289-1297. pp 126-136. Huertas-Ceballos, Logan S, Bennett C, Macarthur C: Han R, Ungar W, Macarthur C: Cost effectiveness Pharmacological interventions for recurrent abdominal Narayanan U, Knights S, Weir S, Livingston M, analysis of a proposed public health legislative/ pain (RAP) and irritable bowel syndrome (IBS) in Fehlings D: Does the health status of caregivers educational strategy to reduce tap water scald injuries childhood. Cochrane Database Systems Review 2008: influence their perception of the health-related quality in children. Injury Prevention 2007: 13: pp 248-253. 1: CD003017. of life of children with severe non-ambulatory cerebral palsy? Developmental Medicine and Child Neurology 2007: 49(111): pp 31-21. 48 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Narayanan U, Weir S, Smith J, Fehlings D: Impact of Stevens B, McGrath P, Gibbins S, Beyene J, Breau L, Yantzi NM, Rosenberg MW, McKeever P: Getting out of comorbidities on the health-related quality of life of Camfield C, Finley A, Franck L, Howlett A, Johnston C, the house: the challenges mothers face when children children with severe non-ambulatory cerebral palsy. McKeever P, O’Brien K, Ohlsson A, Yamada J: have long-term care needs. Health and Social Care in Developmental Medicine and Child Neurology 2007: Determining behavioural and physiological responses the Community 2007: 15(1): pp 45-55. 49(111): p 68. to pain in infants at risk for neurological impairment. BOOKS AND BOOK CHAPTERS Pain 2007: 127: pp 94-102. Peter E, Spalding K, Kenny N, Conrad P, McKeever P, England K, Eakin J, Gastaldo D, McKeever P: Macfarlane A: Neither seen nor heard: children and Sutcliffe TL, Gaetz WC, Logan WJ, Cheyne DO, Neoliberalizing home care: managed competition and home care policy in Canada. Social Science and Fehlings DL: Cortical reorganization after modified restructuring home care in Ontario. In Neo-liberalization: Medicine 2007: 64(8): pp 1624-1635. constraint-induced movement therapy in pediatric States, Networks, Peoples (England K, Ward K, eds.). hemiplegic cerebral palsy. Journal of Child Neurology Blackwell Publishing Ltd., London, UK, 2007. Pope E, Krafchik B, Macarthur C, Stempak D, 2007: 22(11): pp 1281-1287. FUNDING Stephens D, Weinstein M, Ho N, Baruchel S: Oral versus high-dose pulse corticosteroids for problematic Tallett S, Lingard L, Leslie K, Pirie J, Jeffries A, A molecular genetic study of autism and related infantile hemangiomas: a randomized, controlled trial. Spero L, Schneider R, Hilliard R, Rosenfield J, phenotypes in extended pedigrees. Szatmari P, Hellman J, Mian M, Hurley J: Measuring educational Zwaigenbaum L, Scherer S, Vincent J, Roberts SW, workload: a pilot study of paper-based and PDA tools. Goldberg J, Mahoney W, Fernandez B. Canadian Medical Teacher 2008: 30(3): pp 296-301. Institutes of Health Research ($550,000 2006-2011) chronic abdominal pain: role of selected laboratory Wright V, Rosenbaum P, Goldsmith C, Law M, Fehlings An evaluation of the long-term effectiveness of investigations (stool ova and parasites, complete D: How do changes in body functions/structures, botulinum toxin in children with spastic cerebral palsy. Fehlings D, Goldsmith C, Narayanan U, Rosenbaum P, Pediatrics 2007: 119: pp 1239-1247. Soon G, Saunders N, Ipp M, Sherman PM, Macarthur C: Community-based case-control study of childhood blood count, and erythrocyte sedimentation rate). activity, and participation relate? A study of children Journal of Pediatric Gastroenterology and Nutrition with cerebral palsy (CP). Developmental Medicine and Wright W. Canadian Institutes of Health Research 2007: 44: pp 524-526. Child Neurology 2008: 50: pp 283-289. ($425,956 2008-2012) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 49 A qualitative observational study of resident oral Books for siblings of children with autism. Johnson K, Critical reflections on ambulatory training for children paediatric developmental case presentations. McKeever P, Kawamura A. Bloorview Research with cerebral palsy. Gibson B, Wright V, Yong NL, Orsino A, Kawamura A, Lingard L, Fehlings D. Institute Seed Grant ($5,500 2007-2008) McKeever P, McLaren C, Fehlings D. Health Care, Technology and Place: Interdisciplinary Capacity Bloorview Research Institute ($10,553 2008-2009) Canadian Injury Indicators Development, Phase II. Enhancement Team Grant ($8,000 2007-2008) Autism genome project. Scherer SW, Szatmari P, Pike I, Macpherson A, Macarthur C, Warda L, Babul S, Paterson AD, Vincent JB, Zwaigenbaum L, Roberts W, Goodman D, Raina P. Canadian Institutes of Health Development of an indicator of the impact of assistive Wellington SR, Fernandez BA, Miller FA, Hallmayer JF. Research ($225,000 2008-2011) devices on children with disabilities and their families. Ryan S, Campbell K, McKeever P, Montpetit K, Genome Canada ($15,611,534 2005-2009) Cerebral Palsy Outcomes Project (CPOP). Fehlings D, Perkin S, Rigby P, Roxborough L, Stevens J. Canadian Autism spectrum disorders: pathways to better Kolaski K, Gaebler-Spira D, Kuroda M, Msall M, Institutes of Health Research ($225,000 2008-2011) outcomes. Szatmari P, Bryson SE, Fombonne E, Thornton L, Moberg-Wolff E, Krach L, Hurvitz E. and Research Institute ($100,000 2008-2011) Zwaigenbaum L, Smith I, Waddell C, Vaillancourt T, American Academy for Cerebral Palsy and Mirenda P, Roberts W, Volden J. Canadian Institutes Developmental Medicine ($25,000 2007-2008) Discriminating autism from other atypical patterns of development in a community sample. Roncadin C, of Health Research ($2,125,000 2004-2009) Clowning around: the effects of therapeutic clowning Roberts W, Niccols A, Brian J, Bryson S, Smith I, Autism Treatment Network: Co-operative multicentre on patients and nurses in a paediatric rehabilitation Zwaigenbaum L. The Ontario Mental Health program for research and treatment of autism. facility. Kingsnorth S, McKeever P, Blaine S. Bloorview Foundation (Type A Grant) ($142,005 2008-2010) Roberts W, Loh A, Kawamura A, Feigenbaum A, Children’s Hospital Foundation ($24,985 2008-2009) Early developmental trajectories in autism. Weiss S, Marcon C, Goldfarb C, Berall G, Bradley E, Brian J, Freeman N, Atkinson A, Taylor M, Stinson J, Costs incurred by families of children newly diagnosed Zwaigenbaum L, Bryson SE, Roberts S, Smith I, Mitchell S, Kawamura A, Green P. Autism Speaks with cancer. Stevens B, Tsimicalis A, Greenberg M, Szatmari P, Vaillancourt T. Canadian Institutes of ($450,000 2008-2010) McKeever P, Ungar W, Guerriere D, Agha M. National Health Research ($932,585 2006-2011) Cancer Institute of Canada ($330,712 2006-2008) 50 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Enhancing inter-subjectivity in infants at high risk for From knowledge generation to knowledge translation: Genome wide DNA methylation profiling in autism. autism. Bryson S, Brian J, Smith I, Zwaigenbaum L, a systems approach to reducing the burden of injury in Weksberg R, Roberts SW, Scherer S. Canadian Roberts SW. Autism Speaks ($638,006 2007-2010) Canada. Macarthur C, Raina P. Canadian Institutes of Institutes of Health Research ($649,518 2008-2011) Health Research ($942,361 2003-2008) HCTP & The Change Foundation Strategic Alliance. Evaluating outcomes associated with multidisciplinary care for children with cerebral palsy using clinical Functional status outcome indicators for young McKeever P, Coyte PC. The Change Foundation practice improvement (CPI) methodology. Damiano D, children with cerebral palsy: evaluation of the (2003-2008 $200,000) Chambers H, Revivo G, Brunstrom J, Horn S, comparative abilities of two internationally-recognized Gassaway J, Krach L, Gormley M, Novacheck T, measures to detect change, and determination of Identity, embodiment and heart transplantation: Matthews D, Chang F, Stevenson R, Abel M, benchmarks for clinically important change. Wright V, a phenomenologically-informed exploration. Ross H, McLaughlin J, Bjornson K, Rathjen K, Delgado M, Stratford P, Goldsmith C, Rigby P, Fehlings D, McKeever P, Shildrick M, Poole J. Advanced Heart Dabrowski E, Gaebler-Spira D, Dias L, Narayanan U, Rosenbaum P. Canadian Institutes of Health Failure Fund (2008-2009 $75,000) Fehlings D, Graham K, Koman A. American Academy Research ($174,565 2008-2010) Inappropriate living environments for children for Cerebral Palsy and Developmental Medicine Clinical Research Planning Grant ($29,000 2006-2007) Functional outcomes following orthopaedic surgery with acquired brain injury. Law M, Colantonio A, based on gait laboratory versus observational gait DeMatteo C, Macarthur C. Ontario Neurotrauma Evaluation of the reliability, validity and analysis in ambulatory children with cerebral palsy: Foundation ($150,000 2005-2007) responsiveness of the Kinematic Dystonia measure in a randomized controlled trial. Narayanan U, Willan A, children with upper extremity dystonia. Kawamura A, Wedge J, Fehlings D. Pediatric Orthopaedic Society Fehlings D, Morris A, Campbell K. Bloorview Research of North America (POSNA) ($31,000 2006-2008) Interdisciplinary capacity enhancement grant for “Health Care, Technology and Place”: a CIHR Centre for Research Development. Coyte PC, McKeever P. Institute Seed Grant ($16,652 2007-2008) Functional outcomes following orthopaedic surgery Canadian Institutes of Health Research Evaluation of quality of movement in ambulatory based on gait laboratory versus observational gait ($1,000,000 2003-2008) children with cerebral palsy: validity and sensitivity analysis in ambulatory children with cerebral palsy: to change of the quality FM. Rosenbaum P, a pilot randomized controlled trial. Narayanan U, Wright V, Mesterman R, Fehlings D, Kim M. Fehlings D, Willan A, Wedge J, Redekop S. McKeever P, Hodnett E, Carter M, Vanderby S, Physicians’ Services Incorporated Foundation Bloorview Research Institute Seed Grant Adams A, Theodore D, Jewell S, Koziel N. ($125,000 2007-2009) Competition ($25,015 2006-2007) Philips Electronics ($134,578 2006-2008) Kids’ ambient MRI suite conversion study. Babyn P, THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 51 Medical Technologies Research and Commercialization Role of telecommunication in augmentative The efficacy of an intelligent prompting system to Collaborative. Phipps D, Macarthur C, Boehm L, and alternative communications. Macarthur C. develop self-care activities in children with autism. Duschenne-Smith J. Canadian Institutes of Health Bell Canada ($150,000 2005-2007) Mihailidis A, Roberts SW, Polatajko H, Chau T. SickKids Foundation ($129,470 2007-2009) Research, National Sciences and Engineering Research Council of Canada and Social Sciences and School, home and neighbourhood accessibility: Humanities Research Council ($615,000 2005-2008) physically disabled children’s assessments (The The experience for autism over the course of paediatric ScHaN Project). McKeever P, Ruddick S, Dunn J, Yantzi development. Nicholas D, Roberts SW, McKeever P. Long-term follow-up of boys with DMD. N, Young N. Canadian Institutes of Health Research SickKids Foundation ($129,821 2008-2010) Leshner R, Biggar D. Co-operative International ($360,768 2008-2011) The experience of maternal and family care for Neuromuscular Research Group Washington, DC ($400,000 2007-2012) Strategic Training Program Grant for “Health Care, autism over the course of paediatric development. Technology and Place.” McKeever P, Coyte PC. Nicholas D, Roberts SW, McKeever P. Social Neuroimaging and frontal lobe function in children Canadian Institutes of Health Research Sciences and Humanities Research Council with autism. Taylor M, Roberts SW, Donner EJ, ($2,146,250 2002-2009) of Canada ($154,000 2008-2010) The development and evaluation of the HAT-D: a The lived experiences of mothers of children with discriminative tool differentiating types of hypertonia autism. Nicholas D, Roberts SW, McKeever P. Outcomes research in paediatrics. Parkin P, in children. Fehlings D, Jethwa A, Macarthur C. Social Sciences and Humanities Research Macarthur C. SickKids Foundation ($750,000 2008-2013) Bloorview Research Institute ($9,606 2006-2007) Council of Canada ($154,000 2008-2011) Pain in infants with disabilities: a program of The development of a virtual reality therapy home- The paediatric hospital atrium: designers’ intentions Bayless S, Shroff M. National Alliance Autism Research ($115,907 2006-2008) research, phase II. Stevens B, McGrath P, Beyene J, based system to improve hand usage and function versus children’s experiences. Adams A, McKeever P, Breau LM, Camfield CS, Finley GA, Gibbins S, in children with hemiplegia. Fehlings D, Chau T, Spalding K. Canadian Institutes of Health Research Howlett A, Johnston C, McKeever P, O’Brien K, Lam-Damji S, Tse K. Bloorview Research Institute ($291,376 2005-2008) Ohlsson A. Canadian Institutes for Health Research Seed Grant ($24,972 2006-2008) ($676,202 2003-2008) 52 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT The reliability and validity of the Paediatric Autism Validation of accelerometry as a measure of physical Early Diagnosis Tool (PAED) –a pilot project. Roberts W, activity and inactivity in children with chronic diseases. Green P, Shouldice M, Wright V. Bloorview Research Feldman B, Stephens S, Schneidermna-Walker J, Institute Seed Grant ($17,400 2006-2008) Biggar WD, McCrindle B, Longmuir P. Canadian Institutes of Health Research ($60,000 2008-2009). Training program in autism research. Fombonne E, Bryson S, Paterson A, Scherer S, Roberts W, Western Canada standards of care for Duchenne Szatmari P, Smith I, Moore C. Canadian Institutes muscular dystrophy. Mah J, Moore M, Biggar WD. of Health Research, National Alliance for Autism Porticus Foundation ($23,125 2008) Research, Fonds de la Recherche en Santé du Québec ($1,440,000 2003-2009) Women’s income levels and diagnostic delay in breast cancer. Angus J, Bondy S, Paszat L, McKeever P. Understanding chronic pain in infancy. Stevens BJ, Social Sciences and Humanities Research Council Asztalos E, McKeever P, Gibbins S, Pillai Riddell R, of Canada ($90,620 2004-2007) Katz J. Canadian Institutes of Health Research (2005-2008 $141,674) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 53 Dr. Mark Palmert DIVISION HEAD Dr. Mark R. Palmert Dr. Etienne Sochett Dr. Diane Wherrett FULL-TIME Dr. Denis Daneman Dr. Jill Hamilton Dr. Kusiel Perlman PART-TIME Dr. Jacqueline Curtis Dr. Susan Kirsch Dr. Stacey Urbach GYNAECOLOGY Dr. Lisa Allen Dr. Nicolette Caccia Dr. Sari Kives Dr. Melanie Ornstein GYNAECOLOGY FELLOW Dr. Kerith Lucco HONORARY Dr. Robert Ehrlich CROSS-APPOINTEES Dr. Amira Klip Dr. Joanne Rovet SUBSPECIALTY RESIDENTS/ CLINICAL FELLOWS Dr. Abdulla Aljunaibi Dr. Bojana Babic Dr. Corinna Grasemann Dr. Ara Healey Dr. Dror Koltin Dr. Clodagh O’Gorman Dr. Constantine Samaan Division of Endocrinology OVERVIEW Recent initiatives include the development of HONOURS AND AWARDS 2007-08 was a highly productive time for the Division pathways for the care of children with thyroid cancer Allen L: University of Toronto Department of of Endocrinology with ongoing development of existing in collaboration with Oncology, Otolaryngology and Obstetrics and Gynaecology Chairs’ Award for programs and new innovations. We have had in 10 Nuclear Medicine; enhancement of our program Excellence in Undergraduate Teaching, 2007. trainees in our subspecialty training program: five designed to provide care to survivors of paediatric Canadian, two Irish, one Israeli, one Costa Rican cancer who develop endocrine abnormalities; further Hamilton J: NovoNordisk Young Investigator and one from the UAE. We also welcomed Dr. Mark development of our hypothalamic obesity program, with Career Development Award, 2008. Palmert as the new division head. inclusion of a small number of patients with multiple Our areas of clinical focus continue to be: (i) diabetes: medical problems stemming from being overweight; Hamilton J: The Hospital for Sick Children Department and establishment of a new focus on disorders of puberty. of Paediatrics Richard Rowe Junior Faculty Award for our comprehensive program provides ongoing care Excellence in Clinical Medicine, 2007. to nearly 1,000 children and teens with diabetes, Our research efforts have targeted the following including a program for comprehensive assessment areas: (i) prediction and prevention of Type 1 diabetes: Klip A: Editor-in-chief–The American Journal and treatment for adolescents with chronically our group is involved in the multinational TrialNet of Physiology–Endocrinology and Metabolism poor diabetes control; (ii) general endocrinology and TRIGR study groups; (ii) implications of insulin Physiology, 2007. emphasizing the areas of growth, thyroid disease, resistance and obesity in children and adolescents intersex conditions and reproductive health; (iii) and the evolution of these abnormalities in young Klip A: Glassman Lecturer, Hebrew University, calcium: the Calcium-Bone and Osteoporosis Clinics children; (iii) study of the factors affecting metabolic Jerusalem, 2008. assesses children with abnormal bone health from control and the early complications of diabetes; a wide variety of causes; and (iv) the gynaecology (iv) psychosocial and psychological outcomes in PUBLICATIONS section; which provides care to pre-teen and diabetes and other endocrine disorders; (v) evaluation Aanstoot H-J, Anderson BJ, Daneman D, Danne T, adolescent girls across a wide spectrum of disorders. of disorders of calcium and bone mineral metabolism Donaghue T, Kaufman F, Réa RR, Uchigata Y: The in paediatrics. A new focus is clinical and laboratory global burden of youth diabetes: perspectives and based investigation of complex genetic traits, with an potential, a charter paper. Pediatric Diabetes 2007: emphasis on identification of genes that regulate the 8: pp 1-44. timing of puberty. SUBSPECIALTY RESIDENTS/ CLINICAL FELLOWS Dr. Rayzie Shulman Dr. Julieta Solis Chacon RESEARCH FELLOWS Dr. Shazhan Amed Dr. Meranda Nakhla ADMINISTRATIVE STAFF Joyce Robinson Elizabeth Rodgers Minet Smith Zarine Tilak Díaz M, Antonescu CN, Capilla E, Klip A, Planas JV: Allen L, Brewer M, Bryson P, Evans G, Frappier JY, Cherney DZ, Miller JA, Scholey JW, Bradley T, Slorach C, Jamieson MA, Lynde C, Murphy KJ, Provencher DM, Curtis JR, Dekker MG, Nasrallah R, Hebert RL, Fish glucose transporter (GLUT) -4 differs from Shier M, Steben M, Lerch R, Lalonde A, Senikas V: Sochett EB: The effect of cyclooxygenase 2 inhibition rat GLUT-4 in its traffic characteristics but can Canadian consensus guidelines on human on renal hemodynamic function in humans with Type 1 translocate to the cell surface in response to insulin papillomavirus. Obstetrics and Gynaecology Canada diabetes. Diabetes 2008: 57 (3): pp 688-695. in skeletal muscle cells. Endocrinology 2007: 148: pp 5248-5257. 2007: 29: pp S1-S59. Colton P, Olmsted M, Daneman D, Rydall A, Rodin G: Amed S, Delvin E, Hamilton J: Variation in growth Five year prevalence and persistence of disturbed Daneman A, Daneman D: McCune-Albright syndrome. hormone immunoassays in clinical practice in Canada. eating behaviour and eating disorders in girls with type Journal of Pediatric Endocrinology and Metabolism Hormone Research 2008: 69: pp 290-294. 1 diabetes. Diabetes Care 2007: 30: pp 2861-2862. 2007: 20: p 1265. Antonescu CN, Díaz M, Femia G, Planas JV, Klip A: Colton P, Olmsted M, Daneman D, Rydall A, Rodin G: Dugani CB, Randhawa VR, Cheng AW, Patel N, Klip A: Clathrin-dependent and independent endocytosis Natural history and predictors of eating disturbances Selective regulation of the perinuclear distribution of of glucose transporter 4 (GLUT4) in myoblasts: in girls with type 1 diabetes. Diabetic Medicine 2007: glucose transporter 4 (GLUT4) by insulin signals in regulation by mitochondrial uncoupling. Traffic 2008: 24: pp 424-429. muscle cells. European Journal of Cell Biology 2008: 87(6): pp 337-351. April 24 [Epub ahead of print]. Conwell L, Forrest C, Allen U, Daneman D: Necrotizing Guilbert E, Broditsky R, Black A, Kives S, Leboeuf M, Cameron FJ, Northam EA, Ambler GR, Daneman D: fasciitis in adolescents with poorly controlled Type 1 Routine psychological screening in youth with type 1 diabetes mellitus: report of two cases. Pediatric Mirosh M, Senikas V, Wagner MS, Weir E, York-Lowry J, diabetes and their parents: a notion whose time has Diabetes 2007: 8: pp 397-400. Reid R, Trussell J: Canadian consensus guidelines on continuous and extended hormonal contraception. come? Diabetes Care 2007: 30: pp 2716-2724. Conwell L, Pope E, Artiles A, Mohanta A, Daneman A, Journal of Obstetrics and Gynaecology Canada 2007: Daneman D: Dermatological complications of 29: pp S1-S32. continuous subcutaneous insulin infusion (CSII) in children and adolescents. Journal of Pediatrics 2008: 152: pp 622-628. 56 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Hiraki LT, Hamilton J, Silverman ED: Measuring Koltin D, Daneman D: Dead-in-bed syndrome – Rachmiel M, Buccino J, Daneman D: Exercise permanent damage in pediatric systemic lupus a diabetes nightmare. Pediatric Diabetes 2008: and type 1 diabetes mellitus in youth: review erythematosus. Lupus 2007: 16: pp 657-662. May 21 [Epub ahead of print]. and recommendations. Pediatric Endocrinology Hodges CA, Palmert MR, Drumm ML: Infertility in Makitie O, Toiviainen-Salo S, Marttinen E, Kaitila I, females with cystic fibrosis is multifactorial: evidence Sochett E, Sipila I: Metabolic control and growth Rachmiel M, Kives S, Atenafu E, Hamilton J: Primary from mouse models. Endocrinology 2008: 149(6): during exclusive growth hormone treatment in X-linked amenorrhea as a manifestation of polycystic ovarian pp 2790-2797. hypophosphatemic rickets. Hormone Research 2008: syndrome in adolescents: a unique subgroup? 69(4): pp 212-220. Archives of Pediatrics and Adolescent Medicine 2008: Reviews 2007: 5: pp 656-665. 162: pp 521-525 HPV Consensus Guidelines Committee (Allen L, Brewer M, Bryson P, Evans G, Frappier JY, Jamieson MA, Nadkarni D, Spitzer RF, Colgan T, Kives S, Allen L: Lynde C, Murphy KJ, Provencher DM, Shier M, Uterine asymmetry and dysmenorrhea in a young Robertson D, Kives S: Adolescent consent in Steben M, Lerch R, Lalonde A, Senikas V): Canadian woman. Journal of Obstetrics and Gynaecology reproductive and sexual health decision-making: consensus guidelines on human papillomavirus. Canada 2008: 30: pp 301-302. should there be an arbitrary age of consent or should it be based on the “evolving capacities Journal of Obstetrics and Gynaecology Canada 2007: 29: pp S1-S59. Ishikura S, Koshkina A, Klip A: Small G proteins in O’Connor DL, Khan S, Wishuhm K, Vaughan J, of the child”? Journal of Pediatric and Adolescent Jefferies A, Campbell D, Asztalos E, Feldman M, Gynaecology 2008: 21: pp 47-51. Rovet J, Westall C, Whyte H: Growth and nutrient Roher N, Samokhvalov V, Díaz M, MacKenzie S, Klip A, insulin action: rab and rho families at the crossroads intakes of human milk-fed premature infants of signal transduction and GLUT4 vesicle traffic. Acta provided with extra energy and nutrients after Planas JV: The proinflammatory cytokine tumour Physiology 2008: 192: pp 61-74. hospital discharge: a pilot randomized controlled necrosis factor increases the amount of glucose trial. Pediatrics 2007: 121: pp 766-776. transporter-4 at the surface of muscle cells independently of changes in Interleukin-6. Endocrinology 2008: 149(4): pp 1880-1889. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 57 Segal P, Hamilton J, Sermer M, Connelly P, Hanley A, Wells GD, Noseworthy MD, Hamilton J, Tein I: Banerjee S, Tran K, Li H, Cimon K, Daneman D, Zinman B, Retnakaran R: Maternal obesity and familial Skeletal muscle dysfunction in obesity: new ideas Simpson S, Campbell K: Short-acting insulin analogs history of diabetes have opposing effects on infant and concepts. Canadian Journal of Neurological for diabetes mellitus: meta-analysis of clinical birthweight in women with mild glucose intolerance in Sciences 2008: 35: pp 31-40. outcomes and assessment of cost-effectiveness BOOKS AND BOOK CHAPTERS Technologies in Health, Ottawa, 2007. (Technology Report 87). Canadian Agency for Drug pregnancy. Journal of Maternal-Fetal and Neonatal Medicine 2008: 21: pp 73-79. Al-Abdulrazzaq D, Allen LM: Pediatric and adolescent Shulman DI, Francis GL, Palmert MR, Eugster EA, gynecology. In: The Hospital for Sick Children Caccia N, James A: Planned delivery by caesarean for the Lawson Wilkins Pediatric Endocrine Society Handbook of Pediatrics, 11th Edition. Elsevier, section may be hazardous for mother and baby. In: Drug and Therapeutics Committee: Use of aromatase Canada, 2007. AboutKidsHealth, www.aboutkidshealth.ca, 2008. Allen LM. Management issues in child gynecology. In: Hamilton J, Hozjan I: Lumps, bumps and other Progress in pediatric Urology, Volume 10 (Pajpai M, swellings: lymphatics and skin issues. In: Turner inhibitors in children and adolescents with disorders of growth and adolescent development. Pediatrics 2008: 121(4): pp 975-983. Gearhart JP, Mouriquand PDE, Mitra DK, Radhakrishan J, Syndrome: Across the Lifespan. (Hamilton J, Hozjan I, Spitzer R, Salle P, Wherrett D, Colgan T, Chitayat D, Mathews R, Etker S, eds.). Penwel Publishers, eds.). Castlemore Graphics, Brampton, Ontario, 2008. Dodge J, Allen L: Luteoma of pregnancy presenting New Delhi, 2008. with virilization in the female infant. Journal of Hamilton J, Hozjan I: Turner Syndrome: Across the Obstetrics and Gynaecology Canada 2007: 29: Amed S, Hamilton J: When sugar is not so sweet: Lifespan. Castlemore Graphics, Brampton, Ontario, 2008. pp 835-840. diabetes and the metabolic syndrome. In: Turner Syndrome: Across the Lifespan (Hamilton J, Hozjan I, Hodges CA, Palmert MR: Genetic regulation of the Spitzer RF, Caccia N, Kives S, Allen LM: Hysteroscopic eds.). Castlemore Graphics, Brampton, Ontario, 2008. variation in pubertal timing. In: When Puberty is unification of a complete obstructing uterine septum: Precocious: Scientific and Clinical Aspects Case report and review of the literature. Fertility and (Pescovitz OH, Walvoord EC, eds.). Humana Press Inc., Sterility 2008: March 5 [Epub ahead of print]. Totowa, NJ, 2007. 58 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Kives S, Lacey J. Normal menstrual physiology. Bypassing insulin resistance of muscle cells caused Comparison of continuous versus cyclical use of a In: Adolescent Health Care: A Practical Guide, by ceramide, oxidative stress and macrophage-co- 30-MCG ethinyl estradiol 0.15 mg desogestrel oral 5th Edition (Neinstein LS, Gordon CM, Katzman DK, culture. Klip A. Canadian Diabetes Association contraceptive pill in adolescents. Allen L, Brown C. Rosen DS, Woods ER, eds.). Lippincott Williams & ($300,000 2006-2009) Physicians’ Services Incorporated Foundation ($75,000 2006-2008) Wilkins, Philadelphia, 2007. Clinical Co-ordinating Centre for Epidemiology of Rovet J, Brown R. Congenital hypothyroidism. In: Diabetes Intervention and Complications (DCCT/EDIC). Cross-sectional study of overweight in paediatric Neurogenetic Developmental Disorders: Manifestation Palmert MR (Principal Investigator until August 2007; survivors of acute lymphoblastic leukaemia. Nathan and Identification in Childhood (Mazzocco M, Ross J, now Consultant). National Institutes of Health/ PC, Urbach SL, Barrera M, Barron M, D’Agostino N, eds.). MIT Press, Boston, 2007. National Institute of Diabetes and Digestive and Staneland P, Charron, Greenberg ML. The University Kidney Diseases ($54,312,694 2006-2016) of Toronto, Faculty of Medicine Dean’s Fund ($9,998 2007-2008) Tran K, Banerjee S, Li H, Cimon K, Daneman D, Simpson S, Campbell K. Long-acting insulin analogues Combined diazoxide and metformin therapy in for diabetes mellitus: meta-analysis of clinical children with hypothalamic obesity secondary Evaluation of hepatic function and autoimmunity in outcomes and assessment of cost-effectiveness. to craniopharyngioma: a pilot study. Hamilton J, girls with Turner Syndrome. Hamilton J, McAssey K, Canadian Agency for Drugs and Technologies in Daneman D, Ahmet A. Canadian Institutes of Milkiewicz P, Heathcote J, Norman G (Investigator Health, Ottawa, 2007. Health Research ($49,500 2006-2008) initiated study). Hoffman-La Roche Canada ($23,980 2006-2008) FUNDING Comparison of continuous versus cyclic use of a A phase IV open-label study of predictive markers in 30-MCG ethinyl estradiol 0.15 mg desogestrel oral Genetic factors that regulate the onset of puberty. growth hormone deficient and Turner Syndrome contraceptive pill in adolescents. Allen L, Brown C. Palmert MR. National Institutes of Health/National pre-pubertal children treated with SAIZEN. Hamilton J Azko Nobel – Organon Canada ($24,500 2006-2008) (Principal Investigator SickKids site). Serono Canada Institute of Child Health and Human Development ($1,633,256 2005-2010) Incorporated ($2,400 2005-2008) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 59 Growth hormone therapy and bone quality in Intracellular compartment plasticity and signal Post-marketing surveillance program for Nutropin. paediatric osteoporosis. Sochett E, Grynpas M. Eli transmission in insulin action. Klip A. Canadian Hamilton J. Genentech National Co-operative Growth Lilly Canada ($125,000 2006-2009) Institutes of Health Research ($759,560 2005-2010) Study (NCGS) ($45,300 2001-2008) Hemodynamic modulation in type 1 diabetes. Miller J, MRI analyses of visuospatial processing deficits Psychosocial adjustment in genetic males born with Sochett E, Cherney D. Canadian Institutes of Health in children born preterm. Rovet J, Desrocher M, ambiguous genitalia. Wherrett DK. The Hospital for McAndrews MP. March of Dimes Sick Children Research Institute Seed Grant (US$335,945 2007-2010) ($15,590 2001-2008) for glucose transport. Klip A. Canadian Institutes of Multiple courses of antenatal corticosteroids for The comprehensive care clinic for children with central Health Research ($643,800 2002-2007) preterm birth study: a five-year follow-up (MACS-5). tumours at The Hospital for Sick Children: evaluating Asztalos E, Rovet J. Canadian Institutes of Health the efficacy of an integrated, multidisciplinary model Research ($3,585,161 2005-2012) of care for children with hypothalamic dysfunction. Research ($105,000 2007-2012) Hormonal regulation of p38MAPK and its implications Insulin resistance and beta cell dysfunction in early Hamilton J, Barrera M, Guger S, Popov T, Schulte F. childhood: the role of maternal and infant metabolic National surveillance of non-type 1 diabetes mellitus Brain Tumour Research Assistance and Information Zinman B. Canadian Institutes of Health Research in Canadian children. Hamilton J, Dean H, Amed S, Network (b.r.a.i.n. Child) ($27,931 2006-2008) ($341,706 2005-2008) Sellers E, Dannenbaum D, Laubscher L, Booth G. risk factors. Hamilton J, Hanley A, Retnakaran R, Canadian Diabetes Association ($97,939 2006-2008) The genetics and neuroendocrinology of short stature. Hamilton J (Principal Investigator SickKids site). Insulin sensitization by muscle contraction pathways: implications for insulin resistance. Klip A. Canadian Neuroimaging studies of hippocampal formation in Lilly International Growth Study (GeNeSIS) Institutes of Health Research and National Science children with pre- and post-natal thyroid hormone ($58,542 2002-2008) Foundation of China - China-Canada Joint Health insufficiencies. Rovet J, Desrocher M, McAndrews MP, Research Initiative ($90,000 2006-2009) Sheard E. Canadian Institutes of Health Research ($728,640 2006-2011) 60 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT The GH Monitor Registry. Hamilton J (Principal Treatment options for Type 2 diabetes in adolescents Investigator SickKids site). Serono Canada and youth (TODAY). Palmert MR (Principal Investigator Incorporated ($6,200 2008-2010) until August 2007), Dahms W. National Institutes of The GLUT4 interactomes and their metabolic and Kidney Diseases ($3,240,000 2002-2010) Health/National Institute of Diabetes and Digestive implications. Klip A. Canadian Institutes of Health Research ($818,206 2007-2012) Type 1 diabetes TrialNet–the impact of GAD on preservation of cell function. Wherrett DK. National Thyroid hormone insufficiencies and visual perceptual Institutes of Health (US$450,000 2007-2008) deficits in preterm infants. Rovet, J, Westall C, Asztalos E, Perlman K, Heon E. March of Dimes Type 1 diabetes genetics consortium. Wherrett DK. (US$253,083 2004-2007) National Institutes of Health contract with Benaroya Research Institute (US$15,000 2003-2008) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 61 Dr. Peter Durie DIVISION HEAD Dr. Stanley Zlotkin FULL-TIME Dr. Peter Durie Dr. Nicola Jones Dr. Dr. Dr. Dr. Dr. Dr. Anne Griffi ths Simon Ling Vicky Ng Paul Pencharz Philip Sherman Mary Zachos PART-TIME Dr. Glenn Berall Dr. Carol Durno Dr. Paul Kortan Dr. Margaret Marcon Dr. Gary May CROSS-APPOINTEES Dr. Mary Corey CLINICAL FELLOWS Dr. Fatma Al-Madhaki Dr. Tanja Gonska Dr. Keith Ooi Dr. Juan Ossa RESEARCH FELLOWS Dr. Eric Benchimol Dr. Juan Gana Dr. Orlee Guttman Dr. Seamus Hussey Dr. Aleixo Muise Dr. Mary Sherlock Dr. Dan Turner Dr. Catharine Walsh Dr. Thomas Walters Dr. Eytan Wine Dr. Jason Yap Division of Gastroenterology, Hepatology and Nutrition OVERVIEW This year, staff and fellows in the division have Benchimol E: Trainee Scholarship, The Division of Gastroenterology, Hepatology and been honoured with awards, fellowships and named Gastroenterology Research Group and The American Nutrition is one of the only programs in Canada with a lectureships, including Canadian Association of Gastroenterological Association Institute, 2007. fully integrated specialty program in clinical paediatric Gastroenterology Visiting Professorship, Fellow of GI, hepatology, transplantation (liver and small bowel) the American Society of Nutrition, the Joseph M. West Gana J: Canadian Child Health Clinician Scientist and nutrition, and vertical programs in education and Family Memorial Award, the Levy/Thomson Award for Program Career Enhancement Program Award, 2008. research which span the clinical spectrum. The Outstanding Oral Presentation, the Miriam Neveren program offers one year of clinical training and two Memorial Award, the Canadian Child Health Clinician Gana J: Levy/Thomson Award for Outstanding Oral research years. The clinical component includes a wide Scientist Program (CCHCSP) Career Enhancement Presentation. Gana JC, Roberts EA, Ng VL, Ling SC. array of experiences including: procedures (endoscopy, Program Award, and many more. The staff members Non-invasive diagnosis of esophageal varices in manometry and calorimetry), inpatient management of of the division have published over 60 peer-reviewed children. Gastroenterology residents-in-training (GRIT) complex patients, including those awaiting and publications, nine books or book chapters and have as part of the Canadian Digestive Diseases Week undergoing transplantation, those with inflammatory received well over $10 million of grant funding for (CDDW), Montreal, 2008. bowel disease and more; ambulatory clinics (including projects spanning the next five years. home TPN, dysphasia, general GI, IBD, hepatology, Guttman O: CASL/Schering-Plough Canada transplantation and nutrition); and evidence-based HONOURS AND AWARDS educational rounds and training in ethics and Benchimol E: Canadian Child Health Clinician-Scientist communication. Our research spans the spectrum from Program Doctoral Award, 2008. Hussey S: The CAG/CIHR/CCFC Fellowship, 2008-2010. as inflammatory bowel disease, pancreatic diseases, Benchimol E: The Hospital for Sick Children Clinician- Hussey S: The Chisholm Memorial Fellowship, 2008. ulcer disease, liver diseases, GI microbiology and Scientist Training Program Research Fellowship, 2007. Hepatology Fellowship, 2007. basic and translational to patient-based in such areas immunology, nutritional metabolism and nutrient Hussey S: The Elizabeth Arbuthnot Dyson requirements. Many of our trainees in the research Benchimol E: Joseph M. West Family Memorial Fund, Fellowship, 2008. program (two years) combine a formal degree-granting Department of Post-Graduate Medical Education, program at the University of Toronto (M.Sc. or PhD) University of Toronto, 2007. Hussey S: The Miriam Neveren Memorial Award, 2008. our fellows have gone on to very successful careers in Benchimol E: American College of Gastroenterology Hussey S: The Edward Christie Stevens Fellowship, 2008. academic paediatric gastroenterology. Clinical Research Award, 2007. within their research fellowship years. The majority of SUBSPECIALTY RESIDENTS Dr. Kesete Bezu ADMINISTRATIVE STAFF Chona Callejo Roxanne Francis Margaret Johnson Sherry Joy Nnenna Ngoka Lilatool Shakur Suzanne Shek THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 63 Ling SC: Paediatric Transplant Bursary Award, Wine E: Canadian Institute of Health Research/ Barr R, Atkinson S, Pencharz P, Arguelles GR: Nutrition Canadian Transplant Society. Paediatric liver Canadian Association of Gastroenterology/Industry and cancer in children. Pediatric Blood and Cancer transplantation: 20-year experience at The Hospital (Astra Zeneca) Research Initiative Award, 2007. 2008: 50(S2): p 437. for Sick Children. Canadian Society of Transplantation PUBLICATIONS Barron MA, Pencharz PB: Nutritional issues in infants Adu-Afarwuah S, Lartey A, Brown KH, Zlotkin S, with cancer. Pediatric Blood and Cancer 2007: Pencharz P: Fellow of the American Society Briend A, Dewey KG: Randomized comparison of 49(S7): pp 1093-1096. for Nutrition, 2008. three types of micronutrient supplements for home Annual Meeting, Mont-Tremblant, Quebec, 2008. fortification of complementary foods in Ghana: Beck P, Shaffer E, Gall D, Sherman PM: The natural Sherman P: Canada Research Chair, Tier 1 effects on growth and motor development. American history and significance of ultrasonographically (renewal), 2008. Journal of Clinical Nutrition 2007: 86: pp 412-420. defined polypoid lesions of the gallbladder in children. Journal of Pediatric Surgery 2007: 42: pp 1907-1912. Sherman P: Canadian Association of Gastroenterology Adu-Afarwuah S, Lartey A, Brown KH, Zlotkin S, Briend A, Dewey KG: Home fortification of Bellissimo N, Desantadina V, Pencharz PB, Berall GB, complementary foods with micronutrient supplements Thomas SG, Anderson GH: A comparison of short-term Wine E: Canadian Child Health Clinician Scientist is well accepted and has positive effects on infant appetite and energy intakes in weight and obese boys Program (CCHCSP) Career Enhancement Program iron status in Ghana. American Journal of Clinical following glucose and protein drinks. International Award, 2007. Nutrition 2008: 87: pp 929-938. Journal of Obesity 2008: 32: pp 362-336. Visiting Professor Award, 2008. Wine E: The Hospital for Sick Children Clinician Ball RO, Urschel KL, Pencharz PB: Nutritional Bellissimo N, Pencharz PB, Berall GB, Goode RC, Scientist Training Program (CSTP), 2007. consequences of interspecies differences in arginine Thomas SG, Anderson GH: Effect of television viewing and lysine metabolism. Journal of Nutrition 2007: 137: at mealtime on food intake after a glucose preload pp 1626S-1641S. in boys. Pediatric Research 2007: 61: pp 745-749. Wine E: The University of Toronto Faculty of Medicine, Postgraduate Award (Javenthey Soobiah, Charles Fell Charitable Trust, Chisholm Memorial Fellowship Joseph M. West Family Memorial Fund), 2007. 64 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Bellissimo N, Pencharz PB, Berall GB, Goode RC, Castellani C, Cuppens H, Macek Jr M, Cassiman JJ, Draper H, Diamond IR, Temple M, John P, Ng VL, Thomas SG, Anderson GH: Reproducibility of short- Kerem E, Durie P, Tullis E, Assael BM, Bombieri C, Fecteau A: Multimodal management of endangering term food intake and subjective appetite scores after Brown A, Casals T, Claustress M, Cutting GR, hepatic hemangioma impact on transplant avoidance. a glucose preload, ventilation threshold and body Dequeker E, Dodge J, Doull I, Farrell P, Ferec C, Journal of Pediatric Surgery 2008: 43: pp 120-126. composition in boys. Applied Physiolology and Girodon E, Johannesson M, Kerem B, Knowles M, Nutrition Metabolism 2008: 33: pp 326-337. Munck A, Pignatti PF, Radojkovic D, Rizzotti P, Schwarz Elango R, Ball RO, Pencharz PB: Indicator amino M, Stuhrmann M, Tzetis M, Zielenski J, Elborn JS: acid oxidation: concept and application. Journal Benchimol E, Kirsch R, Viero S, Griffiths AM: Consensus on the use and interpretation of cystic of Nutrition 2008: 138: pp 243-246. Collagenous colitis and eosinophilic gastritis in a fibrosis mutation analysis in clinical practice. Journal four-year old girl: a case report and review of the of Cystic Fibrosis 2008: 7: pp 179-196. Elango R, Ball RO, Pencharz PB: Individual amino acid requirements in humans: an update. Current Opinion literature. Acta Paediatrica 2007: 96: pp 1365-1367. in Nutrition and Metabolic Care 2008: 11: pp 34-39. Dassopoulos T, Nguyen GC, Sitton A, Bromfiled GP, Benchimol EI, Seow CH, Steinhart AH, Griffiths AM: Schumm LP, Wu Y, Riguiero M, Siemanowski B, Traditional corticosteroids for induction of remission Griffiths AM, Brant S, Rious JD, Yang H, Duerr RH, Elango R, Humayun MA, Ball RO, Pencharz PB: Lysine in Crohn’s disease. Cochrane Database System Silverberg M, Cho JH, Steinhart H: Assessment of requirement of healthy school-age children determined Review 2008: 2: CD006792. reliability and validity of IBD phenotyping within the by indicator amino acid oxidation method. American NIDDK IBD genetics consortium (IBDGC). Journal of Clinical Nutrition 2007: 86: pp 360-365. Benchimol EI, Turner D, Mann EH, Thomas KE, Inflammatory Bowel Diseases 2007: 13: pp 975-983. Gao Y, Sherman PM, Sun Y, Li D: Multiplexed Gomes T, McLernon RA, Griffiths AM: Toxic megacolon in children with inflammatory bowel disease: clinical Dorfman R, Sandford A, Taylor C, Huang B, high-throughput electrokinetically-controlled and radiographic characteristics. American Journal of Frangolias D, Wang Y, Sang R, Pereira L, Sun L, immunoassay for the detection of specific bacterial Berthiaume Y, Tsui L-C, Paré PD, Durie P, Corey M, antibodies in human serum. Analytica Chimica Acta Zielenski J: Complex two-gene modulation of lung 2008: 606: pp 98-107. Gastroenterology 2008: May 26 [Epub ahead of print]. disease severity in children with cystic fibrosis. Journal of Clinical Investigation 2008: 118: pp 1040-1049. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 65 Gareau M, Jury J, MacQueen G, Sherman PM, Humayun MA, Elango R, Moehn S, Ball RO, Barrett JC, Hansoul S, Nicolae DL, Cho JH, Duerr RH, Perdue M: Probiotic treatment of rat pups normalizes Pencharz PB: Application of the indicator amino acid Rioux RD, Brant SR, Silverberg MS, Taylor KD, corticosterone release and ameliorates colonic oxidation technique for the determination of metabolic Barmada MM, Bitton A, Dassopoulos T, Datta LW, dysfunction induced by maternal separation. Gut availability of sulphur amino acids from casein versus Green T, Griffiths AM, Kistner EO, Murtha MT, 2007: 56: pp1522-1528. soy protein isolate in adult men. Journal of Nutrition Regueiro MD, Rotter JI, Schumm LP, Steinhart AH, 2007: 137: pp 1874-1879. Targan SR, Xavier R, the NIDDK IBD Genetics Consortium, Libioulle C, Sandor C, Lathrop M, Green GJ, Weitzman SS, Pencharz PB: Resting energy expenditure in children with stage IV neuroblastoma. Hussey S, Terebiznik MR, Jones NL: Autophagy: healthy Belaiche J, Dewit O, Gut I, Heath S, Laukens D, Pediatric Research 2008: 63: pp 332-336. eating and self digestion for gastroenterologists. Mni M, Rutgeerts P, Van Gossum A, Zelenika D, Heuschkel R, Salvestrini C, Beatties RM, Hildebrand H, Journal of Pediatric Gastroenterology, Hepatology, Franchimont D, Hugot JP, de Vos M, Vermeire S, Nutrition 2008: 46: pp 496-506. Louis E, the Wellcome Trust Case Control Consortium, Cardon LR, Anderson CA, Drummond H, Nimmo E, Walters T, Griffiths AM: Guidelines for the management of growth failure in childhood Ip H, Hyder Z, Haseen F, Rahman M, Zlotkin S: Ahmad T, Prescott NJ, Onnie CM, Fisher SA, Ghori J, inflammatory bowel disease. Inflammatory Bowel Improved adherence and anemia cure rates with Bumpstead S, Gwillam R, Tremelling M, Deloukas P, Diseases 2008: 14: 839-849. flexible administration of micronutrient Sprinkles: Mansfield J, Jewell D, Satsangi J, Mathew CG, a new public health approach to anaemia control. Parkes M, Georges M, Daly MJ. Genome-wide Humayun MA, Elango R, Ball RO, Pencharz PB: A European Journal of Clinical Nutrition 2007: association defines more than 30 distinct re-evaluation of protein requirement in young men using September 26 [Epub ahead of print]. susceptibility loci for Crohn’s disease. Nature the indicator amino acid oxidation technique. American Journal of Clinical Nutrition 2007: 86: pp 995-1002. 66 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Genetics 2008: June 29 [online publication]. Johnson-Henry K, Donato K, Gordanpour M, Lenoir-Wijnkoop I, Sanders M, Cabana M, Caglar E, Mager D, Marcon M, Wales P, Pencharz PB: Use Sherman PM: Lactobacillus rhamnosus strain GG Corthier G, Rayes N, Sherman PM, Timmerman H, of N-acetyl cysteine for the treatment of parenteral prevents enterohemorrhagic Escherichia coli-induced Vaneechoutte M, Van Loo J, Wolvers D: Probiotic nutrition (PN) induced liver disease in children on changes in epithelial barrier function. Infection and and prebiotic influence beyond the intestinal tract. home PN. Journal of Pediatric Gastroenterology Immunity 2008: 76: pp 1340-1348. Nutrition Reviews 2007: 65: pp 469-489. and Nutrition 2008: 46: pp 220-223. Moehn S, Bertolo RFP, Martinazzo-Dallagnol E, Kappelman M, Bousvaros A, Hyams J, Markowitz J, Lewis K, Caldwell J, Phan V, Prescott D, Nazli A, Griffiths AM and the Pediatric IBD Registry Group: Wang A, Soderholm J, Perdue M, Sherman PM, Pencharz PB, Ball RO: Metabolic availability of lysine Variation in the initial management of children with McKay D: Decreased epithelial barrier function evoked in feedstuffs determined using oral isotope delivery. Crohn’s disease. Inflammatory Bowel Diseases 2007: by exposure to metabolic stress and non-pathogenic E. Livestock Science 2007: 109: pp 24-26. 13: pp 890-895. coli is enhanced by TNFα. American Journal of Physiology 2008: 294: pp G669-G678. Muise AM, Walters TD, Wine E, Griffiths AM, Turner D, Griffiths AM, Bousvaros A, Hyams J: Body mass index Ling SC: Congenital cholestasis syndromes: what Silverberg MS, Rotin D: Protein tyrosine phosphatase in children with newly diagnosed inflammatory bowel happens when children grow up? Canadian Journal sigma is associated with ulcerative colitis. Current disease: observations from two multicentre North of Gastroenterology 2007: 21: pp 743-751. Biology 2007: 17: 1212-1218. Madore A-M, Prevost C, Dorfman R, Taylor C, Durie P, Nasr A, Avitzur Y, Ng VL, De Silva N, Wales PW: The Duerr RH, Regueiro MD, Ngan BY, Xu W, Sherman PM, Kugathasan S, Nebel J, Skelton J, Markowitz J, Keljo D, American inception cohorts. Journal of Pediatrics 2007: 151: pp 523-527. Zielenski J, Laprise C: Distribution of CFTR mutations use of conjugated hyperbilirubinemia greater than 100 Leach ST, Mitchell HM, Geczy CL, Sherman PM, Day AS: in Saguenay-Lac-Saint-Jean: proposal of a panel of lmol/L as an indicator of irreversible liver disease in S100 calgranulin proteins S100A8, S100A9 and mutations for population screening. Genetics in infants with short bowel syndrome. Journal of S100A12 are expressed in the inflamed gastric mucosa Medicine 2008: 10: pp 201-206. Pediatric Surgery 2007: 42: pp 359-362. of helicobacter pylori-infected children. Canadian Journal of Gastroenterology 2008: 22: pp 461-464. Ng VL, Anand R, Martz K, Fecteau A: Liver retransplantation in children: SPLIT database analysis of outcome and predictive factors for survival. American Journal of Transplantation 2008: 8: pp 386-395. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 67 Ng VL, Otley AR: Understanding quality of life for Pencharz PB: Assessment of protein nutritional status. children after liver transplantation: a work in progress. Pediatric Blood and Cancer 2008: 50(S2): pp 445-446. Rosh JR, Gross T, Mamula P, Griffiths AM, Hyams J: Hepatosplenic T-cell lymphoma in adolescents and young adults with Crohn’s disease: a cautionary tale. Liver Transplantation 2008: 14: pp 415-417. Puntai J, Markowitz J, Lerer T, Hyams J, Kugathasan S, O’Shea A-M, Wilson G, Ling SC. Minassian B, Cutz E: Griffiths AM, Otley A, Crandall W, the Pediatric IBD Lafora-like ground glass inclusions in hepatocytes of Collaborative Research Group: The effect of early Inflammatory Bowel Diseases 2007: 13: pp 1024-1030. Samonte V, Sherman PM, Taylor G, Carricato M, paediatric patients–report of two cases. Pediatric and immunomodulator use in moderate to severe pediatric Fecteau A, Ling S, Ng V: Scurvy diagnosed in a Developmental Pathology 2007: 10: pp 351-357. Crohn disease. Inflammatory Bowel Diseases 2008: pediatric liver transplant patient awaiting combined February 17 [Epub ahead of print]. kidney and liver retransplantation. Pediatric Transplantation 2008: 12: pp 363-367. Panaccione R, Fedorak R, Aumais G, Bernard E-J, Bernstein CN, Bitton A, Croitoru K, Dieleman LA, Rafii M, Elango R, Courtney-Martin G, House J, Fisher L, Enns R, Feagan BG, Franchimont D, Greenberg GR, Pencharz PB: High-throughput and simultaneous Griffiths AM, Marshall J, Pare P, Patel S, Penner R, measurement of homocysteine and cysteine in to measure resting energy expenditure in clinical Render C, Seidman E, Steinhart AH: Review and human plasma and urine by liquid chromatography- practice in children. Journal of Pediatrics 2008:152: clinical perspectives for the use of infliximab in electrospray tandem mass spectrometry. Analytical pp 437-439. ulcerative colitis. Canadian Journal of Biochemistry 2007: 371: pp 71-81. Shakur YA, Richards H, Pencharz PB: Is it necessary Sharieff W, Dofonsou J, Zlotkin S. Is cooking food in Gastroenterology 2008: 22: 261-272. Rafii M, McKenzie JM, Roberts SA, Steiner G, Ball RO, iron pots an appropriate solution for the control of Pencharz P: Energy balance and body composition. Pencharz PB: The in vivo regulation of phenylalanine anaemia in developing countries? A randomized clinical Pediatric Blood and Cancer 2008: 50: p 468. hydroxylation using enrichment in Apo-B 100. American trial in Benin. Public Health Nutrition 2007: 15: pp 1-7. Journal of Physiology 2008: 294: pp E475-E479. Pencharz PB, Hsu JW, Ball RO: Aromatic amino acid requirements in healthy human subjects. Journal of Nutrition 2007: 137: pp 1576S-1578S. 68 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Urschel KL, Rafii M, Pencharz PB, Ball RO: A multi- Shugart YY, Silverberg MS, Duerr RH, Taylor KD, Turner D, Grossman A, Rosh J, Gilman AR, Wang MH, Zarfas K, Schumm LP, Bromfielf G, Kugathasan S, Baladassano R, Griffiths AM: tracer stable isotope quantification of the effects of Steinhart AH, Griffiths AM, Kane SV, Barmada MM, Methotrexate following unsuccessful thiopurine arginine intake on whole-body arginine metabolism in Rotter JI, Mei L, Bernstein CN, Bayless TM, Langelier D, therapy in pediatric Crohn’s disease. American Journal neonatal piglets. American Journal of Physiology Cohen A, Bitton A, Rioux JD, Cho JH, Brant SR: of Gastroenterology 2007: 102: pp 2804-2812. 2007: 293: pp E811-E818. An SNP linkage scan identifies significant Crohn’s disease loci on chromosomes 13q13.3 and, in Jewish Turner D, Otley AR, Mack D, Hyams J, de Bruijne J, Wallon C, Yang PC, Keita A, Ericson AC, McKay DM, families, on 1p35.2 and 3q29. Genes and Immunity Uusoue K, Walters TD, Zachos M, Mamula P, Sherman PM, Perdue MH, Soderholm JD: Corticotropin- 2008: 9: pp 161-167. Beaton DE, Steinhart AH, Griffiths AM: Development, releasing hormone (CRH) regulates macromolecular validation, and evaluation of pediatric ulcerative permeability via mast cells in normal human colonic Toiviainen-Salo S, Raade M, Durie PR, Ip W, Marttinen E, colitis activity index: a prospective multicentre study. biopsies in vitro. Gut 2008: 57: pp 50-58. Savilahti E, Makitie O: MRI findings of the pancreas in Gastroenterology 2007: 133(2): pp 423-432. Ward LM, Gaboury I, Ladhani M, Zlotkin S: Vitamin patients with Shwachman-Diamond syndrome and mutations in the SBDS gene. Journal of Pediatrics Turner D, Steinhart AH, Griffiths AM: Omega-3 fatty D-deficiency rickets among children in Canada. Canadian 2008: 152: pp 434-436. acids (fish oil) for maintenance of remission in Medical Association Journal 2007: 177: pp 161-166. ulcerative colitis. Cochrane Database System Turner D, Griffiths AM: Esophageal, gastric and Wilschanski M, Durie PR: Patterns of GI disease in Review 2007: CD006443. adulthood associated with mutations in the CFTR duodenal manifestations of IBD, and the role of upper endoscopy in IBD diagnosis. Current Gastroenterology Turner D, Walsh CM, Benchimol EI, Mann EH, Reports 2007: 9: pp 475-478. Thomas KE, Chow C, McLernon RA, Walters TD, gene. Gut 2007: 56: pp 1153-1163. Steinhart AH, Griffiths AM: Severe paediatric ulcerative Zlotkin S: Editorial - ‘Control of Anemia: the time to act colitis: incidence, outcomes, and optimal timing for is now’. Indian Pediatrics 2007: 44: pp 84-86. second line therapy. Gut 2008: 57: pp 331-338. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 69 BOOKS AND BOOK CHAPTERS Wallace J, Sherman PM: Resolution of mucosal A multi-disciplinary human study on the genetic, Anemia Review Panel: anaemia Guidelines for Family inflammation. In: Progress in Inflammation Research environmental and microbial interactions that cause Medicine, 2nd Edition. MUMS Guideline Clearinghouse, Series: Resolution of Inflammation (Rossi A, IBD. Croitoru K, Griffiths AM, Ropelski M, Moyeddi P. Toronto, 2008. Sawatsky D, eds.) Berkhauser Verlag AG, Basel, 2007. Crohn’s and Colitis Foundation of Canada ($5,600,000 2007-2012) Durie PR: Schwachman-Diamond Syndrome. In: Walters TR, Griffiths AM: Growth impairment in IBD. Pediatric Gastrointestinal Disease, 5th Edition, In: Pediatric Inflammatory Bowel Disease (Baldassano A novel candidate susceptibility gene at the IBD6 Volume 2 (Kleinman RE, Sanderson IR, Goulet O, R, Mamula P, Markowitz J, eds.). Springer, USA, 2008. locus. Griffiths AM, Rotin D. Crohn’s and Colitis Foundation of Canada New Innovations Grant Sherman PM, Mieli-Vergani G, Shneider BL, eds.). BC Decker Inc., Hamilton, 2008. Wine E, Terebiznik M, Jones NL: Microbial interactions ($50,000 2007) with gut epithelium. In: Paediatric Gastrointestinal Disease, 5th Edition, Volume 1 (Kleinman R, Goulet O, A prospective study of linear growth in paediatric in inflammatory bowel disease. In: Pediatric Mieli-Vergnani G, Sherman P, Schneider B, eds.). IBD. Denson LA, Griffiths AM. Crohn’s and Colitis Nutrition in Practice (Berthold K, ed.) Karger, BC Decker Inc., Hamilton, 2008. Griffiths AM, Carricato M: Enteral nutrition Basel, Switzerland, 2008. Foundation of America ($150,000 per year 20072010) FUNDING Griffiths AM, Hugot J-P: Crohn’s disease. In: Paediatric A multicentre study of the safety and efficacy of Adalimumab in pediatric Crohn’s disease: a multicentre Gastrointestinal Disease, 5th Edition, Volume 1 N-acetylcysteine in the treatment of acute liver failure study. Griffiths AM. Abbott Laboratories (per patient (Kleinman R, Goulet O, Mieli-Vergnani G, Sherman P, in paediatric patients not caused by acetaminophen. remuneration; anticipated $90,000 2007-2009) Schneider B, eds.). BC Decker Inc., Hamilton, 2008. Ng VL, Fecteau A. National Institutes of Health/ National Institute of Diabetes and Digestive and Kidney Diseases (US$69,000 2005-2010) 70 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Arginine and CF. Grasemann H, Ratjen F, Development and validation of a disease-specific Epidemiology of eosinophilic esophagitis in children Pencharz P. Canadian Cystic Fibrosis Foundation health-related quality of life questionnaire for children and adolescents in Canada. Marcon MA, Dourin E. ($140,000 2007-2010) after liver transplantation. Otley AR, Ng VL, Nicholas Directing study which involves all 16 paediatric Assessment of activity and prediction of outcome in DB, Thompson J, Gilmour S, Watters C. Canadian centres in Canada. J. Campbell Fund ($62,966 Institutes of Health Research ($280,576 2005-2009) 2006-2008) paediatric ulcerative colitis. Turner D, Steinhart H, Griffiths AM. Canadian Association of Development and validation of an objective skill Evaluation of a novel parenteral lipid source in the Gastroenterology/Canadian Institutes of Health assessment for colonoscopy. Ling SC, Walsh C. prevention of PN associated cholestasis. Wales P, Research ($65,000 per year 2006-2008) The Hospital for Sick Children Paediatric Pencharz P. Fresenius-Kabi ($159,850 2008) Consultants Partnership ($4,968 2007) Evaluation of CFTR function ex-vivo in sweat glands Cell signals: cell signalling in mucosal inflammation of perfused skin as a translational tool for future drug and pain. Ellen R, Jones NL. Canadian Institutes Development of a new sweat test using sweat of Health Research ($300,000 2002-2008) potential measurements. Durie PR. Cystic Fibrosis trials in CF. Durie PR, Pang C, Bear CE, Gonska T. Foundation Therapeutics, Inc. ($70,818 US 2008) (SickKids) Research Institute, New Ideas Grant ($50,000 2008-2009) Clinical Research Facilitation Award. Durie PR. Cystic Fibrosis Foundation Therapeutics, Inc. Development of a piglet short gut model. Turner J, (US$108,280 2007-2008) Ball R, Pencharz P, Wales P. Stollery Children’s Food intake control in children. Anderson GH, Hospital Foundation ($29,223 2007) Berall GB, Goode RC, Pencharz PB. Canadian Institutes of Health Research ($104,219 2007-2010) Clinician scientist training program in clinical nutrition research. Pencharz P. Canadian Institutes of Health Diagnosing cystic fibrosis in neonates and adults: Research ($265,000 2002-2008) where do we draw the line? Durie PR, Tullis E, Ratjen F. Canadian Cystic Fibrosis Foundation transplantation. Ng VL, Fecteau A. National Institutes Determining the threshold of CFTR function that ($120,000 per year 2008-2011) of Health/National Institute of Diabetes and causes CF disease. Durie PR. Canadian Cystic Functional outcomes after paediatric liver Digestive and Kidney Diseases (US$6,000 2007-2008) Fibrosis Foundation ($110,000 per year 2005-2008) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 71 Genetic predictors of time to recurrence following ileal Innovative strategies for optimizing management North American IBD Genetics Consortium. Cho J, resection in early onset Crohn’s disease. McLeod R, of infants with biliary atresia. Guttman O Duerr R, Silverberg M, Griffiths AM, Rotter J, Griffiths AM. Crohn’s and Colitis Foundation (co-supervisors: Ng VL, Roberts EA.) Canadian Brandt S, Rioux J. National Institutes of Health of Canada ($150,000 per year 2007-2010) Association for the Study of Liver Disease/ ($3,000,000 2007-2011) Canadian Institutes of Health Research Host responses to Helicobacter pylori infection: Fellowship Award ($55,000 2007-2008) Opportunity fund: funding for infrastructure associated with a Canada Research Chair. Sherman PM. Canada role of STAT3 during H. pylori infection. Jones NL. Canadian Institutes of Health Research International Collaboration Project. Zlotkin S. Foundation for Innovation, Leaders ($427,348 total ($100,000 2007-2008) Canadian Institutes of Health Research [$170,939 direct from CFI] 2008) ($25,000 2007-2008) Outcomes in severe paediatric ulcerative colitis: Immune regulation and biomarker development in paediatric and adult onset human autoimmune Investigation of Helicobater pylori survival in host cells. a multicentre study. Griffiths AM. Schering Canada diseases. Bar-Or A, Banwell B, Piccirillo CA, Jones NL. Early Researcher Award, Ontario Ministry ($350,000 2007-2009) Polychronakos C, Sherman PM. Canadian Institutes of of Research and Innovation ($150,000 2006-2011) Pico-salax versus poly-ethylene glycol for bowel Health Research Team Grant ($2,018,445 2007-2112) Knowledge translation award. Zlotkin S. Canadian Induction of autophagy by H. pylori. Jones NL. Institutes of Health Research ($100,000 2006-2007) cleanout before colonoscopy in children: a single blinded randomized controlled trial. Ling SC, Griffiths AM, Turner D. Ferring Inc. ($24,000 2006-2008) Canadian Association of Gastroenterology/ Canadian Institutes of Health Research/Abbott Molecular genetics, SBDS function and deficiency in ($45,000 2007-2009) SDS. Rommens JM, Durie PR. National Institutes of Protocol AI463-08: evaluation of the Health ($1,250,000 2004-2009) pharmacokinetics, safety, tolerability and efficacy of entecavir (ETV) in paediatric subjects with chronic Infliximab in paediatric ulcerative colitis: a multicentre study. Griffiths AM. Centocor (per patient Neonatal protein and energy metabolism. Pencharz P. hepatitis B virus (HBV) infection who are HBeAg- remuneration; anticipated $100,000 2007-2009) Medical Research Council/Canadian Institutes of positive. Ling SC. Bristol-Myers Squibb Health Research ($136,817 2006-2011) ($25,000 per patient, 2007-2013) 72 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Role of autophagy in Helicobacter pylori infection Studies of paediatric liver transplantation (SPLIT). The contribution of genetic modulators of disease and disease. Jones NL. Canadian Institutes of Health Ng V, Fecteau A. National Institutes of Health/ severity in cystic fibrosis to other diseases with Research ($103,959 per year 2008-2013) National Institute of Diabetes and Digestive and similarities of clinical phenotype. Durie PR, Zielenski J. Kidney Diseases (US$75,000 2005-2010) Genome Canada ($5,787,662 2005-2009) inflammatory bowel disease. Sherman PM. Crohn’s Studies of primary sclerosing cholangitis (STOPSC). The use of indicator amino acid oxidation to determine and Colitis Foundation of Canada Fay Shapiro Cutler Ling SC, Durie P, Griffiths AM. Musette and Allen amino acid requirement. Pencharz P, Ball R. Medical Grant-In-Aid of Research ($375,000 2007-2010) Morgan Jr. Foundation (annual and patient-based Research Council/Canadian Institutes of Health stipends, $10,000 per year plus $200 per patient Research ($152,967 2005-2010) Role of probiotics in the management of experimental Salary award: Tier 1 (renewal). Sherman PM. 2006-2009) Training grant: cell signalling in mucosal inflammation Canada Research Chair ($1,400,000 2008-2015) Targeted small molecule therapies for cystic and pain. Ellen R, Sherman PM. Canadian Institutes SBDS protein expression in peripheral blood fibrosis. Bear CE, Deber CM, Durie PR, Lingwood C, for Health Research ($300,000 per year 2002-2008) leukocytes. Durie PR, Rommens JM, Shimamura A. Rotin D, Ratjen F, Yeger H. Canadian Cystic Shwachman Diamond Syndrome Foundation Fibrosis Foundation - BREATHE Program (US$30,000 per year 2006-2008) ($480,000 per year 2004-2009) Upper limits of leucine requirements. Pencharz P. International Council for Amino Acid Science ($155,850 2008) Short bowel syndrome, neonatal piglet model. Turner J, Team in childhood obesity research. McCrindle B, Ball R, Pencharz P, Wales P. SickKids Foundation Anderson GH, Hamilton J, Parkin P, Pencharz P. ($62,690 2008-2009) Canadian Institutes of Health Research placebo controlled study of VX770 in patients with ($506,842 2008; $464,474 2008-2011) cystic fibrosis having G551D mutations to evaluate VX06-770-101: a Phase 2a, randomized, double-blind, Sprinkles project support. Zlotkin S. HJ Heinz safety, phamacokinetics and biomarkers of CFTR Company Foundation ($600,000 2006-2007) activity. Durie PR (Toronto site). Vertex Pharmaceuticals, Inc. (US$30,000 per year 2007-2008) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 73 Dr. Angela Punnett DIVISION HEAD Dr. Victor Blanchette FULL-TIME Dr. Oussama Abla Dr. Sarah Alexander Dr. Ute Bartels Dr. Sylvain Baruchel Dr. Eric Bouffet Dr. Leonardo Brandão Dr. Manuel Carcao Dr. Helen Chan Dr. John Doyle Dr. Yigal Dror Dr. Adam Gassas Dr. Ron Grant Dr. Abha Gupta Dr. Hans Hitzler Dr. Annie Huang Dr. Meredith Irwin Dr. Walter Kahr Dr. Melanie Kirby Dr. David Malkin Dr. Ahmed Naqvi Dr. Dr. Dr. Dr. Dr. Dr. Dr. Paul Nathan Isaac Odame Angela Punnett Lillian Sung Uri Tabori Sheila Weitzman Suzan Williams CROSS-APPOINTEES/ PART-TIME Dr. Anthony Chan Dr. Stephen Comay Dr. Lee Ann Gallant Dr. Mark Greenberg Dr. David Hodgson Dr. Nicole Inch Dr. Dr. Dr. Dr. Dr. Normand Laperriere Barbara-Ann Millar Aubrey Maze Ben Saxon Jill Solomon Division of Haematology/Oncology OVERVIEW program (Program Co-Heads, Drs. David Malkin and cancer genetics, blood and marrow transplantation The Division of Haematology/Oncology provides a Rosanna Weksberg). The haematology section has and paediatric thrombosis and haemostasis. diagnostic service and specialist care for children large programs in paediatric thrombosis and <18 years of age with cancer (paediatric oncology), haemostasis, the paediatric haemoglobinopathies and Research in the division spans clinical to basic non-malignant blood disorders (paediatric haematology) the paediatric marrow failure syndromes. The division science with a special focus on translational research. and hematopoietic stem cell (blood and marrow) is an active member of the Children’s Oncology Group Currently, 13 of the division’s 27 full-time faculty have transplantation. The division is the largest of its kind (COG), the largest paediatric clinical trials group in appointments as clinician-scientists or clinician- in Canada and among the largest of such programs North America (SickKids Principal Investigator, Dr. Ron investigators in the Department of Paediatrics with worldwide. Approximately 300 new cancer cases are Grant), and is committed to the improvement in parallel appointments in the Research Institute. registered in the oncology program each year, and The outcomes of children with cancer through participation Research in the division is multidisciplinary, involving Hospital for Sick Children (SickKids) is the designated in ethics approved, prospective clinical trials. The health care professionals with expertise in a variety of site for haematopoietic stem cell transplantation for division is a member of the paediatric oncology phase I areas (e.g., nursing, pharmacy, psychology, social patients < 18 years of age in the Province of Ontario. Consortium of COG (Principal Investigator, Dr. Sylvain work). The division is committed to facilitating Baruchel), and is an active participant in activities of knowledge exchange through international Reflecting the size and diversity of its clinical services, the Société Internationale d’Oncologie Pédiatrique partnerships with a particular focus on countries the division is functionally organized into six sections (SIOP), the Histiocyte Society and the Berlin Frankfurt lacking the health care resources available in Canada. and three programs: the leukemia/lymphoma section Munster (BFM) paediatric leukemia clinical trials group. A major development over the past year was the (Section Head, Dr. Sheila Weitzman); the approval of the Comprehensive Cancer Centre at neurooncology (brain tumor) section (Section Head, Dr. The division is the largest Royal College of Physicians Eric Bouffet); the solid tumor section (Section Head, and Surgeons of Canada accredited training program SickKids. The Centre will serve as the “hub” linking Dr. Ron Grant); the blood and marrow transplantation for the specialty paediatric haematology/oncology in clinicians and researchers working in the field of section (Section Head, Dr. John Doyle); the Canada (Training Program Director, Dr. Angela cancer at SickKids with the goal of establishing haematology section (Section Head, Dr. Victor Punnett). The education program in the division also SickKids as an international leader in the field of Blanchette); the inpatient services section (Section includes a very large international training program in pediatric cancer research, education and clinical care. The Executive of the Comprehensive Cancer Centre Head, Dr. Sarah Alexander); the long-term follow up paediatric haematology/oncology and offers program (Program Head, Dr. Mark Greenberg); the new subspecialty training opportunities in areas such as includes Dr. David Malkin, Dr. David Kaplan, Dr. James agents and experimental therapies program (Program paediatric leukemia/ lymphoma, neurooncology, Rutka, Judy Van Clieaf and Dr. Victor Blanchette. Dr. A. Lee Chong Dr. C. Macartney Dr. A. Muhammad Dr. J. Robertson Dr. M. Rojas Dr. K. Scheinemann Dr. C. Segbefia Dr. P. Sivaprakasam ADMINISTRATIVE STAFF Bibi Ali Ingrid Argiropoulos Barbara Black Sandra Carbone-Walker Connie Grillo Lucy Holford Head, Dr. Sylvain Baruchel); and the cancer genetics CLINICAL AND RESEARCH FELLOWS Dr. I. Abosoudah Dr. F. Al-Abbas Dr. S. Al-Afghani Dr. T. Al-Asaad Dr. R. Al-Mahmoud Dr. O. Al-Sharif Dr. Q. Alharbi Dr. P. Angelini Dr. L. Avila Dr. J. Baker Dr. V. Breakey Dr. M. Cada Dr. P. Gibson Dr. C. Lam Dr. Dr. Dr. Dr. Dr. Dr. O. Teuffel T. Truong M. Van Den Akker B. Williams P. Wong P. Yenson Shanaz Karim Philippa McCaffrey Christal Malcolm Sylvia Muir Annabel Sousa Laurel Willmott Arlene Zaldivar The administrative team for the division includes Dr. Sung L: University of Toronto Junior Victor Blanchette (Division Head), Dr. Sheila Weitzman Researcher Award, 2008. Alexander SW, Hopewell S, Hunter S, Chouksey A: Rituximab and desensitization for a patient with severe factor IX deficiency, inhibitors and history of (Associate Director, Clinical), Judy Van Clieaf (Director, Child Health Services) and Lucy Holford (Team Leader, PUBLICATIONS anaphylaxis. Journal of Paediatric Haematology Administrative and Support Services). Abdelhaleem M, Shago M, Beimnet K, Sayeh E, Oncology 2008: 30: pp 93-95. Bartakke S, Weitzman S: Childhood acute myeloid HONOURS AND AWARDS leukaemia with hemophagocytosis by the blasts and Alkalbani N, Weitzman S, Abdelhaleem M, Carcao M, Blanchette V: Dr. Richard Rowe Award for Clinical inv(8)(p11q13) with MOZ-TIF2 fusion transcripts. Abla O: Acute lymphoblastic leukaemia presenting Excellence, Department of Paediatrics, 2008. Journal of Pediatric Haematology and Oncology 2007: with gross hematuria. Paediatrics and Child Health 29: pp 643-645. 2007: 12: pp 573-574. Abdelhaleem M, Shago M, Sayeh E, Abla O: Almanjomi F, Armstrong D, Abla O, Naqvi A, Weitzman S, Greenberg M: O. Harold Warwick Prize, 2007. Hitzler H: Alvin Zipursky Teaching Award, Division of Childhood myeloid/natural killer precursor acute Hitzler J: Lumbar spinal fluid collections in children Haematology/Oncology, The Hospital for Sick Children. leukaemia with der(5) t(4;5)(q31;q31.3) and t(14;17) treated with intrathecal chemotherapy: elevated CSF 2008. (q32;q23). Cancer Genetics and Cytogenetics 2007: protein as a diagnostic clue. Pediatric Blood and 178: pp 141-143. Cancer 2008: 5: pp 295-298. Abdullah S, Diezi M, Sung L, Dupuis L, Geary D, Attias D, Weitzman S: The efficacy of rituximab in Irwin M: MDM2 International Workshop Young Investigator Award, 2007. Abla O: Sevelamer hydrochloride: a novel treatment high-grade pediatric B-cell lymphoma/leukaemia: Irwin M: American Society of Pediatric Haematology of hyper-phosphatemia associated with tumor lysis a review of available evidence. Current Opinion in Oncology Young Investigator Award, 2007. syndrome in children. Pediatric Blood and Cancer Pediatrics 2008: 20: pp 17-22. 2008: 51: pp 59-61. Ayi K, Min-Oo G, Serghides L, Crockett M, Kirby-Allen M, Irwin M: Elsie Winifred Crann Memorial Trust Quirt I, Gros P, and Kain KC: Pyruvate kinase Award for Research in Life Sciences, University Abla O, Dror Y, Shago M: Translocation (X:10) in a of Toronto, 2007. child with therapy-related acute myeloid leukaemia deficiency protects and malaria. New England following chemotherapy for Ewing’s sarcoma. Cancer Journal of Medicine 2008 358: pp 1805-1810. Malkin D: Man of Distinction Honoree, Genetics and Cytogenetics 2007: 178: pp 168-169. Israel Cancer Research Fund, 2008. 76 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Baker JM, Viero S, Kim PC, Grant RM: Stage III cystic Bouffet E, Tabori U, Bartels U: Paediatric Diaz RJ, Laughlin S, Nicolin G, Buncic JR, Bouffet E, partially differentiated nephroblastoma recurring after ependymomas: should we avoid radiotherapy? Bartels U: Assessment of chemotherapeutic response nephrectomy and chemotherapy. Pediatric Blood and Lancet Oncology 2007: 8: pp 665-666. in children with proptosis due to optic nerve glioma. Child’s Nervous System 2008: 24: pp 707-712. Cancer 2008: 50: pp 129-31. Bulley S, Gassas A, Dupuis LI, Aplenc R, Beyene J, Barrette S, Bernstein ML, Robison LL, Samson Y, Greenberg ML, Doyle JJ, Sung L: Inferior outcomes for Dupuis L, Sibbald C, Schechter T, Ansari M, Gassas A, Theoret Y, Kassir N, Champagne MA, Doyle J: Brossard J, Weitzman S, Woods WG: Incidence of overweight children undergoing allogeneic stem cell neuroblastoma after a screening program. Journal transplantation. British Journal of Haematology 2007: IV busulfan dose individualization in children of Clinical Oncology 2007: 25: pp 4929-4932. 140: pp 214-217. undergoing hematopoietic stem cell transplant: Bartakke S, Abdelhaleem M, Abla O: Childhood Calado RT, Graf SA, Wilkerson KL, Kajigaya S, acute lymphoblastic leukaemia simulating multifocal Ancliff PJ, Dror Y, Chanock SJ, Lansdorp PM, osteomyelitis. British Journal of Haematology 2007: Young NS: Mutations in the SBDS gene in acquired Epstein I, Stevens S, McKeever P, Baruchel S, Jones H: 139: p 2. aplastic anemia. Blood 2007: 110: pp 1141-1146. Using puppetry to elicit children’s talk for research. limited sampling strategies. Biology of Blood and Marrow Transplantation 2008: 14: pp 576-582. Nursing Inquiry 2008: 15: pp 49-56. Bartakke S, Abdelhaleem M, Carcao M: Valproic acid Castelliono RC, DeBortoli M, Skapura DG, Lin LL, induced pure red cell aplasia and megakaryocytic Perlaky L, Sheldon MH, Irwin MS, Kim JYH: Feldman BM, Aledort L, Bullinger M, Delaney FM, dysplasia. British Journal of Haematology 2008: Medulloblastomas overexpress TAp73 and DNp73. Doria AS, Funk S, Giangrande P, Lundin B, Manco- 141: p 133. BMC Cancer 2007: 7: p 127. Johnson M, Miners A, Scriba PC, Srivastava A, Bernhard N, Morris SK, Taylor G, Oudjhane K, Chan AK, Black L, Ing C, Brandao LR, Williams S: haemophilia prophylaxis: governmental and insurer Baruchel S: Massive intrathoracic tumor in a relatively Utility of a PTT in monitoring unfractionated heparin perspectives. Proceedings of the Second International asymptomatic boy. Pediatric Annals 2007: pp 634-635. in children. Thrombosis Research 2007: October 31 Prophylaxis Study Group (IPSG) Symposium, October [Epub ahead of print]. 2005, Munich, Germany. Haemophilia 2007: 13(6): Schramm W, Blanchette VS: The economics of pp 745-749. Blanchette V, Bolton-Maggs P: Childhood immune thrombocytopenic purpura: diagnosis and Chera R, Gupta AA, Bailey D, Somers RG, Kukreti V, management. Pediatric Clinics in North Crump M: Small intestinal B-cell lymphoma in a America 2008: 55: pp 393-420. patient with lymphangiectasia secondary to abdominal Blanchette V: Musculoskeletal measurement tools lymphangioma. Journal of Clinical Oncology 2008: 26: from the International Prophylaxis Study Group (IPSG). pp 675-678. Haemophilia 2008: 14: pp 162-169. Feldman BM, Funk S, Lundin B, Doria AS, Ljung R, THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 77 Glade Bender JL, Adamson PC, Reid JM, Xu L, Finkelstein Y, Schechter T, Garcia-Bournissen F, Kirby Gassas A, Ishaqi MK, Afzal S, Finkelstein-Schechter T, M, Nurmohamed L, Juurlink D, Blanchette V, Koren G: Dupuis A, Doyle J: A comparison of the outcomes of Baruchel S, Shaked Y, Kerbel RS, Cooney E, Stempak D, Is morphine exposure associated with acute chest children with acute myelogenous leukaemia in either Chen HX, Krailo MD, Ingle AM, Blaney SM, Kandel JJ, syndrome in children with vaso-occlusive crisis of first or second complete remission (CRI vs CR2) Yamashiro DJ: Phase I trial and pharmacokinetic study sickle cell disease? A six-year case-crossover study. following allogeneic hematopoietic stem cell of bevacizumab in pediatric patients with refractory Clinical Therapeutics 2007: 29: pp 2738-2743. transplantation at a single transplant center. Bone solid tumours: a children’s oncology group study. Marrow Transplantation 2008: 41: pp 941-945. Journal of Clinical Oncology 2008: 26: pp 399-405. Prophylaxis for severe haemophilia: clinical challenges Gassas A, Sung L, Saunders EF, Doyle J: Graft- Green GJ, Weitzman SS, Pencharz PB: Resting energy in the absence as well as in the presence of inhibitors. versus-leukaemia effect in hematopoietic stem cell expenditure in children newly diagnosed with stage Haemophilia 2008: 14: pp 196-201. transplantation for pediatric acute lymphoblastic IV neuroblastoma. Pediatric Research 2008: 63: leukaemia: significantly lower relapse rate in unrelated pp 332-336. Fischer K, Valentino L, Ljung R, Blanchette V: Gallie BL, Zhao J, Vandezande K, White A, Chan HSL: transplantations. Bone Marrow Transplantation 2007: Global issues and opportunities for optimized 40: pp 951-955. 2007: 49: pp 1083-1090. Greenberg J, Somme S, Russnes HE, Durbin AD, Malkin D: The estrogen receptor pathway in retinoblastoma care. Pediatric Blood and Cancer Gerrard M, Cairo MS, Weston C, Auperin A, Pinkerton R, rhabdomyosarcoma: a role for estrogen receptor-ß Lambilliote A, Sposto R, McCarthy K, Lacombe MJ, in proliferation and response to the antiestrogen Gassas A, Ishaqi MK, Afzal S, Dupuis A, Doyle JJ: Perkins SL, Patte C: FAB LMB96 International Study 4’OH-tamoxifen. Cancer Research 2008: 68: Outcome of haematopoietic stem cell transplantation Committee: excellent survival following two courses of pp 3476-3485. for paediatric acute lymphoblastic leukaemia in third COPAD chemotherapy in children and adolescents with complete remission: a vital role for graft-versus-host- resected localized B-cell non-Hodgkin’s lymphoma: Gupta A, Tyrrell P, Valani R, Benseler S, Weitzman S, disease/graft-versus-leukaemia effect in survival. results of the FAB/LMB 96 international study. British Abdelhaleem M: The role of the initial bone marrow British Journal of Haematology 2008: 140: pp 86-89. Journal Haematology 2008: 141: pp 840-847. aspirate in the diagnosis of hemophagocytic lymphohistiocytosis. Pediatric Blood and Cancer 2008: 51: pp 402-404. 78 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Gupta A, Weitzman S, Abdelhaleem M: The role of Husain M, Naqvi A, Ngan B, Aiuti A, Atkinson A, Komvilaisak P, Grant R, Weitzman S, Brandao L, hemophagocytosis in bone marrow aspirates in the Grunebaum E, Roifman C: Burkitt’s lymphoma in a Blaser S, Chait P, Connolly B, Williams S: Epidural diagnosis of hemophagocytic lymphohistiocytosis. patient with adenosine deaminase deficiency-severe hematomas following tissue plasminogen activator Pediatric Blood and Cancer 2008: 50: pp 192-194. combined immunodeficiency treated with polyethylene (tPA) therapy for pulmonary embolism in a pediatric glycol-adenosine deaminase. Journal of Pediatrics patient with stage IV Burkitt’s lymphoma: a case 2007: 151: pp 93-95. report. Thrombosis Research 2008: 121: pp 709-712. Irwin MS, Thiele CJ, Kaplan DR: Neuroblastoma cells Jackson EM, Shaikh TH, Gururangan S, Jones MC, Koo A, Sung L, Allen U, Naqvi A, Drynan-Arsenault J, isolated from bone marrow metastases contain an Malkin D, Nikkel SM, Zuppan CW, Wainwright LM, Dekker A, Maloney AM, Dupuis L: Efficacy and safety enriched tumor-initiating cell. Cancer Research 2007: Zhang F, Biegel JA: High-density single nucleotide of caspofungin for the empiric management of fever in 67: pp 11234-11243. polymorphism array analysis in patients with germline neutropenic children. Pediatric Infectious Disease deletions of 22q11.2 and malignant rhabdoid tumor. Journal 2007: 26: pp 854-856. Hansford L, McKee A, Zhang L, George R, Gerstle JT, Thorner P, Smith KM, Look AT, Yeger H, Miller FD, Hargrave D, Chuang N, Bouffet E: Conventional MRI Human Genetics 2007: 122: pp 117-127. Kretschmar C, Kleinberg L, Greenberg M, Burger P, cannot predict survival in childhood diffuse intrinsic Holmes E, Wharam M: Pre-radiation chemotherapy pontine glioma. Journal of Neurooncology 2008: 86: Johnston D, Keene D, Lafay-Cousin L, Steinbok P, pp 313-319. Carret A, Crooks B, Strother D, Wilson B, Odame I, with response-based radiation therapy in children with Eisenstat D, Mpofu C, Zelcer S, Huang A, Bouffet E: central nervous system germ cell tumors: a report Supratentorial primitive neuroectodermal tumors: A from the Children’s Oncology Group. Pediatric Blood Carcao M: A comprehensive review of rFVIIa usage in Canadian Paediatric Brain Tumor Consortium report. and Cancer 2007: 48: pp 285-291. a tertiary care pediatric centre. Pediatric Blood and Journal of Neuro-oncology 2008: 86: pp 101-108. Heller M, Lau W, Pazmino-Canizares J, Brandao LR, Kuhne, T, Blanchette V, Buchanan GR, Ramenghi U, Cancer 2008: 50: pp 1013-1017. Klaassen RJ, Blanchette VS, Barnard D, Wakefield CD, Donato H, Tamminga RY, Rischewski J, Berchtold W, Hitzler, JK: Acute megakaryoblastic leukaemia in Curtis C, Bradley CS, Neufeld EJ, Buchanan GR, Silva Imbach P, on behalf of the Intercontinental Childhood Down’s Syndrome. Pediatric Blood and Cancer 2007: MP, Chan AK, Young NL: Validity, reliability and ITP Study Group: Splenectomy in children with 49: pp 1066-1069. responsiveness of a new measure of health-related idiopathic thrombocytopenic purpura: a prospective quality of life in children with immune study of 134 children from the Intercontinental thrombocytopenia purpura: The Kids’ Tools (KIT). Childhood ITP Study Group. Pediatric Blood and Journal of Pediatrics 2007: 150: pp 510-515. Cancer 2007: 49: pp 829-834. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 79 Lafay-Cousin L, Bartels U, Raybaud C, Kulkarni A, Lau LM, Stuurman K, Weitzman S: Skeletal McDonnell C, Barlow R, Campisi P, Grant RM, Malkin D: Guger S, Huang A, Bouffet E: Neuroradiological Langerhans cell histiocytosis in children: permanent Fatal peri-operative acute tumour lysis syndrome findings of bleomycin extravasation in cystic consequences and health-related quality of life in precipitated by dexamethasone. Anaesthesia 2008: craniopharyngioma. Journal of Neurosurgery 2007: long-term survivors. Pediatric Blood and Cancer 2008: 63: pp 652-655. pp 107: pp 318-323. 50: pp 607-612. Metzger ML, Stewart CF, Freeman BB, Billups CA, Hoffer FA, Wu J, Coppes MJ, Grant RM, Chintagumpala Lafay-Cousin L, Sung L, Carret AS, Hukin J, Strother D, Liang ML, Ma J, Ho M, Solomon L, Bouffet E, Rutka JT, Wilson B, Zelcer S, Odama I, Silva M, Mpofu C, Hawkins C: Tyrosine kinase expression in pediatric M, Mullen EA, Alvarado C, Daw NC, Dome JS: Johnson D, Bouffet E, on behalf of the Canadian high grade astrocytoma. Journal of Neurooncology Topotecan is active against Wilms’ tumor: a result Paediatric Brain Tumour Consortium: Carboplatin 2008: 87: pp 247-253. of a multi-institutional phase II study. Journal of Clinical Oncology 2007: 25: pp 3130-3136. hypersensitivity reaction in paediatric low grade glioma patients: a national experience. Cancer 2008: 112: pp 892-899. Larouche V, Huang A, Bartels U, Bouffet E: A difficult Mabbott D, Barnes M, Laperriere N, Landry SH, Bouffet E: Neuro-cognitive function in same-sex twins Morel CF, Gassas A, Doyle JJ, Clarke JT: following focal radiation for medulloblastoma. Journal Unsuccessful treatment attempt: cord blood stem of Neurooncology 2007: 9: pp 460-464. cell transplantation in a patient with Niemann-Pick disease type A. Journal of Inherited Metabolic start: tumors of the central nervous system in the first year of life. Pediatric Blood and Cancer 2007: Mabbott D, Penkman L, Witol A, Strother D, Bouffet E: 49: pp 1074-1082. Relations among core neuro-cognitive functions in children with posterior fossa tumours. Lau KK, Stoffman JM, Williams S, McCusker P, Neuropsychology 2008: 22: pp 159-168. Disease 2007: 30: p 987. Nathan PC, Ness KK, Greenberg ML, Whitton J, Hudson M, Mertens A, Laverdiere C, Wolden S, Davidoff A, Robison LL, Zeltzer L, Gurney J: Quality Brandao L, Patel S, Chan AK, Canadian paediatric of life in survivors of childhood Wilms’ tumor or Thrombosis and Hemostasis Network: neonatal renal Manjomi F, Armstrong D, Abla O, Naqvi A, Weitzman S, vein thrombosis: review of the English-language Hitzler J: Lumbar spinal fluid collections in children neuroblastoma: a report from the Childhood Cancer literature between 1992 and 2006. Pediatrics 2007: treated with intrathecal chemotherapy: elevated CSF Survivor Study. Pediatric Blood and Cancer 2007: 120: pp e1278-e1284. protein as a diagnostic clue. Pediatric Blood and 49: pp 704-715. Cancer 2008: 51: pp 295-298. Lau LM, Nugent J, Zhao X, Irwin MS: HDM2 antagonist Nutlin-3 disrupts p73-HDM2 binding and enhances p73 function. Oncogene 2008: 27: pp 997-1003. 80 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Rosenberg PS, Alter BP, Link DC, Stein S, Rodger E, Nathan PC, Patel S, Dilley K, Goldsby R, Harvey J, Punnett A, Sung L, Price V, Das P, Diezi M, Doyle J, Jacobsen C, Kadan-Lottick N, McKinley K, Millham AK, Dupuis LL: Achievement of target cyclosporine Boyard AA, Aprikyan AA, Bonilla MA, Dror Y, Moore I, Okcu MF, Woodman CL, Brouwers P, concentrations as a predictor of severe acute GvHD Kannourakis G, Newburger PE, Boxer LA, Dale DC: Neutrophil elastase mutations and risk of leukemia Armstrong FD: Guidelines for identification of, in children undergoing haematopoietic stem cell advocacy for, and intervention in neurocognitive transplantation and receiving cyclosporine and in severe congenital neutropenia. British Journal of problems in survivors of childhood cancer. Archives methotrexate prophylaxis. Therapeutic Drug Haematology 2008: 140: pp 210-213. of Pediatrics and Adolescent Medicine 2007: 161: Monitoring 2007: 29: pp 750-757. Rubinger M, Lillicrap D, Rivard GE, Teitel J, Carcao M, pp 798-806. Hensman C, Walker I, and the Association of Qaddoumi I, Musharbash A, Elayyan M, Mansour A, Nicholson A, Berger K, Bohn R, Carcao M, Fischer K, Al-Hussaini M, Drake J, Swaidan M, Bartels U, Hemophilia Clinic Directors of Canada: A prospective Gringeri A, Hoots K, Mantovani L, Schramm W, Bouffet E: Closing the survival gap: implementation of surveillance study of factor VIII inhibitor development Van Hout BA, Willan AR, Feldman BM: Recommendations medulloblastoma protocols in a low-income country in the Canadian haemophilia A population following for the reporting of economic evaluations of hemophilia through a twinning program. International Journal of the switch to a recombinant factor VIII product prophylaxis: a nominal group consensus statement on Cancer 2008: 15: pp 1203-1206. formulated with sucrose. Haemophilia 2008: 14: pp 281-286. behalf of the economics expert working group of the International Prophylaxis Study Group. Haemophilia Ramphal R, Grant RM, Dzolganovski B, Constantin J, 2008: 14: pp 127-132. Tellier R, Allen U, Weitzman S, Matlow A, Petric M, Rujkijyanont P, Watanabe KI, Ambekar C, Wang H, Sung L: Herpes simplex virus in febrile neutropenic Beyene J, Dror Y: Sbds-deficient cells undergo Pongtanakul B, Das PK, Charpentier K, Dror Y: children undergoing chemotherapy for cancer: accelerated apoptosis through the Fas-pathway Outcome of children with anaplastic anemia treated a prospective cohort study. Pediatric Infectious and not through the Bax/Bc12/BclXL pathway. with immunosuppressive therapy. Pediatric Blood and Diseases 2007: 26: pp 700-704. Haematologica 2008: 93: pp 363-371. Cancer 2008: 50: pp 52-57. Risebrough N, Oh P, Blanchette V, Curtin J, Hitzler J, Scheinemann K, Weitzman S, Hitzler J, Doyle J, Price VE, Blanchette VS, Ford-Jones EL: The Feldman B: Cost-utility analysis of Canadian tailored Abla O: Isolated central nervous system relapse prevention and management of infections in children prophylaxis, primary prophylaxis and on-demand in childhood acute promyelocytic leukemia. Journal with asplenia or hyposplenia. Infectious Disease therapy in young children with severe haemophilia A. of Pediatric Haematology Oncology 2008: 30: Clinics of North America 2007: 21: pp 697-710. Haemophilia 2008: April 16 [Epub ahead of print]. pp 160-162. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 81 Shah N, Leaker MT, Teshima I, Baruchel S, Tabori U, Malkin D: Risk stratification in cancer Tomlinson D, Isitt JJ, Barron RL, Doyle J, Judd P, Abdelhaleem M, Ye C: Late-appearing Philadelphia predisposition syndromes: lessons learned from novel Gassas A, Naqvi A, Sung L: Determining the chromosome in childhood acute myeloid leukaemia. molecular developments in Li-Fraumeni syndrome. understandability and acceptability of an oral Pediatric Blood and Cancer 2008: 50: pp 1052-1053. Cancer Research 200: 68: pp 2053-2057. mucositis daily questionnaire. Journal of Pediatric Oncology Nursing 2008: 25: pp 107-111. Shah NC, Ray A, Bartels U, Rutka J, Bouffet E, Drake J, Tabori U, Revah G, Nathan P, Rachlis A, Malkin D: Tomlinson D, Judd P, Hendershot E, Maloney AM, Hawkins CE, Huang A: Diffuse intrinsic brainstem Toxicity and outcome of children with treatment- tumors in neonates. Journal of Neurosurgery, related acute myeloid leukemia. Pediatric Blood and Sung L: Establishing literature-based items for an Pediatrics 2008: 1(5): pp 382-385. Cancer 2008: 50: pp 17-23. oral mucositis assessment tool in children. Journal of Strahm B, Durbin AD, Sexsmith E, Malkin D: The Teo JT, Klaassen R, Fernandez CV, Yanofsky R, CXCR4-SDF1 alpha axis is a critical mediator of Wu J, Champagne J, Silva M, Lipton JH, Brossard J, rhabdomyosarcoma metastatic signalling induced by Samson Y, Abish S, Steele M, Ali K, Dower N, Sung L: Meaurement of oral mucositis in children: bone marrow stroma. Clinical and Experimental Uma Athale, Jardine L, Hand JP, Tsangaris E, a review of the literature. Supportive Care in Cancer Metastasis 2008: 25: pp 1-10. Beyene B, Dror Y: Clinical and genetic analysis 2007: 15: pp 1251-1258. Pediatric Oncology Nursing 2008: 25: pp 139-147. Tomlinson D, Judd P, Maloney AM, Hendershot E, of unclassifiable inherited bone marrow failure Sung L, Lange BJ, Gerbing RB, Alonzo RA and syndromes. Pediatrics 2008: 122: pp 139-148. Traivaree C, Blanchette V, Armstrong D, Floros G, Stain AM, Carcao M: Intracranial bleeding in Feusner J: Microbiologically documented infections and infection-related mortality in children with acute Terasaki M, Bouffet E, Fukushima S, Shigemori M: haemophilia beyond the neonatal period–the role of myeloid leukaemia. Blood 2007: 110: pp 3532-3539. Pilot trial of the rate of response, safety, and CT imaging in suspected intracranial bleeding. tolerability of temozolomide and oral VP-16 in patients Haemophilia 2007: 13: pp 552-559. Sung L, Nathan PC, Alibhai SMH, Tomlinson G, with recurrent or treatment-induced malignant central Beyene J: Meta-analysis: effect of prophylactic nervous system tumors. Surgical Neurology 2008: Truong T, Beyene J, Hitzler J, Abla O, Maloney AM, hematopoietic colony-stimulating factors on mortality 69: pp 46-50. Weitzman S, Sung L: Features at presentation predict children with acute lymphoblastic leukemia at low risk and outcomes of infection. Annals of Internal Medicine 2007: 147: pp 400-411. Tomlinson D, Bartels U, Hendershot E, Constantin J, for tumor lysis syndrome. Cancer 2007: 110: Wrathal G, Sung L: Challenges to participation in pp 1832-1839. paediatric palliative care research: a review of the literature. Palliative Medicine 2007: 5: pp 435-440. 82 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Tsuchida R, Das B, Yeger H, Shibuya M, Baruchel S, Kirby M: Haematology. In: The Hospital for Sick A phase I study of IV feneretinide in recurrent Thorner PS, Malkin D: VEGF/Flt1 signalling regulates Children Handbook of Pediatrics, 11th Edition. neuroblastoma. Baruchel S. New Advances recovery of proliferative capacity of tumor side- Elsevier, Toronto, 2007. Neuroblastoma Therapy ($3,200 per patient 2008) chemotherapy in osteosarcoma. Oncogene 2008: Malkin D: Molecular biology of childhood neoplasms. A phase I study of MIBG ultratrace in recurrent 27: pp 3923-3934. In: Molecular Basis of Cancer, 3rd Edition (Mendelsohn neuroblastoma. Baruchel S. New Advances J, Howley PM, Israel MA, Gray JW, Thompson CB, eds.). Neuroblastoma Therapy ($3,200 per patient 2008) population cells following exposure to cytotoxic Villani A, Bouffet E, Blaser S, Millar BA, Bartels UK: Saunders/Elsevier, Philadelphia, 2008. A phase I study of sorafenib in recurrent solid tumours. Inherent diagnostic and treatment challenges in germinoma of the basal ganglia: a case report and Malkin D, Helman L: Molecular biology of childhood Reference laboratory. Wideman B, Baruchel S. review of the literature. Journal of Neuro-oncology cancers. In: Principles and Practice of Oncology, 8th Children’s Oncology Group ($32,000 2007-2008) 2008: 88: pp 309-314. Edition (DeVita VT, Jr., Hellman S, Rosenberg SA, eds.). JB Lippincott Co. Philadelphia, 2008. dietary therapies for cancer. Pediatric Blood and Cancer 2008: 50: pp 494-497. Truong T, Weingarten K, Abla O: Oncology. In: The A phase I/II study of CP751,851 and IGF IR antibody in refractory/recurrent solid tumours. Baruchel S. Weitzman S, Egeler MR: Langerhans cell histiocytosis–update for the pediatrician. FUNDING Current Opinion in Pediatrics 2008: 20: pp 23-29. A group-wide phase II study of ET-743 in recurrent paediatric sarcoma. Baruchel S. Children’s Oncology Group ($75,000 2007-2010) Pfizer ($22,349 per patient 2008-2010) A phase I/II study of temsirolimus in refractory/ recurrent solid tumours (Funding includes an additional $31,800 per patient) Baruchel S. Al-Anazi A, Al-Ansara K, Al-Farsi S, Al-Harbash Z, Benseler S, Calpin C, Cheng A, Do A, Freedman S, Sanofi -Aventis Pharmaceuticals Hospital for Sick Children Handbook of Pediatrics, 11th Edition. Elsevier, Toronto, 2007. BOOKS AND BOOK CHAPTERS A phase I study of VEGF trap in children with refractory solid tumours. Baruchel S. Weitzman S: Complementary and alternative (CAM) A phase 1 study of IV feneretinide in recurrent Harrington L, Jain S, Kirby M: Sickle cell disease. In: leukaemia and lymphoma–a TACL study. Baruchel S. The Hospital for Sick Children Handbook of Paediatric Therapeutic Advances Childhood Leukaemia Emergency Medicine (Lalani A, Schneeweiss S, eds.). Consortium ($20,000 per patient 2007) Wyeth CSA ($75,072 2008-2010) Jones and Bartlett Publishers, Toronto, 2007. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 83 Child Health Research Institute (Infrastructure for new A phase II molecularly targeted clinical trial defining Canadian Centre for Applied Cancer Genetics. the optimum biological dose of gleevec (imatinib Malkin D, Weksberg R, Meyn S, Ray P, Becker L, Research Building). Rossant J, Malkin D, 24 other mesylate) in paediatric CNS tumours–a multicentre Rutka J, Yeger H, Zielenska M, Alman B. Canada co-applicants. Canada Foundation for Innovation ($91 million 2008-2013) North American study. Baruchel S, Kuttesh J, Foundation for Innovation 40%, OIT 40%, and Goldman S, Bouffet E. Novartis Pharmaceuticals Ballard Foundation 20% of total ($1,811,000 Canada Inc. ($160,000 2003-2008) 2002-2008; additional $216,000 operating funds Childhood Cancer Survivor Study (CCSS) expansion. through OIF 2005-2007) Robison L, Nathan P. National Institute of Health/ National Cancer Institute ($27,365,542 2005-2010) A phase II study of vinblastine in chemotherapy naive children with progessive low grade gliomas. Bouffet E, Cancer and chemosensitivity. Irwin MS. Premier’s Tabori U, Hawkins C, Keene D. Ontario Institute of Research Excellence Award ($150,000 2004-2009) Children with posterior fossa brain tumours: quality of life and predictors of outcome. Kulkarni A, Bouffet E, Cancer Research ($430,197 2007-2010) Career Development Award. Sung L. Canadian Child Pakulis J, Ellerton C, Shams I. b.r.a.i.n. child Acute megakaryocytic leukaemia in Down’s syndrome. Health Clinician Scientist Training Program ($42,685 2006-2008) Hitzler J. National Cancer Institute of Canada ($280,000 2005-2009) Chinese immigrant families’ experiences of care for ($137,484 2006-2009) Careful transitions: developing an integrated service their child with cancer. Klassen A, Sung L, Raina P, Biochemical and genetic analyses of JPO2, a novel delivery model for paediatric palliative care. Antle B, Shaw N, Dix D, Pritchard S, Poureslami I. Childhood c-Myc oncogene interacting protein. New investigators Newman C, Beaune L, Rugg M, Wheat A, Bouffet E, Cancer Foundation ($92,536 2007-2009) equipment grant. Huang A. National Cancer Institute Laxer R, Frager G, Wagner F. SickKids Foundation of Canada ($29,479 2006-2009) ($129,900 2006-2008) Clinical Trial Consortium. Pappo A, Bouffet E, Baruchel S. Ontario Cancer Research Network ($1,000,000 2005-2008) Biochemical and genetic analyses of JPO2, a novel of Characterization of generic alterations that converge c-Myc oncogene interacting protein. Huang A. National on cell adhesion pathways in paediatric sPNET. Cancer Institute of Canada ($440,082 2006-2009) Huang A. b.r.a.i.n. child ($32,000 2008) Comparative effectiveness of conventional and Characterization of JPO2, a novel c-Myc co- trial participation. Gallie BL, Chan HSL, Eysenbach G, transforming protein. Rodrigues T and Huang A. Kushniruk A, Heon E. Ontario Institute for Cancer informatics-assisted clinical management and clinical Canadian Institute of Health Research Doctoral Research ($446,066 2003-2006 with two-year no Research Awards ($63,000 2007-2010) cost extension 2007-2008) 84 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Complications vasculaires de l’irradiation crânienne Development of a feasible tool to measure oral Genetic causes of neutropenia and susceptibility pour tumeur cérébrale durant l’enfance et mucositis in children with cancer. Sung L, Judd P, to leukaemia in patients with inherited neutropenia. l’adolescence. Bouffet E, Bernier V, de Vathaire F, Tomlinson D, Doyle JJ, Hendershot EI, Maloney AM, Dror Y. Neutropenia Association of Canada Doz F, Grill J, Frappaz D, Plantaz D. Commission Feldman BM. Paediatric Oncology Group of Ontario ($30,000 2007-2008) d’Epidémiologie du Conseil de Radioprotection EDF ($13,334 2007-2008) Genetic determinants of predisposition to childhood ($90,000 2008-2009) Establishing surrogate markers of angiogenesis in brain tumour initiation and progression. Tabori U, Core neuro-cognitive functions in children treated for paediatric oncology phase I and II clinical trials: Malkin D. b.r.a.i.n. child ($49,500 2008-2010) posterior fossa tumours. Mabbott DJ, Bouffet E, a Children’s Oncology Group Study. Baruchel S, Noseworthy M, Laughlin S, Kulkarni A, Laperriere N, Stempak D, Bertolini F, Entz-Werle N. Alex’s Genetic epidemiology of osteosarcoma. Spector L, Sharpe M, Keene D, Montour-Proulx I, Briere M, Lemonade Stand Foundation ($198,417 2007-2009) Malkin D, Ross J, Sklar C, Nagarajan R. National Institutes of Health ($1,000,000 2006-2011) Strother D, Witon A, Wilson B, Chafe S, McConnell D, Hukin J, Fryer C. National Cancer Institute of Canada Establishment of a comprehensive genetic bank for ($750,000 2007-2012) children with brain tumours. Tabori U, Malkin D. Genetic variation and risk of infection in children with b.r.a.i.n. child ($42,600 2008-2010) primary acute myeloid leukaemia. Sung L. Ministry of Research and Innovation ($100,000 2007-2011) Cross-cultural validation of the CHO-KLAT (Canadian Haemophilia Outcomes–Kids’ Life Assessment Tool). Evaluation of biomarkers in relation to recurrence rate Blanchette V, Young N, McCusker P. Bayer Health in childhood ependymoma. Hukin J, Tabori U, Hawkins C. Genetics, genomic research and consent: attitudes Care Canada ($260,000 2006-2008) C-17 Research Network ($140,000 2007-2009) of paediatric cancer survivors. Patenaude A, Malkin D. Defining the clinical phenotype of inherited marrow Functional analysis of the p53 family members National Institutes of Health ($100,000 2006-2008) failure syndromes and the risk factors of severe p73 and p63 roles in apoptosis, development and Genomic analyses of paediatric supratentorial haematological complications and cancer by the cancer. Irwin MS. Canada Research Chair, Tier II primitive neuroectodermal tumours. Huang A, Li M, Canadian Inherited Marrow Failure Registry (CIMFR). ($500,000 2003-2008) Rutka J. American Brain Tumour Association Basic Science Research Award ($70,000 2006-2008) Dror Y. Council of Canadian Paediatric Haematology/ Oncology Directors Research Network Genetic alterations converge on cell adhesion ($83,000 2005-2008) pathways in childhood sPNET. Huang A. Brain Tumour Society USA ($200,000 2008-2010) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 85 Group Chair Grant, Children’s Oncology Group. Infrastructure Grant Paediatric Consortium. Mechanism of pre-B cell leukemogenesis. Subversion Institutional Principal Investigator. Malkin D. National Blanchette VS, Devine S, Weitzman S, Portwine C, of developmental and survival programs in Cancer Institute (US$900,000 2003-2008) Halton J, Jardine L, Silva M. Ontario Cancer Research leukemogenesis. Guidos C, Hitzler J. Specialized Network ($990,404 2006-2011) Center of Research, Leukaemia Lymphoma Society ($750,000 2004-2009) Hope: the complex journey of parents of children with cancer. Antle B, Barrera M, Beaune L, Capra M, Immune thrombocytopenic purpura (ITP) quality D’Agostino N, Maloney AM, Nicholas D, Volpe J. of life instrument cross-cultural validation study. Moderate term musculoskeletal outcomes with Canadian Institutes of Health Research Blanchette VS, Klaassen R, Young N. Amgen escalating dose prophylaxis: the Canadian ($291,067 2005-2008) b.r.a.i.n.child Canada ($45,000 2007) Haemophilia Prophylaxis Follow-Up Study (CHPS-3). International registry of paediatric cutaneous T-cell 2006-2009) Feldman BM, Blanchette Versus Bayer ($742,500 ($25,000 2005-2008) Identification of genetic pathways that regulate lymphoma: a longitudinal, prospective, observation survival and development of cancer and cancer study of cutaneous T-cell lymphoma in the paediatric Molecular and pharmacologic correlates of acute stem cells. Guidos C, Hitzler J. Genome Canada population. Pope E, Weinstein M, Weitzman S, myeloid leukaemia in Down syndrome. Taub J, Hitzler J. ($330,000 2005-2009) Taylor G. Canadian Dermatology Foundation National Institutes of Health ($2,189,783 2007-2011) ($10,000 2004-2008) Molecular basis of CNS leukaemia. Subversion of Identification of interstitial germline deletions in developmental and survival programs in children with complex clinical syndromes that In vivo antitumour and antimetastatic activity of include medulloblastoma. Taylor M, Bouffet E. administration of CXCR4 antagonist and anti-CTLA4 leukemogenesis. Danska J, Hitzler J. Specialized Pediatric Brain Tumour Foundation Institute Award in neuroblastoma. Baruchel S. Pfizer Canada Inc. Centre of Research, Leukaemia Lymphoma Society ($100,000 2006-2008) ($45,000 2007-2008) ($750,000 2004-2009) Identification of leukemogenic genes that convert a In vivio anti-tumour and anti-metastatic activity of Molecular determinants of tumour formation in normal human haematopoietic cell into a leukemic administration of sutent and low-dose chemotherapy Li-Fraumeni Syndrome. Malkin D. National Cancer stem cell. Dick J, Hitzler J. Specialized Centre of in preclinical neuroblastoma mouse model. Baruchel S., Institute of Canada ($423,000 2007-2010) Research (SCOR), Leukaemia Lymphoma Society Zhang L. Pfizer Canada Inc. ($50,000 2007-2008) Grant ($748,575 2004-2009) 86 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT National study of prophylaxis in adolescents and P53 Family in cancer and chemosensitivity. Phase II study of high dose chemotherapy with young adults with inherited severe bleeding disorders. Irwin MS. Premier’s Research Excellence Award cyclosporin for vision-threatening intraocular Carcao MD, Blanchette VB, Chan A, Teitel J, Lillicrap D, ($150,000 2004-2009) retinoblastoma. Chan HSL, Gallie BL, Heon E. Ontario Institute for Cancer Research ($551,723 2003-2008) Wu J. Bayer/Canadian Blood Services/Hema-Quebec Partnership Fund ($165,899 2007-2008) P73 Modulatory proteins: roles in apoptosis and chemosensitivity. Irwin MS. National Cancer Pilot study of a comprehensive lifestyle program in Neuro-imaging of white matter and neuro-cognitive Institute of Canada/Canadian Cancer Society children receiving treatment for acute lymphoblastic outcome following cranial radiation for paediatric brain ($400,682 2007-2010) leukaemia (ALL). Nathan P, Wright M, Collins L, Birken C, Dettmer E. Pediatric Oncology Group of Ontario tumours. Mabbot DJ, Bouffet E, Noseworthy M, Suzanne M, Kulkarni A, Norman L, Michael S, Paediatric Phase I Consortium. Baruchel S, Pappo A, Montour-Proulx I, Daniel K, Strother D, Witol A, Gammon J. National Cancer Institute/Children’s Wilson B, Chafe S, McConnel D, Hukin J, Freyer C. Oncology Group ($250,000 2002-2007) ($15,000 2008) Pilot study of once-a-day prophylaxis for youth and young adults with severe haemophilia A. Feldman B, C17 Research Network ($95,000 2006-2008) Neuroblastoma stem cell bank and a preclinical Pain in child health: an innovative, transdisciplinary, Rivard G, Carcao M, St-Louis J, Teitel J, Pullenayegum E, cross-Canada research training consortium. McGrath PJ, Blanchette V. Bayer/Talecris/Canadian Blood research unit in novel therapeutic drug discovery. Barr R, Baruchel S, Brown S, Camfield C, Chambers C, Services/Hema-Quebec Partnership Fund ($200,000 Baruchel S. Solving Kids Cancer NYC Connolly J, Craig K, Dube L, Feldman B, Finley G, Frager G, 2007-2009) ($400,000 2008-2009) Gibbins S, Grunau R, Harman K, Hetherington C, Howlett A, Johnston C, Kenny N, Koren G, Langer J, Predicting the risk of infection in children receiving New surrogate markers of angiogenesis in paediatric Murray M, Oberlander T, Ohlsson A, Otley A, Rathwell T, chemotherapy for acute myeloid leukaemia. Sung L, brain tumours. Baruchel S, Bartels U, Hawkins C, Salter M, Schellinck H, Stevens B, Taddio A, Unruh A, Allen U, Beyene J. Canadian Institute for Health Stempak D, Bouffet E, Abdelhaleem M, Doyle J. Von Baeyer C, Walker C. Canadian Institutes of Health Research ($582,104 2008-2012) b.r.a.i.n.child ($97,446 2006-2008) Research ($1,800,000 2002-2008) Predicting the risk of infection in children receiving chemotherapy for acute myeloid leukaemia. Sung L, Orphan late effect in paediatric brain tumour survivors. Palliative chemotherapy versus supportive care alone Lafay-Cousin L, Sockett E, Bouffet E. b.r.a.i.n.child for end-of-life. Sung L, Hinds P, Llewellyn-Thomas H, Beyene J, Allen U. National Cancer Institute of ($30,000 2005-2007) Volpe J, Capra M, Gammon J, Baruchel S, Bouffet E, Canada ($817,963 2008-2012) Barrera M, Tomlinson D, Greenberg ML. National Cancer Institute of Canada ($131,626 2005-2008) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 87 TACL 2006-001: a phase I study of intravenous Presentation, management and outcomes evaluation South Asian immigrant families’ experiences of care of children with vascular tumours. Pope E, Mahant S, for their child with cancer. Klassen A, Sung L, Raina P, fenretinide in children with recurrent or resistant ALL, Blanchette V, Weitzman S, Baruchel S, John P, Shaw N. National Cancer Institute of Canada AML and NHL. Harned T, Maurer B, Punnett A. Brandao L. The Hospital for Sick Children Paediatric ($139,067 2007-2009) Therapeutic Advances in Childhood Leukaemia ($20,000 per patient 2007) Consultants Partnership’s Grant ($9,900 2006-2008) Subversion of developmental and survival programs in Prognostic and therapeutic implications for telomere leukemogenesis. Project 1: identification of Telomere maintenance in the biologic behaviour of low- maintenance and telomerase inhibition in paediatric leukemogenic genes that convert a normal human grade gliomas. Malkin D, Tabori U. b.r.a.i.n. child high grade gliomas. Tabori U. Canadian Institute for hematopoietic cell into a leukemic stem cell. Mak T, ($60,000 2004-2007) Health Research ($385,811 2008-2011) Dick J, Hitzler J. Leukaemia Lymphoma Society ($748,575 2004-2009) Thalassemia Clinical Research Network Registry. Kirby M. National Institutes of Health ($45,696 2005-2010) Prospective cohort study of genetic variation and risk of infection in Canadian children with newly Subversion of developmental and survival programs in diagnosed acute myeloid leukaemia. Sung L, Beyene J, leukemogenesis. Project 2: mechanism of pre-B cell The biologic and prognostic role of replicative Allen U. National Cancer Institute of Canada leukemogenesis. Guidos C, Hitzler J. Leukaemia and oncogene induced senescence in paediatric ($444,424 2005-2008) Lymphoma Society ($750,000 2004-2009) low grade gliomas. Tabori U. Brain Tumour Society ($220,000 2007-2009) Role of p73 in neuroblastoma and sensitivity to Subversion of developmental and survival programs in The effects of additional videotaped information on therapy. Irwin MS. Elsie Winifred Crann Memorial leukemogenesis. Project 3: molecular basis of CNS Trust Award for Research in Life Sciences, University leukaemia. Danska J, Hitzler J. Leukaemia Lymphoma the experience of parents of children with newly of Toronto ($35,000 2008-2010) Society ($750,000 2004-2009) diagnosed acute lymphoblastic leukaemia (ALL). Abla Sibling and parent bereavement from childhood TACL 2005-003: a study of bortezomib with H. Novopharm/Childhood Cancer Foundation Grant cancer. Gerhardt C, Barrera M, Capra M. National chemotherapy for relapsed/refractory acute ($75,000 2008) Cancer Institute of Canada/The Hospital for Sick lymphoblastic leukaemia. Messinger Y, Bostrom B, Children ($92,000 2005-2010) Punnett A. Therapeutic Advances in Childhood The genetic basis of severe sickle cell disease: Leukaemia ($20,000 per patient 2007) the never lost international cohort study. Children’s O, Punnett A, Barrera M, Hetherington R, Rolbin-Tseitlin Discovery Institute ($149,968 2008-2012) 88 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Validation of a new paediatric scoring system from the The Kids’ ITP Tools (KIT). Licensing. Blanchette VS, The role of VPS33B in platelet alpha granule formation. Young N, Klaassen R, Barnard D. Amgen Canada Kahr WHA. Heart and Stroke Foundation of Ontario International Haemophilia Prophylaxis Study Group: ($90,000 2007) ($210,750 2006-2009) validity and reliability of the combined haemophilia The Ontario Paediatric Oncology Clinical Trials The role of VPS33B in platelet alpha granule formation. Funk S, Bergstrom BM, Zourikian, Hilliard P, Research Network. Blanchette V, Malkin D, Devine S, Kahr WHA. Canadian Institutes of Health Research van der Net JJ, Engelbert R, Petrini P, van den Berg HM, Baruchel S, Weitzman S, Bouffet E. Ontario Cancer Canada ($441,912 2006-2010) Manco-Johnson M, Rivard G. Bayer Healthcare LLC, joint health score (HJHS). Feldman B, Blanchette VS, Biological Products Division ($195,000 2005-2007) Research Network ($1.2 million 2006-2009) To improve infrastructure for paediatric cancer clinical The prevalence of symptomatic von Willebrand disease trials. Pappo A, Malkin D, Devine S, Hitzler H, Silva M, Validation of the oral mucositis daily questionnaire and in the paediatric population. James PD, Blanchette VS, Barr R, Halton J, Jardine L, Baruchel S, Bouffet E. evaluation of importance of mucositis in children with Lillicrap D, Silva M, Rand ML, Brandao L. Canadian Ontario Cancer Research Network ($990,404 acute leukaemia, non-Hodgkin’s lymphoma and stem Haemophilia Society ($139,800 2007-2009) 2005-2010) cell transplantation recipients. Sung L, Tomlinson D, Judd P, Gassas A, Naqvi A, Doyle J. Connaught New The role of SBDS in granulopoiesis. Dror Y. SickKids Foundation ($64,480 2006-2008) Staff Matching Grant ($29,978 2008-2010) Treatment choices for children with typical acute immune thrombocytopenic purpura: development of a decision aid. Beck C, Parkin P, Birken C, Blanchette V, Validation of the oral mucositis daily questionnaire The role of SBDS in promoting cell survival. Boydell K, Llewellyn-Thomas H. Dean’s Fund New and evaluation of importance of mucositis in children Dror Y. Canadian Institutes of Health Research Staff Competition ($10,000 2006-2011) with acute leukaemia, non-Hodgkin’s lymphoma and stem cell transplantation recipients. Sung L, ($250,000 2006-2011) Understanding collaboration in a shared care practice Tomlinson D, Judd P, Gassas A, Naqvi A, Doyle J. The role of SBDS in promoting cell survival. of paediatric oncology patients: a pilot study. Punnett A, Pediatric Oncology Group of Ontario ($13,166 2008-2009) Dror Y. SDS Project Inc. ($60,000 2006-2008) Lingard L. The Hospital for Sick Children Paediatric Consultants Partnership’s Grant ($7,024 2007-2008) The roles of the novel p53 family proteins in Weekly vinblastine for chemotherapy naïve children with progressive low grade glioma. Bouffet E, Pond G, cancer and chemosensivity. Irwin MS. Canada Moghrabi A, Carret AS, Jabado N, Samson Y, Crooks B, Research Chair, Tier II Renewal (Salary Award) Hand JP, Strother D, Wilson B, Hukin J, Mpofu C, ($500,000 2008-2013) Whitton A, Zelcer S, Silva M, Brossard J, Johnston D, Keene D, Bartels U, Tabori U, Hawkins C. Ontario Institute of Cancer Research ($536,885 2007-2010) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 89 Dr. Chaim Roifman DIVISION HEAD Dr. Chaim Roifman FULL-TIME Dr. Adelle Atkinson Dr. Eyal Grunebaum PART-TIME Dr. Milton Gold Dr. David Hummel Dr. Sasson Lavi Dr. Maria Triassi Dr. Stephen Feanny CLINICAL FELLOWS Dr. Fotini Kavadas Dr. Elana Lavine Dr. Amit Nahum Dr. Vy Kim Dr. Nashat Al-Sukaiti CLINICAL ASSISTANT Dr. Julia Upton ALLERGY NURSE Elisabeth White IMMUNOLOGY NURSE Brenda Reid SENIOR SCIENTIST Dr. Amos Cohen RESEARCH ASSOCIATE Dr. Nigel Sharfe Division of Immunology/Allergy OVERVIEW The Hypogammaglobulinemia Program at SickKids is cent survival rate is achieved by a widely used The Division of Immunology/Allergy continues as a recognized globally for its innovative approach and standard protocol exercised in most other countries leader in patient care, research and education in long-term follow up of over 150 patients. We in Europe and the U.S. Our protocol has become the benchmark for treatment of severe combined immunology. This year marks the launch of the transformed the therapy of these patients from International Network of JMF Centres of Excellence multiple hospital-based admissions to visiting the immunodeficiency when a full HLA-matched related in Immunodeficiency. Under Dr. Roifman’s leadership, short stay unit and subsequently receiving treatment donor is not available and it has been adapted by many transplant centres across the world. In addition, we these international programs are facilitating early in their community. The proficiency of our team, recognition, diagnosis, research and effective coupled with convenient care at a medical centre have shown that this protocol has excellent results treatment of primary immune deficiencies. The close to home, provides our patients with the in a group of patients with residual T-cells. network will collaborate with the NIH (U.S.A.) on a personalized treatment they require. To further 12-month grant program in support of research in enhance this program, we are now implementing Our scientific and clinical achievements lead to much primary immunodeficiency (PID). home therapy as the ideal model of treatment. Our recognition. This includes membership by Dr. Roifman recent symposium for a Canadian consensus on home in the World Health Organization Scientific Committee The Primary Immunodeficiency Program at The Hospital treatment has resulted in funding by the Canadian for Clinical Immunology, which defines the for Sick Children is the largest centre of its kind in Immunodeficiency Society and a publication in the classification of PIDs. Similarly, we are commonly Canada and one of the largest in the world. Medical Journal of Clinical Immunology. editing a clinics of North America book on intravenous immunoglobulin therapy. services are provided to over 2,000 outpatients referred from physicians and hospitals nationally. Innovative protocols created by members of the This number has steadily increased by 30 per cent division have elevated the profile of the Bone Marrow Research remains a major focus of activity in the over the last 10 years. To diminish waiting time in the Transplantation (BMT) Program as one of the best in division. Dr. Roifman’s laboratory continues to develop Immunology Clinic, we have recently established the the world for survival of PID patients. In 1990, we innovative anti-cancer treatments. Novel compounds Complex Immunodeficiency Clinic. This addition has pioneered the implementation of matched unrelated were designed against targets in solid tumours which improved evaluation of our complex patients by donor transplants for PID patients. Today, we have led to significant tumour shrinkage in vitro and in providing extensive time and attention to their demonstrate the impact of our procedures with recent vivo. Moreover, since Dr. Roifman’s lab synthesized conditions. publications in JAMA, BMT and JACI. These papers CR4, another novel compound capable of killing illustrate an 80 per cent survival rate for SickKids’ acute lymphocytic leukaemia (ALL) and acute myeloid patients utilizing our protocol and in some publications leukaemia (Blood 2003), it is currently being given 100 per cent survival rate. In contrast, only a 50 per to patients with ALL in a phase one clinical study RESEARCH TECHNOLOGISTS Lorand Cimpeon Tom Grunberger Yiping Gu Vivien Lee Weixian Min Alka Arora Yongmao Yu Murugesapillai Mylvaganam JunYan Zhang MEDICAL LAB TECHNOLOGISTS Andrea Bates Dr. Harjit Dadi ADMINISTRATIVE STAFF Jayne Enhaynes Ellis Dervis Sandra Mendonca conducted by LymphoSign. Recently, novel compounds Education remains an important activity of every HONOURS AND AWARDS which effectively kill multiple myeloma cells have been member of the division. The tireless dedication to Roifman CM: Schwartz-Reisman Israel Scholar, 2007. synthesized and tested. Meanwhile, the discovery of a teaching by your has recently been recognized by genetic aberration in the RNA component of RMRP has the approval of reviewers from the Royal College Roifman CM: Chair of the Jeffrey Modell Centres initiated testing of patients for this defect (JACI 2006). of Physicians and Surgeons. Beyond this program, Network, 2007. we continue to attract more than 25 outstanding Dr. Grunebaum and his group have successfully candidates every year from across the world. Reid B: Nurses Awareness Initiative, Canadian developed and described a novel method to efficiently International trainees have the opportunity to Immunodeficiency Society, 2007. deliver full length enzymes such as purine nucleoside participate in a flexible program which can be phosphorylase (PNP) into cells (Journal of Clinical predominantly research oriented. Our commitment PUBLICATIONS Investigation 2006). To further this endeavour, his to education goes beyond the training program. Al-Jenaidi F, Outi M’ekitie O, Grunebaum E, Sochett E: laboratory continues to develop safer methods of Dr. Roifman established a new international fellowship Parathyroid gland dysfunction in 22q11.2 deletion syndrome. Hormone Research 2007: 67: pp 117-122. inserting genes into bone marrow cells. This will program, The Robert A. Good/JM Fellowship in facilitate therapy for diseases such as PNP and Immunodeficiency and Transplantation. In addition, adenosine deaminase (ADA) deficiency, while Dr. Adelle Atkinson was granted the CI Society AL-Zahrani D, Gruneabum E, Roifman CM: Safety Physician’s Awareness Award for a third consecutive and efficacy of measles-mumps-rubella vaccine year. Similarly, Brenda Reid was presented with a on patients with DiGeorge/22q11 microdeletion In view of his academic achievements and his multiple nursing education grant by the CI Society for her syndrome. Pediatric Academic Society 2007: contributions to clinical immunology in Canada, the leadership in the teaching of PID care to nurses globally. 61: p e6293.14. Launched in 2007, the recipients of this research Thanks to the support of the Donald & Audrey Atkinson AR, Roifman CM: Low serum IgG2 levels improving the substandard outcomes for these patients. CI Society created the Chaim Roifman Scholar Award. grant will study the clinical, cellular and molecular Campbell Chair, the Jeffrey Modell Foundation, the CI in infancy can be transient. Pediatrics 2007: 120: characteristics of genetically determined immunodeficiency Society, Canadian Institute of Health Research, March pp e543-e574. diseases, discover improved diagnostic tools for PID, of Dimes, and the Canadian Centre for Primary research novel therapeutic approaches for PID, or Immunodeficiency, children who come for treatment in advance education of patients and medical our division will receive the best care possible, as professionals on PIDs. soon as possible. 92 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Geha RS, Notarangelo LD, Casanova JL, Chapel H, Kavadas F, Somech R, Pollanen M, Roifman CM: Manlhiot C, Tyrrell PN, Liang L, Atkinson AR, Lau W, Conley ME, Fischer A, Hammartrom L, Nonoyama S, Hemophagocytic lymphocytosis in a patient with Feldman BM: Safety of intravenous immunoglobulin in Ochs HD, Puck J, Roifman CM, Seger R, Wedgwood J: T-B+NK severe combined immunodeficiency. the treatment of junvenile dermatomyositis: adverse Primary immunodeficency diseases: an update from Pediatric Academic Society 2007: 61: pp e6286.9. reactions are associated with immunoglobulin A content. Pediatrics 2008: 121(3): pp e626-e630. the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Ku CL, von Bernuth H, Picard C, Zhang SY, Chang HH, Committee Meeting. Journal of Allergy and Clinical Yang K, Chrabieh M, Issekutz AC, Cunningham CK, Nofech-Mozes Y, Blaser SI, Roifman CM: Neurological Immunology 2007: 120(4): pp 776-794. Gallin J, Holland SM, Roifman CM: Selective manifestations in severe combined immunodeficiency predisposition to bacterial infections in IRAK-4 secondary to adenosine deaminase deficiency. Grunebaum E, Notarangelo LD, Roifman CM: deficient children: IRAK-4 dependent TLRs are Pediatric Neurology 2007: 37(3): pp 218-221. Successful correction of severe combined immune otherwise redundant in protective immunity. Journal of deficiency following transplantation with bone Experimental Medicine 2007: 204(10): pp 2407-2422. Roifman CM, Grunebaum E, Dalal I, Notorangelo LD: Lai YH, Gruneabum E, Atkinson A, Cutz E, Roifman combined immunodeficiency. Immunologic Research CM: Pulmonary and hepatic disease in a patient with 2007: 38: p 191. Matched unrelated bone marrow transplant for severe marrow from HLA-matched unrelated donors. Italian Journal of Pediatrics 2007: 33: pp 24-26. Grunebaum E, Roifman CM: Clinical and immunological partial adenosine deaminase (ADA) deficiency. features of patients with WHIM syndrome (warts, Pediatric Academic Society 2007: 61: p e6286.5. Somech R, Seaban-Adel K, Kirby-Allen M, Atkinson AR: Evaluation and management of pediatric patients with hypogammaglobulinemia, infections and myelokathexis) with or without mutations in the CXCR4 gene. Pediatric Lavine E, Picard C, Casanova JL, Roifman CM: Cellular anaphylactoid reactions to deferoxamine mesylate. Academic Society 2007: 61: p e6286.1. and humoral aberrations in a kindred with IL-1 receptor Annals of Allergy, Asthma and Immunology 2007: associated kinase 4 deficiency. Journal of Allergy and 99(6): pp 575-576. Husain M, Grunebaum E, Naqvi A, Atkinson A, Clinical Immunology 2007: 120(4): pp 948-950. Somech R, Grunebaum E, Roifman CM: Short-lived Ngan BY, Aiuti A, Roifman CM: Burkitt lymphoma in antibody response as a manifestation of primary adenosine deaminase deficient-severe combined Lavine E, Roifman CM: Clinical course and immunodeficiency treated with polyethylene glycol- immunological features of siblings with IRAK-4 deficiency. immunodeficiency. Pediatric Academic Society 2007: adenosine deaminase. Journal of Pediatrics 2007: Pediatric Academic Society 2007: 61: p e6286.6. 61: p e6286.2. 151: pp 93-95. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 93 BOOKS AND BOOK CHAPTERS FUNDING Irwin J, Atkinson AR: What’s a mother to do? In: A clincial study of the pharmacokinetics, efficacy by prolonged intracellular enzyme replacement Anaphylaxis Canada Newsletter. Anaphylaxis Canada, and safety of immune globulin intravenous (human) therapy. Grunebaum E. March of Dimes ($390,000 Omr-IgG-am IGIV in subjects with primary immune 2007-2010) Toronto, 2007. Correcting purine nucleoside phosphorylase deficiency deficiency diseases. Roifman CM, Roberts R, Kim Vy, Atkinson AR: Allergy and immunology. Melamed IR, Moy J, Grunebaum E, Sussman GL, Correcting purine nucleoside phosphorylase using In: The Hospital for Sick Chidlren Handbook of Chouksey A, Stein M, Wasserman RL, Levy R, McNeil lenti-virus mediated human PNP gene delivery into Pediatrics (Bismilla Z, Gupta S, Lam C, eds.). D, Suez D. FFF Enterprises ($550,000 2006-2008) haematopoietic stem cells. Grunebaum E. SickKids Foundation/Canadian Institutes of Health Research/ Elsevier, Toronto, 2008. A clinical study of the pharmacokinetics, efficacy Institute of Human Development, Child and Youth Roifman CM: Human interleukin-2 receptor alpha and safety of immune globulin intravenous (human) Health ($130,000 2006-2008) Deficiency. In: Primary Immunodeficiency Diseases: A Omr-IgG am’99 IGIV in subjects with primary immune Molecular and Genetic Approach (Ochs HD, Smith CIE, deficient diseases. Grunebaum E, Somech R. Puck JM, eds.). Oxford University Press, New York, 2007. FFF Enterprises ($55,000 2007-2008) Correcting PNP deficiency by intracellular delivery of PNP fused to protein transduction domains. Grunebaum E. US Immunodeficiency Network Sharfe N, Roifman CM: ZAP-70: 15 years on and still Canadian Centre for Primary Immunodeficiency. an expanding world. In: Lymphocyte Activation and Roifman CM. Canadian Centre for Primary Signal Transduction. Transworld Research Network, Immunodeficiency ($410,000 2003-2007) India, 2007. 94 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT (US$330,000 2006-2008) Fellowship in immunology. Roifman CM. Canadian Friends of Tel-Aviv University ($55,000 2006-2007) Genetics of primary immunodeficiency. Roifman CM. Novel synthetic styryl-acrylonitrile inhibitors of tumour Primary immunodeficiency (PID) Registry. Roifman CM. Canadian Immunodeficiency Society ($100,000 angiogenesis: in vivo translation. Roifman CM. Ontario Canadian Blood Services/Bayer ($194,150 2004-2008) 2007-2010) Cancer Research Network ($512,179 2004-2007) Home immunoglobulin therapy. Roifman CM. Canadian Nursing awareness for primary immune deficiency. diseases. Grunebaum E. Jeffrey Modell Foundation Immunodeficiency Society ($430,000 2007-2011) Reid B. Canadian Immune Deficiency Society ($11,000 2006-2007) Retrovirus gene therapy for primary immunodeficiency ($6,500 2007) Immunology and transplantation. Roifman CM. Role of Eph receptors in the immune system. McMillan Binch Barristers and Solicitors ($75,000 Pathogenesis of immune malignancies. Roifman CM. Roifman CM. Canadian Institutes of Health Research, 2002-2007) Audey Stanleigh Leukaemia Research Foundation 2nd renewal ($350,000 2004-2007) ($52,500 2005-2008) Molecular basis of primary immunodeficiency. Roifman CM. Jeffrey Modell Foundation ($250,000 2006-2011) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 95 Dr. Ian Kitai Dr. Anupma Wudhwa DIVISION HEAD Dr. Upton Allen FULL-TIME Dr. Sean Ari Bitnun Dr. Anne G. Matlow Dr. Anne Opavsky Dr. Stanley E. Read Dr. Dat Tran Dr. Anupma Wadhwa Dr. Valerie Waters PART-TIME Dr. Jason Brophy Dr. Ian Kitai Dr. Alana Rosenthal CROSS-APPOINTEES Dr. Susan Richardson Dr. Sharon Walmsley Dr. Elaine Wang HONORARY Dr. Susan King CLINICAL FELLOWS Dr. Tariq Al Fawz Dr. Amal Al-Maani Dr. Hadi Al-Qahtani Dr. Michelle Bridge Dr. Yuho Horikoshi Dr. Spenta Kakalia Dr. Fatima Kakkar Dr. Jackeline Pando Kelly Dr. Caroline Kerrison Dr. Ronan Timothy Leahy Dr. Shaun Morris Dr. Wilson Pau RESEARCH FELLOWS Dr. Michelle Barton-Forbes Dr. Spenta Kakalia Dr. Sean O’Riordan Division of Infectious Diseases OVERVIEW Our research initiatives are structured around themes In the area of education, representatives from our The Division of Infectious Diseases is a leader in clinical, that include host susceptibility and related factors division continue to play a key role in activities of the educational and research activities locally, nationally and innovative antimicrobial and associated therapies. Royal College of Physicians and Surgeons of Canada, and internationally. The division has active inpatient as Specific research initiatives include multicentred and in many areas of undergraduate, graduate, post well as ambulatory consultant services that include paediatric HIV studies related to maternal-to-child graduate and continuing medical education activities. teams that focus on general infectious diseases and transmission and studies on new antiretrovirals, Faculty members continue to hold editorships, visiting infectious diseases in immunocompromised hosts, vaccine studies and research into the mechanisms of professorships and related academic honours respectively. We are a resource for issues related to host susceptibility to viral infections. These infections nationally and internationally. infectious disease outbreaks, including new and include viral myocarditis, influenza illness, HIV, emerging infections, infection control and the EBV-related post-transplant lymphoproliferative HONOURS AND AWARDS specialized areas of HIV, congenital infections, disorder (PTLD) and encephalitis. Multicentred trials Allen U: Advanced to Fellow, Infectious Diseases management of infectious complications or organ related to the diagnosis and prevention of infections Society of America. transplantation, and the ever-increasing problem in transplant patients are ongoing, as well as of multi-drug resistant tuberculosis. participation in national surveillance studies (IMPACT) Allen U: Honorary Fellowship, Royal College of and national collaborative paediatric studies through Physicians of UK. Division members play a leading role in national the Paediatric Investigators Collaborative Network on and international epidemiological, clinical, research Infections in Canada (PICNIC). Our research in Allen U: Invited discussant. Challenging cases: advisory and related committees (e.g., the Infectious infection control includes the clinical and molecular annual meeting. Infectious Diseases Society of Diseases Society of America, the Canadian Paediatric epidemiology of paediatric nosocomial infections. America, San Diego, CA, 2007. Transplantation, the Government of the Bahamas The division has continued to have a leadership role in Allen U: Keynote lecture – 60th anniversary, and the HIV/AIDS program in Russia). structuring the approach to the management of major Faculty of Medicine, University of the West Indies. Society, Health Canada, the American Society for emerging and resurgent infectious disease threats. In this regard, the division has been very actively Allen U: Visiting professor and external examiner, involved in influenza pandemic planning efforts at University of West Indies, Jamaica. SickKids as well as provincially and nationally. CLINICAL NURSES Debra Louch Georgina MacDougall RESEARCH ASSOCIATE Dr. Nasser Khodai Booran CLINICAL RESEARCH NURSE COORDINATORS Cheryl Arneson Suganya Lee Brenda O’Doherty Kim Simpson ADMINISTRATIVE STAFF Brenda Bishop Marsha Frances-Reid Savannah Hatt Wayne Moore Zita Rotar Bebi Wali THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 97 Brophy J: Clinical Track Rapporteur, Canadian Allen U and the Infectious Diseases and Immunization Allen U and the Infectious Diseases and Immunization Association for HIV Research, 2007. Committee, Canadian Pediatric Society: Human Committee, Canadian Paediatric Society: West Nile papillomavirus vaccine for children and adolescents. virus in the context of climate change. Paediatrics and Paediatrics and Child Health 2007: 12: pp. 599-603. Child Health 2008: 13: pp. 399-400. Allen U and the Infectious Diseases and Immunization Amin R, Richardson SE, MacGregor D, Tellier R, Heurter Matlow A. Sydney Marie Snyder Endowed Lectureship, inaugural talk: To err is human but what about the kids? Children’s National Medical Centre, Washington, DC, 2008. PUBLICATIONS Committee, Canadian Paediatric Society: Infection H, Fearon M, Ford-Jones E, Bitnun A: Acute childhood control in paediatric office settings. Paediatrics and encephalitis and encephalopathy associated with Child Health 2008: 13: pp. 408-419. influenza: a prospective 11-year review. Pediatric Infectious Disease Journal 2008: 27: pp 390-395. Allen U: Current status and impact of CMV prevention following pediatric organ transplantation. Pediatric Allen U and the Infectious Diseases and Immunization Transplantation 2008: 12: pp 389-392. Committee, Canadian Pediatric Society: Needle stick injuries in the community. Paediatrics and Child for preventing and treating influenza in children: Allen U: Immunizations for children with cancer. Health 2008: 13: pp. 205-210. commentary. Evidence-Based Child Health 2008: Barton MA, Allen U: Neuraminidase inhibitors 3: pp 585-588. Pediatric Blood and Cancer 2007: 49(S7): pp 1102-1108. Allen U and the Infectious Diseases and Immunization Committee, Canadian Pediatric Society: Prevention of Brophy J, Yau Y, Cox P, Katz K, Bitnun A: A sentinel Allen U and the Infectious Diseases and Immunization congenital rubella syndrome. Paediatrics and Child fatal paediatric case of community associated Committee, Canadian Pediatric Society: Antifungal Health 2007: 12: pp. 795-77. methicillin-resistant Staphylococcus aureus in Canada. Paediatrics and Child Health 2007: 12 pp 319-322 agents for common pediatric infections. Paediatrics and Child Health 2007: 12: pp. 875-878. Allen U and the Infectious Diseases and Immunization Committee, Canadian Pediatric Society: Testing for Conwell LS, Forrest CR, Allen U, Perlman K, Allen U and the Infectious Diseases and Immunization HIV infection in pregnancy. Paediatrics and Child Daneman D: Necrotizing fasciitis in adolescents with Committee, Canadian Pediatric Society: Autistic Health 2008: 13: pp. 221-224. poorly controlled Type 1 diabetes mellitus: report of two cases. Pediatric Diabetes 2007: 8: pp 397-400. spectrum disorder: No causal relationship with vaccines. Paediatrics and Child Health 2007: Allen U and the Infectious Diseases and Immunization 12: pp. 393-395. Committee, Canadian Pediatric Society: Therapy of suspected bacterial meningitis in Canadian children six weeks of age and older. Paediatrics and Child Health 2008: 13: p 309. 98 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Elbers J, Bitnun A, Richardson SE, Ford-Jones L, Tellier Kam A, Ford-Jones L, Malloy P, Khan K, Kitai I: Active Matlow A, Stevens P, Urmson L, Wray R. Improving R, Wald R, Petric M, Kolski H, Heurter H, MacGregor tuberculosis among adolescents in Toronto, Ontario: patient safety through a multi-faceted internal D: Childhood herpes simplex encephalitis: a 12-year clinical features and delays in diagnosis. Pediatric surveillance program. Healthcare Quarterly 2008: prospective study of childhood herpes simplex Infectious Diseases Journal 2007: 26: pp 355-356. 11(3): pp 101-108. Paediatrics 2007: 119: pp 399. Koo A, Sung L, Allen U, Naqvi A, Drynan-Arsenault J, Ofner-Agostini M, Johnston BL, Simor AE, Embil J, Dekker A, Dupuis LL: Efficacy and safety of Matlow A, Mulvey M, Ormiston D, Conly J, Canadian Gravel D, Taylor G, Ofner M, Johnston L, Loeb M, caspofungin for the empiric management of fever in Nosocomial Infection Surveillance Program: Roth VR, Stegenga J, Bryce E: The Canadian neutropenic children. Pediatric Infectious Diseases Vancomycin-resistant enterococci in Canada: results Nosocomial Infection Surveillance Program, Matlow A: Journal 2007: 26: pp 700-704. encephalitis: is there a broader spectrum of disease? from the Canadian nosocomial infection surveillance program, 1999-2005. Infection Control and Hospital Point prevalence survey for health care-associated Epidemiology 2008: 29(3): pp 271-274. infections within Canadian adult acute-care hospitals. Kumar D, Humar A, Plevneshi A, Siegal D, Franke N, Journal of Hospital Infection 2007: 66(3): pp 243-248. Green K, McGeer A, Matlow A: Toronto Invasive Bacterial Diseases Network: Invasive pneumococcal Ota K, Profiti R, Smaill F, Matlow A, Smieja M: Grisaru-Soen G, Lau W, Arneson C, Louch D, Read SE, disease in adult hematopoietic stem cell transplant Identification badges: a potential fomite? Canadian Journal of Infection Control 2007: 22(3): pp 165-166. Bitnun A, Stephens D, King SM: A randomized recipients: a decade of prospective population-based trial (RCT) to assess the effect of withdrawal of surveillance. Bone Marrow Transplantation 2008: intravenous immune globulin (IVIG) therapy for 41(8): pp 743-747. Ramphal R, Grant RM, Dzolganovski B, Constantin J, Tellier R, Allen U, Weitzman S, Matlow A, Petric M, selected children with HIV infection. Pediatrics Manlhiot C, Liang L, Tran D, Bitnun A, Tyrrell PN, Sung L: Herpes simplex virus in febrile neutropenic Feldman BM: Assessment of an infectious disease children undergoing chemotherapy for cancer: a Hawkes M, Barton-Forbes M, Conly J, Nicolle L, history preceding juvenile dermatomyositis symptom prospective cohort study. Pediatric Infectious Barry C, Ford-Jones EL: Superbug at our doorstep. onset. Rheumatology 2008: 47: pp 526-529. Diseases Journal 2007: 26(8): pp 700-704. International 2007: 49: pp 972-977. Canadian Medical Association Journal 2007: 176: pp 54-56. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 99 Waters VJ, Gomez MI, Soong G, Amin S, Ernst R, Salter R, King SM, Smith ML, Bitnun A, Brophy J, Simor AE, Stuart TL, Louie L, Watt C, Ofner-Agostini M, Fernandes-Penney A, Lefebvre A, Louch D, MacDougall Gravel D, Mulvey M, Loeb M, McGeer A, Bryce E, Prince A: Immunostimulatory properties of the G, Moore W, Read SE: Disclosing HIV diagnosis to Matlow A: Canadian Nosocomial Infection Surveillance emerging pathogen Stenotrophomonas maltophilia: Infectious Immunology 2007: 75(4): pp 1698-1703. infected children: a health-care team’s approach. Program. Mupirocin-resistant, methicillin-resistant Vulnerable Children and Youth Studies Journal 2007: Staphylococcus aureus strains in Canadian hospitals. 2: pp 12-16. Antimicrobial Agents Chemotherapy 2007: 51(11): pp BOOKS AND BOOK CHAPTERS 3880-3886. Allen U: Practice point: a pregnant patient with a previous D, Willey BM, Gravel D, Bontovics E, Yaffe B, Katz K: Sincitial (IRIS) Study Groups, Sampalis JS, Langley J, disease. In: Infectious Diseases, 3rd Edition (Armstrong D, Cohen J, eds.). Mosby, London, 2008. Saunders A, Panaro L, McGeer A, Rosenthal A, White pregnancy complicated by group B streptococcal An outbreak of methicillin-resistant staphylococcus Carbonell-Estrany X, Paes B, O’Brien K, Allen U, aureus (MRSA) among healthy newborns and their Mitchell I, Figueras Aloy J, Pedraz C, Michaliszyn AF: mothers. Canadian Journal of Infectious Diseases Development and validation of a risk scoring tool to and Medical Microbiology 2007: 18: pp 128-132. predict respiratory syncytial virus hospitalization in tuberculosis. In: The Canadian Tuberculosis Standards, premature infants born at 33 through 35 completed 6th Edition (Long R, Ellis E, eds.). Health Canada and Schuh S, Lalani A, Allen U, Manson D, Babyn P, weeks gestation. Medical Decision Making 2008: the Canadian Lung Association, Ottawa, 2007. Stephens D, MacPhee S, Mokanski M, Khaikin S, 28(4): pp 471-480. Dick P: Evaluation of the utility of radiography in Kitai IC, Malloy P, Kowalczyk A, Long R: Pediatric Matlow A, Bohnen J: Improving patient safety through acute bronchiolitis. Journal of Pediatrics 2007:150: Tjon J, Lam W, Seto W, Dekker A, Wong C, Atenafu E, pp 429-433. Bitnun A, Waters V, Yau Y, Solomon M, Ratjen F: informatics. In: Pediatric Informatics (Lehmann CU, Pharmacokinetic modelling of once daily dosing Kim GR, Johnson KB, eds.). Springer, New York, 2008. regimen for intravenous tobramycin in paediatric cystic fibrosis patients. Journal of Antimicrobial Chemotherapy 2007: 59: pp 1135-1140. 100 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT standardization of care and beyond: the role of Rosenthal A, Samson L: Bacterial meningitis. A pilot study of genetic determinants of severe Genetic determinants of influenza severity in children: In: Therapeutic Choices, 5th Edition. Canadian influenza in children. Tran D, Paterson A, Beyene J, a pre-pandemic community-based feasibility study. Pharmacists Association, Ottawa, 2007. Schuh S, Richardson S. Dean’s Fund New Staff Grant, Tran D, Beyene J, Bitnun A, Ipp M, Paterson A, University of Toronto ($10,000 2007-2008) Richardson SE. Canadian Institutes of Health FUNDING Research ($99,919 2008-2009) A multicentre, double-blind, randomized, placebo- A study of an HPV VLP vaccine in a cohort of HIV controlled, dose-ranging trial evaluating the safety positive girls and women. Louch D, Bitnun A, Dobson Heart failure and the Coxsackie-Adenovirus Receptor and efficacy of RI-001 (RespImmune/RSV-IVIG) in S, Kollmann T, Ogilvie G, Raboud J, Stuart G, Wobeser (CAR): insights through a novel heart targeted CAR respiratory syncytial virus (RSV) infected patients at W, Cote H, Forbes J, Krajden M, Petric M, Rachlis A, transgenic mouse. Opavsky A. Heart and Stroke risk of severe RSV illness. Allen U and ADMA 001 Coutlee F, Klein M, Loutfy M, Pick N, Smaill F, Foundation of Ontario ($278,151 2007-2010) Study Group (Allen U, PI for international study). Walmsley S. Canadian Institutes of Health Research ADMA Biologics (US$45,000 estimate based on ($646,068 2008-2010) hypertriglyceridemia in HIV-infected children taking $9,000 per patient enrolled - estimated $500,000 2007-2008 internationally) A pharmacokinetic/pharmacodynamic and safety Effect of omega-3 fatty acid supplementation on Defining the role of Stenotrophomonas maltophilia in highly active antiretroviral therapy (HAART): a CF patients. Waters V, Yau Y. Bickell Foundation randomized cross-over study. Read S, Bitnun A, ($64,976 2007) Brophy J. Physicians Services Incorporated Foundation ($32,000 2007-2009) evaluation of oseltamivir for the treatment of children less than 24 months of age with confirmed influenza Defining the role of Stenotrophomonas maltophilia in infection (CASG 114). Allen U (lead at SickKids site). CF patients. Waters V, Yau Y. SickKids Summer Division of Microbiology and Infectious Diseases, Research Program ($2,000 2007) National Institutes of Health (US$42,500 based on five patients enrolled at $8,500 per patient) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 101 Effect of omega-3 fatty acid supplementation on Impact of oximetry on disposition in acute bronchiolitis. Mitochondrial DNA damage in infants exposed to HIV hypertriglyceridemia in HIV-infected children taking Schuh S, Freedman S, Coates A, Parkin P, Allen U, antiretroviral drugs in utero. Cote H, Forbes J, Money highly active antiretroviral therapy (HAART): a Willan A, Ungar W, DaSilva Z. Physicians Services D, Burdge D, Harrigan R, Bitnun A, Read S. Canadian randomized cross-over study. Bitnun A, Read S, Incorporated Foundation ($163,000 2007-2009) Institutes of Health Research ($279,414 2006-2008) Research ($25,000 2007-2009) Impact of oximetry on disposition in acute MRSA special innovation award. Barton-Forbes M bronchiolitis. Coates A, Parkin P, Allen U, Willan A, (Allen U, Bayoumi A, supervisors). Infectious Epstein-Barr virus in health and disease in children Ungar W, Da Silva Z. Thrasher Foundation Diseases Society of America ($20,000 2008) and youth: a prospective study of markers of ($125,000 2007-2010) Brophy J. Canadian Foundation for HIV/AIDS Predicting the risk of infection in children receiving virus-host interaction and genetic variation. Allen U (lead Principal Investigator), Beyene J, Mazzulli T, Measuring the quality of hospital care of children and chemotherapy for acute myeloid leukaemia. Allen U, Robinson J, Read S, Grunebaum E, Zielsenska M, youth in Canada. Guttmann A, Anderson GM, Matlow Beyene J. National Cancer Institute of Canada Tellier R, Weitzman S. Canadian Institutes of Health AG, Parshuram C, To T. Canadian Institute of Health ($817,963, 2008-2012) Research ($397,968 2008-2012) Research/Ministry of Health and Long-Term Care (CIHR $25,000 per year for three years and MOHLTC Immunogenicity of the 7-valent conjugate $100,000 per year for three years 2008-2011) Predicting the risk of infection in children receiving chemotherapy for acute myeloid leukaemia. Sung L, pneumococcal vaccine in HIV-infected children. Bitnun Allen U, Beyene J. Canadian Institutes of Health A, Alllen U, Read S. Physicians Services Incorporated Research ($582,104 2008-2012) Foundation ($16,000 2007-2008) 102 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Prospective cohort study of genetic variation and Use of interferon gamma release assays in children. Randomized double-blind controlled trial of the use of risk of infection in Canadian children with primary Kitai I. The Hospital for Sick Children Paediatric a biofilm antimicrobial susceptibility assay to guide acute myeloid leukaemia. Sung L, Beyene J, Allen U. Consultants Partnership’s Grant ($8,400 2007-2009) antibiotic therapy in chronic Pseudomonas aeruginosa Severe influenza in children: genetic determinants and Ratjen F, Tullis E, Corey M. Innovotech Inc. related epidemiology. Tran D, Beyene J, Paterson A, ($200,000 2008-2011) infected cystic fibrosis patients. Waters V, Yau Y, National Cancer Institute of Canada ($444,424 2005-2008) Retrospective study of caspofungin in organ transplant Richardson, Schuh S. SickKids Foundation recipients. Allen U. Merck Canada ($21,000 2008) ($129,893 2008 2010) What makes an excellent clinician in an academic health sciences centre? Mahant S, Wadhwa A. The RSV infection among transplant recipients and other Hospital for Sick Children Paediatric Consultants high-risk populations. Allen U, Robinson J. Abbott Partnership’s Grant ($8,300 2007) Canada ($236,000 2008-2010) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 103 Dr. Hilary Whyte DIVISION HEAD Dr. Keith Tanswell FULL-TIME Dr. Jacques Belik Dr. Jonathan Hellmann Dr. Dr. Dr. Dr. Dr. Dr. Andrew James Kyong-Soon Lee Patrick McNamara Aideen Moore Christopher Tomlinson Hilary Whyte PART-TIME Dr. Rosemary Moodie ADJUNCT Dr. Xiaoping Luo CROSS-APPOINTEES Dr. Bonnie Stevens SCIENTIFIC STAFF Dr. Virginia Frisk HONORARY FACULTY Dr. Max Perlman Dr.Paul Swyer MOUNT SINAI HOSPITAL Dr. Olufunmi Akindele Dr. Yenge Diambomba Dr. Ann Jefferies Dr. Edmond Kelly Division of Neonatology OVERVIEW The division’s Neonatal-Perinatal Fellowship Training PUBLICATIONS The Division of Neonatology welcomes Dr. Christopher Program continues to train the largest number of Abrahamyan L, Johnson SR, Beyene J, Shah PS, Tomlinson to the faculty at The Hospital for Sick subspecialty trainees in Neonatology of any centre in Feldman BM: Quality of randomized controlled trials in Children and Dr. Maggie Shu to the faculty at Canada, under the leadership of the program director, juvenile idiopathic arthritis. Journal of Rheumatology Mount Sinai Hospital. Dr. Martin Skidmore. 2008: 47: pp 640-645. The three level III NICUs at The Hospital for Sick The division continues to be well supported by major Alfaleh K, Smyth JA, Roberts RS, Solimano A, Asztalos Children, Mount Sinai Hospital and at Women’s funding agencies for its various foci at the three sites, EV, Schmidt B: Prevention and 18-month outcomes of College Hospital continue to suffer from a shortage including lung biology, epidemiology, outcomes-based serious pulmonary hemorrhage in extremely low birth of sufficient level III NICU beds to adequately meet the research and clinical trials. weight infants: results from the trial of indomethacin maternal and neonatal needs of our catchment area, prophylaxis in preterms. Pediatrics 2008: 121(2): which has continued to result in regrettable deferrals HONOURS AND AWARDS of patients out of region, province and country. The Jefferies A: Canadian Association for Medical Ministry of Health and Long-Term Care commissioned Education/Certificate of Merit, 2007. pp e233-e238. a Neonatology Task Force, with province-wide Asztalos E: The need to go beyond: evaluating antenatal corticosteroid trials with long term representation including representatives from the McNamara P: Award for Teaching Excellence 2007- outcomes. Journal of Obstetrics and Gynaecology three Toronto NICUs, to address the issue of current 2008, Neonatal-Perinatal Medicine Training Program, Canada 2007: 29(5): pp 429-432. level III NICU bed requirements across the province, University of Toronto, 2008. and the predicted increase in requirements over the Aziz A, Ohlsson A: Surfactant for pulmonary next 10 to 15 years. This was delivered in 2007, and Ng E: The Jonathan Tolkin Teaching Award, North York hemorrhage in neonates. Cochrane Database an announcement from the Ministry of Health and General Hospital, 2007. Systematic Review 2008: 2: CD005254. Whyte H: Paediatric Emergency Medicine Teaching Azrapoozah A, Lawrence H, Limeback H, Shah PS: Award, part-time staff, June 2008. Xylitol for preventing acute otitis media in children Long-Term Care is expected in June or July, 2008. up to 12 years of age (protocol). Cochrane Database of Systematic Reviews 2008: 2: CD007095. Dr. Karel O’Brien Dr. Arne Ohlsson Dr. Prakesh Shah Dr. Vibhuti Shah Dr. Maggie Shu Dr. Sharon Unger Dr. Kin Fan Young Tai ST. MICHAEL’S HOSPITAL Dr. Tony Barozzino Dr. Douglas Campbell Dr. Michael Sgro SUNNYBROOK HEALTH SCIENCES CENTRE FULL-TIME Dr. Elizabeth Asztalos Dr. Michael Dunn Dr. Robert Jankov Dr. Andrew Shennan Dr. Brian Simmons Dr. Martin Skidmore Dr. Adrian Ziino PART-TIME Dr. Shaheen Doctor Dr. Eugene Ng CLINICAL FELLOWS Dr. Suhair Al-Shanteer Dr. Lucia Aliwalas Dr. Haydi Alwassia Dr. Ijaz Aman Dr. Taiba Balkhair Dr. Rudaina Banihani Dr. Joanne Beamish Dr. Donna Capili Dr. Guo Qiang Cheng Dr. Roxana Conti Dr. Blondel Crosdale Beck J, Brander L, Slutsky AS, Reilly MC, Dunn MS, Berry MA, Shah PS, Brouillette RT, Hellmann J: Finan E, Mak W, Bismilla Z, McNamara PJ: Early Sinderby C: Non-invasive neurally adjusted ventilatory Predictors of mortality and length of stay for neonates discontinuation of intravenous prostaglandin E (1) assist in rabbits with acute lung injury. Intensive Care admitted to children’s hospital neonatal intensive after balloon atrial septostomy is associated with an Medicine 2007: 34: pp 316-323. care units. Journal of Perinatology 2008: 28(4): increased risk of rebound hypoxemia. Journal of pp 297-302. Perinatology 2008: 28(5): pp 341-346. Belik J, Shehnaz D, Pan J, Grasemann H: Developmental changes in arginase expression and Bishara R, Dunn MS, Merko SE, Darling P: Nutrient Fornaro E, Li D, Pan J, Belik J: Prenatal exposure to activity in the lung. American Journal of Physiology - composition of hindmilk produced by mothers of very fluoxetine induces fetal pulmonary hypertension in the Lung Cellular and Molecular Physiology 2008: 294: low birth weight infants born at less than 28 weeks rat. American Journal of Respiratory and Critical Care pp L498-L504. gestation. Journal of Human Lactation 2008: 24(2): Medicine 2007: 176(10): pp 1035-1040. pp 159-167. Gibbins S, Stevens B, Beyene J, Chan PC, Bagg M, Berry MA, Shah PS, Brouillette RT, Hellmann J: Asztalos E: Pain behaviours in extremely low Determinants of mortality and length of stay for El Shahed AI, Dargaville P, Ohlsson A, Soll RF: neonates admitted to children’s hospital neonatal Surfactant for meconium aspiration syndrome in gestational age infants. Early Human Development intensive care units. Journal of Perinatology 2008: full-term/near-term infants. Cochrane Database 2008: 84(7): pp 451-458. 28: pp 297-302. Systematic Review 2007: 3: CD002054. Gibbins S, Stevens B, McGrath P, Yamada J, Beyene J, Camfield C, Finley A, Franck L, Johnston C, O’Brien K, Ohlsson A: Comparison of pain responses in infants of different gestational ages. Neonatology 2007: 93(1): pp 10-18. Dr. Christopher Tomlinson Hamada S, Vearncombe M, McGeer A, Shah PS: Neonatal group B streptococcal disease: incidence, presentation, and mortality. Journal of Maternal, Fetal, and Neonatal Medicine 2008: 2(1): pp 53-57. Dr. Dr. Dr. Dr. Dr. Dr. Walid El-Naggar Manal El Sayed Emer Finan Claudia Garcia Gelvez David Gryn Wenxiang Guo Dr. Dr. Dr. Dr. Dr. Dr. Omer Hamud Doriana Kriguer Ana Teresa Leslie Jillian Lewis Patricia Lewis Linh Ly Dr. Dr. Dr. Dr. Dr. Dr. Pat Mateo Ashok Mittal Adel Mohamed Marcelo Panzenhagen Shilpa Saxena Sandesh Shivananda Dr. Dr. Dr. Dr. Dr. Hanifi Soylu Christopher Tomlinson Connie Williams Man Yi Jennifer Young RESEARCH FELLOWS Dr. Ben Hur Johnson Dr. Mandy Lau Dr. Azhar Masoud ADMINISTRATIVE STAFF Sonia Dos Santos Violeta Dukic Ana Granic Jewel Randolph James AG, Kent A: Spackman. Representation of Jankov RP, Kantores C, Pan J, Belik J: Contribution of Koenen SV, Dunn EA, Kingdom JC, Ohlsson A, Matthews disorders of the newborn infant by SNOMED CT®. xanthine oxidase-derived superoxide to chronic SG: Overexposure to antenatal corticosteroids: a global Studies in Health Technology and Informatics 2008: hypoxic pulmonary hypertension in neonatal rats. concern. Journal of Obstetrics and Gynaecology Canada 136: pp 833-838. American Journal of Physiology - Lung Cellular and 2007: 29(11): p 879. Molecular Physiology 2008: 294: pp L233-L245. Kornecki A, Engelberts D, McNamara PJ, Jankov RP, James AG: Benefits of early childhood interventions last well into adulthood. AboutKidsHealth 2007: Jefferies A, Simmons B, Regehr G: The effect of McCaul C, Ackerly C, Post M, Kavanagh BP: Vascular October 5. www.aboutkidshealth.ca/News/ candidate familiarity on examiner OSCE scores. remodelling alters ventilator-induced lung injury. Benefits-of-early-childhood-interventions-last- Medical Education 2007: 41(9): pp 888-891. Anesthesiology 2008: 108(6): pp 1047-1054. Jefferies A, Simmons B, Tabak D, McIlroy JH, Lee K-S, Lausman AY, Langer JC, Tai M, Seaward PGR, Windrim Roukema H, Skidmore M: Using an objective RC, Kelly EN, Ryan G: Gastroschisis: what is the well-into-adulthood.aspx?articleID=10404& categoryID=news-poh4 James AG: Folic acid supplementation and pre- structured clinical examination (OSCE) to assess average gestational age of spontaneous delivery? eclampsia. AboutKidsHealth 2008: February 14. multiple physician competencies in postgraduate Journal of Pediatric Surgery 2007: 42(11): pp www.aboutkidshealth.ca/News/Folic-acid- training–the CanMEDS roles. Medical Teacher 2007: 1816-1821. supplementation-and-pre-eclampsia.aspx? 29(2-3): pp 183-191. Lee SH, Tomlinson C, Temple M, Amaral J, Connolly articleID=10722&categoryID=news-poh1 Kabra NS, Schmidt B, Roberts RS, Doyle LW, Papile L, BL: Imaging-guided percutaneous needle aspiration or James AG: Folic acid supplementation reduces neural Fanaroff A. Trial of Indomethacin Prophylaxis in catheter drainage of neonatal liver abscesses: 14-year tube defects in Canada. AboutKidsHealth 2007: Preterms Investigators (including Asztalos E): experience. American Journal of Roentgenology 2008: October 26. www.aboutkidshealth.ca/News/Folic- Neurosensory impairment after surgical closure of 190(3): pp 616-622. acid-supplementation-reduces-neural-tube-defects-in- patent ductus arteriosus in extremely low birth weight Canada.aspx?articleID=10428&categoryID=news-poh1 infants: results from the Trial of Indomethacin Lodha A, Furlan AD, Whyte H, Moore AM: Prophylactic Prophylaxis in Preterms. Journal of Pediatrics 2007: antibiotics in the prevention of catheter-associated James AG: Performance and behavioural outcomes 150(3): pp 229-234. bloodstream bacterial infection in preterm neonates: for children with congenital heart conditions. a systematic review. Journal of Perinatology 2008: AboutKidsHealth 2007: July 19. www.aboutkidshealth. April 10 [Epub ahead of print]. ca/News/Performance-and-behavioural-outcomesfor-children-with-congenital-heart-conditions.aspx? articleID=10283&categoryID=news-poh1 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 107 McNamara PJ, Teixiera L, Whyte H, Ly L: The Lodha A, Kamaluddeen MS, Kelly E, Amin H: McDevitt H, Tomlinson C, White MP, Ahmed SF: Clostridium difficile infection precipitating hemolysis in Changes in quantitative ultrasound in infants born hemodynamically significant dustus arterious in glucose-6-phosphate dehydrogenase deficient preterm at less than 32 weeks gestation over the first two critically ill full-term neonates. Fact or fiction? twins causing severe neonatal jaundice. Journal of years of life: influence of clinical and biochemical Biology of the Neonate 2007: 107(1): pp 180-181. Perinatology 2008: 28(1): pp 77-87. changes. Calcified Tissue International 2007: 81(4): pp 263-9. McNamara PJ: Cardiopulmonary resuscitation for A, Helewa M, Hewson S, Kavuma E, Lee SK, Logan AG, McNamara P: Non-conventional use of amplitude 2008: May 26 [Epub ahead of print]. McKay D, Moutquin J-M, Ohlsson A, Rey E, Ross S, integrated electroencephalography (aEEG): lessons from Singer J, Willan AR, Hannah ME: Women’s views of a tertiary NICU. Neonatology Today 2007: 2(1): pp 1-9. neonates: evidence for compressions? Resuscitation Magee LA, von Dadelszen P, Chan S, Gafni A, Gruslin hypertension in pregnancy study) pilot trial. Hypertension in Pregnancy 2007: 26(4): pp 371-387. Morag I, Ohlsson A: Cycled light in the intensive care unit for preterm/low birth weight infants (protocol). their experiences in the CHIPS (control of McNamara PJ, Al-Nabhani D, Finelli M: Cochrane Database Systematic Review 2008:1: Endo-tracheal tube with end-tidal carbon CD006982. dioxide port. Anesthesiology 2008: 108: p 337. Nash A, Secker D, Corey M, Dunn M, O’Connor DL: Malviya M, Ohlsson A, Shah S: Surgical versus medical treatment with cyclooxygenase inhibitors McNamara PJ, Murthy P, Kantores C, Teixeira L, Field testing of the 2006 World Health Organization for symptomatic patent ductus arteriosus in preterm Engelberts D, Van Vliet T, Kavanagh BP, Jankov RP: growth charts from birth to two years: assessment of infants. Cochrane Database Systematic Review Acute vasodilator effect of Rho-kinase inhibitors hospital undernutrition and overnutrition rates and the in neonatal rats with pulmonary hypertension usefulness of BMI. Journal of Parenteral and Enteral unresponsive to inhaled nitric oxide. American Nutrition 2008: 32: pp 145-153. 2008: 1: CD003951. McAuliffe F, Chitayat D, Halliday W, Keating S, Journal of Physiology - Lung Cellular and Molecular Shah V, Fink M, Nevo O, Ryan G, Shannon P, Blaser S: Physiology 2007: 294: pp L205-L213. autopsy findings. Ultrasound Obstetrics & Gynaecology 2008: 31(5): pp 542-548. O’Connor DI, Khan S, Weishun K, Jeffries A, Campbell DM, Asztalos E, Feldman M, Rovet J, Westall C, Whyte H, Rhombencephalosynapsis: prenatal imaging and McNamara PJ, Sehgal AS: Towards rationale Postdischarge Feeding Study Group: Growth and management of the patent ductus arteriosus: the nutrient intakes of human milk-fed preterm infants need for disease staging. Archives of Disease in provided with extra energy and nutrients after hospital Childhood - Fetal and Neonatal Edition 2007: 92(6): discharge. Pediatrics 2008: 121(4): pp 766-776. pp F424-F427. 108 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Ohlsson A, Walia R, Shah S: Ibuprofen for the Shah P, Ohlsson A, Perlman M: Hypothermia to treat Shah PS, Shah N: Heparin bonded catheters for treatment of patent ductus arteriosus in preterm and/ neonatal hypoxic ischemic encephalopathy. Archives prolonging the patency of central venous catheters or low birth weight infants. Cochrane Database of Pediatrics and Adolescent Medicine 2007: 161: in children. Cochrane Database of Systematic Systematic Review 2008: 1: CD003481. pp 951-958. Reviews 2007: 4: CD005983. Padela S, Yi M, Cabacungan J, Shek S, Belcastro R, Shah P, Ohlsson A, Shah J: Continuous negative Shah PS, Shah VS: Continuous heparin infusion Masood A, Jankov RP, Tanswell AK: A critical role for extrathoracic pressure or continuous positive airway to prevent thrombosis and catheter occlusion in fibroblast growth factor-7 during early alveolar pressure for acute hypoxemic respiratory failure to neonates with peripherally placed percutaneous formation in the neonatal rat. Pediatric Research children. Cochrane Database Systematic Review central venous catheters. Cochrane Database 2008: 63: pp 232-238. 2008: 1: CD003699. Systematic Review 2008: 2: CD002772. Perlman M, Shah PS: Time to adopt cooling for Shah P, Ohlsson A: Sildenafil for pulmonary Shah S, Ohlsson A, Halliday H, Shah V: Inhaled versus neonatal HIE: a response to previous commentary. hypertension in neonates. Cochrane Database systemic corticosteroids for the treatment of chronic Pediatrics 2008: 121: pp 616-618. Systematic Review 2007: 3: CD005494. lung disease in ventilated very low birth weight preterm infants. Cochrane Database Systematic Review 2007: 4: CD002057. Schmidt B, Roberts RS, Davis P, Doyle LW, Barrington Shah PS, Aliwalas L, Shah V: Breastfeeding or KJ, Ohlsson A, Solimano A, Tin W, caffeine for apnea breast milk to alleviate procedural pain in neonates: of prematurity trial group (including Asztalos E): a systematic review. Breastfeeding and Medicine Shah V, Hencock R, Shah PS, Ohlsson A, Taddio A: Long-term effects of caffeine therapy for apnea of 2007: 2: pp 74-82. Topical amethocaine gel 4% for intramuscular injection 357: pp 1893-1902. Shah PS, Perlman M, Ohlsson A: Hypothermia to Clinical Therapeutics 2008: 30: pp 166-173. Seghal A, Cook V, Dunn M: Pericardial effusion systematic review. Archives of Pediatrics and Shah V, Ohlsson A, Halliday H, Dunn M: Early associated with an appropriately placed umbilical Adolescent Medicine 2007: 161: pp 951-958. administration of inhaled corticosteroids for in term neonates: a randomized controlled trial. prematurity. New England Journal of Medicine 2007: treat neonatal hypoxic-ischemic encephalopathy: venous catheter. Journal of Perinatology 2007: 27: pp 317-319. preventing chronic lung disease in ventilated very low birth weight preterm neonates. Cochrane Database Systematic Review 2007: 4: CD001969. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 109 Shah V, Ohlsson A: Venepuncture versus heel lance Tessler R, Pan J, Fiori HH, Belik J: Human meconium BOOKS AND BOOK CHAPTERS for blood sampling in term neonates. Cochrane has a pulmonary vascular and airway smooth muscle Asztalos E: Follow-up of the high-risk infant. In Database Systematic Review 2007: 4: CD001452. relaxant effect. Pediatric Research 2008: March 19 Residents Handbook of Neonatology, 3rd Edition [Epub ahead of print]. (Kirpalani HM, Moore A, Perlman M, eds.). BC Decker, Shah V, Shah P: Promising new strategies Hamilton, Ontario, 2007. for bronchopulmonary dysplasia in infants. Tessler R, Wu S, Fiori R, Macgowan CK, Belik J: Pediatric Health 2008: 2(3): pp 315-331. Sildenafil acutely reverses the hypoxic pulmonary Belik, J: Susceptibility of the immature lung to oxidative vasoconstrictor response of the newborn pig. Pediatric and mechanical injury. In: Questions and Controversies Research 2008: April 30 [Epub ahead of print]. in Neonatology Series: Pulmonary Volume (E. Bancalari, Stevens B, Franck L, Gibbins S, McGrath P, Dupuis A, Yamada J, Beyene J, Camfield C, Finley A, Johnston C, ed.). Elsevier, Philadelphia, 2008. O’Brien K, Ohlsson A: Determining the structure of Wu J, Duan R, Cao H, Field D, Newham CM, acute pain responses in vulnerable neonates. Canadian Koehler DR, Zamel N, Pritchard MA, Hertzog P, FUNDING Journal of Nursing Research 2007: 39(2): pp 32-47. Taddio A, Manley J, Potash L, Ipp M, Sgro M, Shah V: Post M, Tanswell AK, Hu J: Regulation of epithelium- Airway modulation of pulmonary vascular tone during specific Ets-like factors Ese-1 and Ese-3 in airway development and following pulmonary hypertension. inflammation. Cell Research 2008: 18: pp 649-663. Belik J. Canadian Institutes of Health Research Routine immunization practices: use of topical Operating Grant ($110,108 2004-2009) anaesthetics and oral analgesics. Pediatrics 2007: Yinon Y, Kelly EN, Ryan G: Fetal pleural effusions. 120: pp e637-e643. Best practice and research. Clinical Obstetrics and Gynaecology 2008: 22(1): pp 77-96. Tallett S, Lingard L, Leslie K, Pirie J, Jefferies L, Spero L, Schneider R, Hilliard R, Rosenfield J, An integrated, shared data management application for the Toronto Tri-Hospital Respiratory Syncyticial Virus Prophylaxis (RSVP) Program. O’Brien K, Bracht Zupancic JAF, Richardson DK, Horbar JD, Carpenter JH, M, Barton P, James AG. Abbott Laboratories Limited ($50,000 2005-2007) Hellmann J, Mian M, Hurley J: Measuring educational Lee SK, Escobar GJ and the Vermont Oxford network workload: a pilot study of paper-based and PDA tools. SNAP pilot project participants: Revalidation of the Medical Teacher 2008: 30(3): pp 296-301. score for neonatal acute physiology in the Vermont Oxford network. Pediatrics 2007: 119: pp 156-163. 110 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT CAP follow up at five years. Schmidt B, Roberts R, Death in Canadian neonatal intensive care units Efficacy and safety of targeting lower arterial oxygen Barrington K, Ohlsson A, Solimano A, Davis P, Doyle L, (NICUs): exploring the ethical determinants. Hellmann saturations to reduce oxygen toxicity and oxidative Tin W. Canadian Institutes of Health Research J, Shah P, Lee SK. Canadian Institutes of Health stress in very preterm infants: Canadian Oxygen Trial ($1,744,740 2005-2009) Research ($99,698 2008-2010) (COT). Schmidt B, Roberts R, Whyte R, Asztalos E, Solimano A, Bairam, Chiu A, Clarke M, Harrold J, Kumar CARESS: Canadian RSV evaluation study of Synagis®. Delayed umbilical cord clamping in preterm infants: M, Moddemann D, Peliowski A, Rabi Y, Roukema H, Mitchell I, Paes B (Allen U, James AG, The Hospital for a randomized controlled trial. Kelly C, Kellie M, Sauve R, Seshia M, Shah PS. Canadian Institutes of Sick Children site investigators). Abbott Laboratories Whittle W, Windrim R, Shah PS. Mount Sinai Health Research ($3,884,860 2006-2011) Ltd. ($120 per patient enrolled 2007-2009) Hospital ($4,000 2008) CIHR Team in Maternal-Infant Care (MICare) grant. Lee Early external cephalic version-2 trial. Hutton, EK, in the regulation of pulmonary vascular tone during SK, Ohlsson A, Shaw N, Liston R, Armson A, Aziz k, Willan AR, Hannah ME, Gafni A, Ohlsson A, Barrett J, development and following pulmonary hypertension. Belik Endothelial eNOS uncoupling and role of BMP-9 Baker R, Saigal S, Skarsgard E, Hayward R, Dendukuri Ross SJ, Windrim R, Delisle MF, Carson GD, Dunn MS, J, Letarte M, Toporsian M, Jerkic M. Heart and Stroke N, O’Campo P, Magee L, Sauve R, Stevens B, Joseph Pollard JK, Natale R. Canadian Institutes of Health Foundation Operating Grant ($86,906 2007-2009) KS, Barrington K, El-Hajj M. Canadian Institutes of Research ($2,853,717 2003-2007) Evaluation of topical liposomal lidocaine and oral Health Research ($4,924,135 2008-2012). Early external cephalic version-2 trial. Hutton EK, sucrose for treatment of pain in newborn infants Conquering pain: the hidden cost of immunization. Taddio Willan AR, Hannah ME, Gafni A, Ohlsson A, Barrett J, undergoing venipuncture: a randomized controlled A, Shah V, Ipp M, Chambers C, Halperin S, Ross SJ, Windrim R, Delisle MF, Carson GD, Dunn MS, trial. Taddio A, Shah V, Katz J, Stevens D, Koren G. Lockett D. The Hospital for Sick Children ($5,000 2008) Pollard JK, Natale R. Canadian Institutes of Health Canadian Institutes of Health Research ($397,450 Research ($169,822 2008-2009) 2007-2009) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 111 External cephalic version-2 trials. Hutton E, Willan A, Hypoglycemia and neuroplasticity in the neonatal Low versus high fidelity simulation in neonatal Hannah M, Gafni A, Ohlsson A, Barrett J, Ross S, period. Shroff M, Moore A, Whyte H, Taylor M. The resuscitation training. Finan E, Bismilla Z, Whyte HE, Windrim R, Delisle M-F, Carson GD, Dunn M, Pollard Department of Medical Imaging at University of McNamara P, LeBlanc V. The Hospital for Sick JK, Natale R, Sylvestre G. Canadian Institutes of Toronto ($35,000 per year 2008-2010) Children Paediatric Consultants Partnership’s Education Research Grants ($5,000 2008-2009) Health Research ($169,822 2008-2009) Infant flow biphasic NCPAP versus infant flow NCPAP Functional MR imaging for early detection and for facilitation of extubation in infants less than Multicentre randomized trial of HeLP (heat loss physiologic characterization of inflammatory arthritis 1,250 grams: a randomized controlled trial. prevention) in the delivery room. Vohra S, Reilly MC, in a rabbit model. Doria SA, Belik J, Kassner A, Cheng O’Brien K, Shah V, Campbell C. Physicians Services Dunn M, Vincer M, Wiebe N, Zayack D, Wimmer J. H-L, Crawley A, Yeung R, Tomlinson G, Gahunia H. Incorporated Foundation ($73,000 2006-2008) 2006-2007) Canadian Institutes of Health Research ($640,465 2004-2008) RSNA Research and Education Foundation ($29,964 Inhibition of apoptosis as a major component of chronic neonatal lung injury. Tanswell AK, Jankov RP. Multiple courses of antenatal corticosteroids for Genome-environment interactions in Type I diabetes. Canadian Institutes of Heath Research ($477,180 preterm birth (MAC). Murphy K, Asztalos E. Canadian Danska J, MacPherson A, Moore AM. Genome Canada 2005-2010) Institutes of Health Research ($2,943,583 2006-2010) ($94,127 2005-2009) INTAPP (International trial of antioxidants for the Multiple courses of antenatal corticosteroids for Group in lung development. Tanswell AK, Belik J, prevention of pre-eclampsia). Fraser W, Xiong X, preterm birth study: a five-year follow-up (MACS-5). Chan A, O’Brodovich H, Post M, Rotin D, Cutz E, Julien P, Thomas N, Magee LA, Ohlsson A. Canadian Asztalos E, Gafni A, Hannah M, Murphy K, Ohlsson A, Hu J, Kavanagh B, Otulakowski G, Yeger H. Canadian Institutes of Health Research ($4,923,447 2002- Ross S, Saigal S, Willan A, Matthews S, Rovet J, Institutes of Health Research ($1,377,695 2004-2009). 2007) Lee S, Amankwah K, Kelly EN, Lee SK, Delisle M-F, Guselle P, Sananes R. Canadian Institutes of Health Research ($3,585,161 2005-2012). 112 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Nasal ventilation in premature infants. Kirpalani H, Novel therapeutic interventions for bronchopulmonary Quality of care ICE (QC-ICE) team in neonatal-perinatal Chiu A, Lemyre B, Millar D, Roberts R, Vaillancourt K, dysplasia: Targeting oxidants and growth factors in the care. Lee KS, Baker GR, Langley JM, MacNab YC, Alvaro R, Connelly R, Howlett A, McNamara P, newborn rat lung. Jankov RP. Canada Foundation for Moehr JR, Ohlsson A, Stevens B, Magee L, Sauve R, Peliowski A, Solimano A, Shah P, Aziz K, Zupancic J, Innovation New Opportunities Fund/Ontario Keller P, Michalos A, Klassen A. Canadian Institutes Meade M. Canadian Institutes of Health Research Research Fund ($297,340 2004-2009) of Health Research ($935,000 2003-2007) Observational study on the effects of enteral feeding Randomized study of vasopressin versus epinephrine ($2,233,100 2006-2009) National health partnership for reducing infections in and feeding methods on respiratory pattern as in a neonatal model of asphyxial cardiac arrest. NICU. Lee SK, Aziz K, Baker GR, Barrington K, Clarke assessed by diaphragm electrical activity (EAdi) in McNamara PJ, Kavanagh BP. American Academy of MCW, Cronin CM, Dunn MS, James AG, Langley J, Lee very low birth weight (VLBW) preterm infants. Ng E, Pediatrics, Neonatal Resuscitation Grant ($28,752 DS, Lefebvre F, Merchant P, Ohlsson A, Orrbine E, Schurr P, Reilly M, Dunn M, Beck J. Physician Services 2006-2008) Peliowski A, Sankaran K, Seshia M, Shaw NT, Synnes Incorporated Foundation ($26,000 2008-2009) Reactive nitrogen species and smooth muscle growth AR, Tan-Dy CR, Walker CR, Whyte RK. Canadian Institutes of Health Research Canadian Neonatal Pharmacology of milrinone in persistent pulmonary regulators in the pathogenesis of newborn pulmonary Network ($99,999 2005-2008) hypertension of the newborn. McNamara P, Taddio A. hypertension. Jankov RP. Career Development Award, The Hospital for Sick Children New Ideas Grant Canadian Child Health Clinician Scientist Program/ Neuroimaging measures of brain function in preterm ($39,349 2006-2008) Canadian Institutes of Health Research ($280,000 2004-2008) neonates. Taylor M, Whyte H, Moore A, McNamara P, Donner E, Sharoff M. Collaborative Health Research Predicting neurobehaviourial outcome in preterm Program ($361,860 2008-2011) neonates. Taylor M, Whyte HE, Moore A, Shroff M, Regulation of normal and oxygen-mediated aberrant Rayboud C, Donner E, Frisk V. Canadian Institutes of postnatal lung growth by growth factors. Tanswell AK. Health Research ($929,910 2007-2012) Canadian Institutes of Health Research ($799,686 2005-2010) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 113 Rho-kinase inhibitor as therapy for chronic neonatal Strategic training initiative in research in reproductive Systematic reviews of determinants and effectiveness pulmonary hypertension. Jankov RP. Canadian health sciences. Fraser WD, Campbell M, Collet J, of primary preventive interventions for low birth weight Institutes of Health Research New Investigator Davidge S, De Koninck M, Dodds L, Dodin S, Fisher W, and preterm birth. Shah P, Ohlsson A, Shah V, Murphy Award ($300,000 2008-2013) Rho-kinase inhibitor as therapy for chronic neonatal Gagnon R, Grant A, Hatem-Asmar M, Heaman M, K, Hutton E, Beyene J, Frick C, Allen V, McDonald S, Joseph K, Klein M, Labrecque M, Lambert R, Lee S, Newburn-Cook C, Scott F. Canadian Institutes of Letourneau L, Leung P, Lye S, McCoshen J, Moutquin J, Health Research ($100,000 2008) pulmonary hypertension. Jankov RP, McNamara PJ. Nisker J, Ohlsson A, Pierson R, Sauve R, Smith G, Von Canadian Institutes of Health Research Operating Dadelszen P, Walker M, Wood S. Canadian Institutes Teaching neonatal intubations to residents: standard Grant ($323,592 2007-2010) of Health Research ($1,800,000 2002-2008) mannequin versus high fidelity simulation. Finan E, Role of peroxynitrite and macrophages in newborn Surveillance of kernicterus in Canada. Sgro M, pulmonary vascular remodelling. Jankov RP. Canadian Campbell D, Shah V. Canadian Medical Protection Institutes of Health Research ($285,888 2005-2008) Association ($44,660 2007-2008) Role of reactive oxygen/nitrogen species in normal Surveillance of Kernicterus in Canada. Sgro M, Institutes of Health Research Group Grant and aberrant postnatal lung growth. Tanswell AK. Campbell D, Shah V. Ontario Federation for ($1,285,443 2004-2009) Canadian Institutes of Health Research ($708,400 Cerebral Palsy ($25,100 2007-2008) Bismilla Z, Whyte HE, V LeBlanc. Physicians Services Incorporated Foundation ($17,000 2007-2008) The developing lung: cellular and molecular physiology. Tanswell AK. Medical Research Council/Canadian The twin birth study: a multicentre randomized 2004-2009) Survey of mode of delivery and maternal and perinatal controlled trial comparing planned caesarean section Safety and efficacy of methylxanthines in the very outcomes in Canada. Lee S, Armson A, Allen V, with planned vaginal birth for twins at 32 to 38 weeks low birth weight infant: five-year follow-up. Schmidt B, Andruschuk J, Attenborough R, Bottomley J, DaSilva O, gestation. Barrett J, Hannah M, Willan A, Ross S, Asztalos E. Canadian Institutes of Health Research Frick C, Heaman M, Murphy P, Pasquier J-C, Bujold E, Hutton E, Allen A, Joseph KS, Armson A, Farrell S, ($1,744,740 2004-2009). Crane J, Dumont A, Janssen P, Klein M, Liu S, Ohlsson A, Asztalos E, Gafni A, Okun N, Leduc L. Moutquin J-M, O’Brien B, Ohlsson A, Platt R, Sauve R, Canadian Institutes of Health Research ($8,608,045 von Dadelszen P, Walker M. Canadian Institutes of 2003-2011) Health Research/World Health Organization ($250,000 2007-2009) 114 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Thyroid hormone insufficiencies and visual perception Utility of modern ultrasound in the evaluation of What is it like for NICU nurses to care for newborns at deficits in preterm infants. Rovet J, Asztalos E, Westall cerebral venous sinuses in neonates. Miller E, Shroff the end of their lives? Lindsay G, Ives-Baine LA, Cross C, Heon E. March of Dimes ($253,083 2004-2007) M, Moore AM. Physicians Services Incorporated N, Hellmann J. University of Ontario Institute of Foundation ($35,500 2007-2009). Technology ($17,850 2007-2008) Asztalos E, Gibbins S, McKeever P, Pillai-Riddell A. Vasopressin in paediatric shock. Choong K, Bohn D, Withdrawal of artificial hydration and nutrition in Canadian Institutes of Health Research ($77,921 Joffe A, Menum K, McNamara P, Litalien C. Canadian newborn infants: determining neonatal nurses’ 2005-2008) Institutes of Health Research ($136,792 2006-2007) perspectives on the ethical and practical issues in Understanding chronic pain in infancy. Stevens B, this practice. Hellmann J, Ives-Baine L, Golec L, Lindsay G, Lee KS, Stephens D, Hetherington R. The Using telemedicine strategies to prevent avoidable Vasopressin in paediatric shock. Choong K, Bohn D, blindness in retinopathy of prematurity: reliability and Joffe A, Menum K, McNamara P, Litalien C. Heart and Hospital for Sick Children Paediatric Consultants measures of pain. Tehrani N, Kertes P, Gibbins S, Stroke Foundation of Canada ($61,623 2006-2007) Partnership’s Grant for Creative Professional Activity James A. EA Baker Foundation of the National ($10,000 2008-2009) Institute of the Blind ($40,000 2006-2008) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 115 Division of Dr. Tino Piscione DIVISION HEAD Dr. Denis F. Geary FULL-TIME Dr. Elizabeth A. Harvey Dr. Diane Hébert Dr. Dr. Dr. Dr. Dr. Valerie Langlois Christoph Licht Tino D. Piscione Lisa A. Robinson Norman D. Rosenblum PART-TIME Dr. Marion Cummings Dr. Rachel Pearl ADJUNCT Dr. Leo Levin HONORARY Dr. Gerald S. Arbus CLINICAL FELLOWS Dr. Thoraya Al Maslamani Dr. Sudarsana De Dr. Meeta Mallik Dr. Rola Saqan SUBSPECIALTY RESIDENTS Dr. Naifain Al-Kalbani Dr. Dana Al-Nabhani Dr. Elizabeth Berger Dr. Mathieu Lemaire Dr. Seetha Radhakrishnan Dr. Lindsay Teskey Division of Nephrology OVERVIEW Dr. Christoph Licht continued to expand his reputation Rosenblum, ND: Canada Research Chair in This has been a very successful year for the as a leader in the field of complement related renal Developmental Nephrology (Tier I), 2005-present. Nephrology Division. disorders, and was appointed as a member of two international working groups within this field. He Rosenblum ND: Selected as Best Doctors The clinical work has remained intense, with ever published a description of a new subgroup of Atypical Physician, 2007. increasing patient complexity and volumes. Hemolytic Uremic Syndrome. The division has been ably represented by Drs. In recognition of her expertise as an educator, Akikusa JD, Schneider R, Harvey EA, Hebert D, Hebert, Robinson and Langlois in the development Dr. Elizabeth Harvey was appointed an examiner Thorner PS, Laxer RM, Silverman ED: Clinical features of the SickKids Transplant Centre, and integration for the Royal College of Physicians of Canada. Also, and outcome of pediatric Wegener’s granulomatosis. of Nephrology within that administrative structure. within the educational arena, Dr. Aoife Waters was Arthritis and Rheumatism 2007: 57: pp 837-844. Dr. Piscione, together with the leadership of the chosen to represent the Department of Paediatrics Molecular Genetics laboratory, was instrumental in the at the National Paediatric Residents and Fellows Bridgewater D, Cox B, Cain J, Lau A, Althaide V, development of clinically approved testing for genetic Research Competition. Gill P, Kuure S, Sainio K, Rosenblum ND. Canonical PUBLICATIONS abnormalities in children with Nephrotic Syndrome. WNT/b-catenin signalling is required for ureteric Dr. Norman Rosenblum served as Associate Editor branching. Developmental Biology 2008: 317(1): The three scientists in the division, Drs. Norman (Renal and Urinary Tract Section) for Rudolph’s Textbook pp 83-94. Rosenblum, Lisa Robinson and Tino Piscione, have of Paediatrics and Dr. Denis Geary edited the first all been successful in their applications for research edition of Comprehensive Pediatric Nephrology. Cain JE, Hartwig S, Bertram JF, Rosenblum ND. manuscripts in highly rated journals. Dr. Robinson’s HONOURS AND AWARDS and future. Differentiation 2008: March 7 role in the development of the Kids Science program Licht C: Appointed Member of the European [Epub ahead of print]. was recognized by a special Synapse Award from Paediatric Research Group for HUS, 2007. grant funding, and have published a variety of BMP signalling in the developing kidney: present CIHR, and Dr. Rosenblum was named Director of the Collins K, Roberts E, Adeli K, Bohn D, Harvey E: Clinician Scientist Training Programs in the Faculty of Licht C: Appointed Associate Member of the Single Pass Albumin Dialysis (SPAD) in fulminant Medicine at U of T. Dr. Robinson was also promoted to European Working Party for the Genetics of wilsonian liver failure: a case report. Pediatric Associate Professor in the Department of Paediatrics. Complement Based Diseases, 2007. Nephrology 2008: February 26 [Epub ahead of print]. RESEARCH FELLOW Dr. Aoife Waters ADMINISTRATIVE STAFF Dana Debernyj Juliet Rodrigo Bernard Singh Marisa Tuzi Tania Viseu POST-DOCTORAL FELLOWS Dr. Darren Bridgewater Dr. Jason Cain Dr. Lijun Chi Dr. Tuncer Onay CLINICAL RESEARCH ASSOCIATES Michelle Frieling Jun Chuan Teh Durkan A, Alexander RT, Liu GY, Rui M, Femia G, Hartwig S, Bridgewater D, Cain J, DiGiovanni V, Licht C, Schlötzer-Schrehardt U, Kirschfink M, Robinson LA: Expression and targeting of CX3CL1 Mishina Y, Rosenblum ND. The BMP receptor ALK3 Zipfel PF, Hoppe B: MPGN II–genetically determined by (Fractalkine) in renal tubular epithelial cells. Journal of controls collecting system formation during murine defective complement regulation? Pediatric kidney development. Journal of the American Nephrology 2007: 22(1): pp 2-9. the American Society of Nephrology 2007: 18: pp 74-83. Society of Nephrology 2008: 19: pp. 117-124. O’Brodovich H, Beyene J, Tallett S, MacGregor D, Geary DF, Hothi D, Harvey EA, Goia C: Evaluating methods for improving ultrafiltration in pediatric Hiraki LT, Benseler SM, Tyrell PN, Hebert D, Harvey E, Rosenblum ND: Performance of a career development hemodialysis. Pediatric Nephrology 2008: 23(4): Silverman ED: Clinical and laboratory characteristics and compensation program at an academic health pp 631-638. and long-term outcome of pediatric systemic lupus sciences centre. Pediatrics 2007: 119: pp e791-e797. erythematosis: a longitudinal study. Journal of Geary DF: Hemolytic uremic syndrome and streptococcus Pediatrics 2008: 152(4): pp 550-556. Pediatrics 2007: 151(2): pp 113-114. Gerstung M, Roth T, Dienes H-P, Licht C, Fries JWU: Józsi M, Licht C, Strobel S, Zipfel SLH, Richter H, 2006 Annual Report of the North American Pediatric Heinen S, Zipfel PF, Skerka C: Factor H autoantibodies Renal Trials and Collaborative Studies (NAPRTCS). in atypical hemolytic uremic syndrome correlate Pediatric Transplantation 2007: 11: pp 366-373. Endothelin-1 induces NF-B via two independent with CFHR1/CFHR3 deficiency. Blood 2008: 111(3): pathways in human renal tubular epithelial cells. pp 1512-1514. Smith RJH, Alexander J, Barlow PN, Botto M, Cassavant TL, Cook HT, de Córdoba SR, Hageman GS, American Journal of Nephrology 2007: 27(3): pp 294-300. Smith JM, Stablein DM, Munoz R, Hebert D, McDonald RA: Contributions of the Transplant Registry: the pneumoniae: improving our understanding. Journal of Koshy S, Geary DF: Anemia in children with chronic Jokiranta TS, Kimberling WJ, Lambris JD, Lanning LD, kidney disease. Pediatric Nephrology 2008: 23(2): Levidiotis V, Licht C, Lutz HU, Meri S, Pickering MC, pp 209-219. Quigg RJ, Rops AL, Salant DJ, Sethi S, Thurman JM, Tully HF, Tully SP, van der Vlag J, Walker PD, Würzner R, Zipfel PF. Dense Deposit Disease Focus Group: new approaches to the treatment of Dense Deposit Disease. Journal of the American Society of Nephrology 2007: 18(9): pp 2447-2456. 118 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Trapp M, Galtseva A, Manning D, Beier D, BOOKS AND BOOK CHAPTERS Langlois V: Laboratory evaluation at different ages. Rosenblum ND, Quarmby L: Defects in ciliary Cochat P, Hebert D: Demographics of pediatric In: Comprehensive Pediatric Nephrology (Geary DG, localization of Nek8 is associated with cystogenesis. renal transplantation. In: Comprehensive Pediatric Schaefer F, eds.). Elsevier Inc., Philadelphia, 2008. Pediatric Nephrology 2008: 23: pp 377-387. Nephrology (Geary DF, Schaefer F, eds.). Elsevier Inc., Licht C, Mengel M: Membranoproliferative Philadelphia, 2008. glomerulonephritis. In: Comprehensive Pediatric Valentini RP, Geary DF, Chand D: Central venous lines for chronic hemodialysis: survey of the Durkan AM, Robinson LA: Acute allograft dysfunction. Nephrology (Geary DF, Schaefer F, eds.). Elsevier Inc., Midwest Pediatric Nephrology Consortium. In: Comprehensive Pediatric Nephrology (Geary DG, Philadelphia, 2008. Pediatric Nephrology 2008: 23(2): p 291. Schaefer F, eds.). Elsevier Inc., Philadelphia, 2008. Waters A, Langlois V, Thorner P, Geary D: Geary DG, Schaefer F: Comprehensive Pediatric development of the kidney: anatomic development of Atypical p-ANCA is not a poor prognostic marker Nephrology (Geary DF, Schaefer F, eds.). Elsevier Inc., the kidney. In: Comprehensive Pediatric Nephrology in postinfectious glomerulonephritis. Pediatric Philadelphia, 2008. (Geary DG, Schaefer F, eds.). Elsevier Inc., Piscione TD, Waters AM: Structural and functional Philadelphia, 2008. Nephrology 2007: 22(9): pp 1383-1386. Hothi D, Geary DF: Pediatric hemodialysis prescription, Rosenblum ND: Developmental biology of the human Waters AM, Wu MYJ, Onay T, Scutaru J, Liu J, Lobe CG, efficacy and outcome. In: Comprehensive Pediatric Quaggin SE, Piscione TD: Ectopic notch activation in Nephrology (Geary DF, Schaefer F, eds.). Elsevier Inc., kidney. In: Seminars in Fetal and Neonatal Medicine developing podocytes causes glomerulosclerosis in Philadelphia, 2008. (Woolf A, Chitty L, eds.). Elsevier, Philadelphia, 2007. transgenic mice. Journal of the American Society of Nephrology 2008: 19(6): pp 1139-1157. Hothi DK, Harvey EA: Anticoagulation in children Rosenblum ND, Salomon R: Disorders of kidney on hemodialysis. In: Handbook of Dialysis Therapy, formation. In: Comprehensive Pediatric Nephrology 4th Edition (Nissenson A, Fine R N, eds.). Elsevier Inc., (Geary DG, Schaefer F, eds.). Elsevier Inc., Philadelphia, 2008. Philadelphia, 2008. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 119 Schroder C, Geary DF: Initiation and choice of dialysis Canadian Paediatric Clinician Scientist Program. Control of branching morphogenesis in the normal and modality. In: Comprehensive Pediatric Nephrology Rosenblum ND. SickKids Foundation ($300,000 per dysplastic kidney. Rosenblum ND. Canadian Institutes (Geary DG, Schaefer F, eds.). Elsevier Inc., year 2002-2014) of Health Research ($147,000 per year 2003-2008) Philadelphia, 2008. Canadian Paediatric Clinician Scientist Program. Control of kidney morphogenesis by sonic hedgehog Waters AM, Piscione TD: Nephrologic disorders. Rosenblum ND. B.C. Research Institute for Children’s and its signalling effectors. Rosenblum ND. Canadian In: Residents’ Handbook of Neonatology, 3rd Edition, and Women’s Health ($150,000 per year 2002-2008) (Kirpalani H, Moore AM, Perlman M, eds.). BC Decker, Institutes of Health Research ($134,419 per year 2006-2009) Inc., Hamilton, Ontario, 2007. Cellular regulation of fractalkine. Lisa Robinson. FUNDING ($171,944 2005-2007) Heart and Stroke Foundation of Ontario, Grant-in-Aid Canadian Child Health Clinician-Scientist Program. Control of murine kidney development by beta0catenin. Rosenblum ND. The Kidney Foundation of Canada ($50,000 per year 2006-2008) Rosenblum ND. Canadian Institutes of Health Clinical genetic test development for focal segmental Research ($300,000 per year 2002-2008) glomerulosclerosis. Piscione TD. National Institutes Epigenetic changes in urothelium following E. coli of Health Office of Rare Diseases ($52,000 2007) infection–new keys to understanding urinary tract Canada Research Chair in Developmental Nephrology infection susceptibility. Rosenblum ND. The Hospital (Tier I). Rosenblum ND. Canada Research Chairs Clinical practice innovation. Geary DF. Program ($200,000 per year 2005-2012) The Hospital for Sick Children Paediatric Consultants Partnership ($10,000 2007) 120 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT for Sick Children ($50,000 2007-2008) Evaluation of photograph CQI instrument. The role of notch signalling in focal segmental Geary DF. Genzyme Inc. ($11,500 2007) glomerulosclerosis. Piscione TD. The Hospital for Sick Children Research Institute ($100,000 2007) Kids Science. Robinson R. Ministry of Research and Innovation, Youth Science and Technology The role of notch signalling in focal segmental Outreach Program ($150,000 2006-2009) glomerulosclerosis. Piscione TD. The Hospital for Sick Children Research Institute ($151,500 2006-2008) Regulation of the membrane-anchored chemokine, fractalkine, by Thromboxane A2. Robinson L. The role of slit/robo signalling in kidney inflammation. Heart and Stroke Foundation of Ontario Robinson R. The Kidney Foundation of Canada ($268,452 2007-2010) ($100,000 2007-2009) The role of notch signalling in focal segmental The role of slit/robo signalling in renal inflammation. glomerulosclerosis. Piscione TD. The American Robinson L. Canada Research Chair, Tier 2 Society of Nephrology ($200,000 2007-2009) ($500,000 2004-2009) The role of slit/robo signalling pathways in kidney inflammation. Robinson L. Early Researcher Award, Government of Ontario ($100,000 2006-2008) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 121 Division of Dr. Gabrielle deVeber DIVISION HEAD Dr. O. Carter Snead III FULL-TIME Dr. Rand Askalan Dr. Brenda Banwell Dr. Gabrielle deVeber Dr. Elizabeth Donner Dr. Cecil Hahn Dr. Daune MacGregor Dr. Berge Minassian Dr. Teesta Soman Dr. Ingrid Tein Dr. Jiri Vajsar Dr. Shelly Weiss PART-TIME Dr. Danielle Andrade Dr. David Berbrayer Dr. Pamela Cooper Dr. Jonathan Gladstone Dr. Jeffrey Kobayashi Dr. Betty Koo THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Dr. Dr. Dr. Dr. Dr. Wayne Langburt Shanna Lindsay William Logan Gilbert Miller Peter Rumney HONORARY Dr. John Lovering Dr. Douglas McGreal Dr. E. Gordon Murphy Dr. Diann Superina-Bell SCIENTIFIC STAFF Dr. Miguel Cortez Dr. Maureen Lovett Dr. James MacFarlane Dr. Hiroshi Otsubo Dr. Elizabeth Pang Dr. Jose Luis Perez Velazquez Division of Neurology OVERVIEW Chan D: William J. Logan Award for Excellence Andrade DM, Minassian BA: Genetics of epilepsies. The major foci of the clinical activities of the Division in Clinical Teaching, Division of Neurology, Expert Review of Neurotherapies 2007: 7(6): pp 727-734. of Neurology are epilepsy, stroke, headache, The Hospital for Sick Children, 2008. neurometabolic disease, neuromuscular disease, Andrade DM, Turnbull J, Minassian BA: Lafora neurogenetics, sleep disorders, movement disorders, Elbers J: Prichard Day Prize Winner, Small vessel CNS disease, seizures and sugars. Acta Myologica demyelinating diseases, functional neuroimaging vasculitis: clinical and pathological correlation, 2008. 2007: 26(1): pp 83-86. Gladstone J: Best Doctors in Canada, Andrade DM, Carlen PL: Novel anti-seizure drug Best Doctors, Inc., 2007-2008. mechanisms. Future Neurology 2007: 2(1): pp 73-86. as postdoctoral training programs in epilepsy, stroke, Minassian B: Canadian Paediatric Society Banwell B: Through the eyes of a child: research neurometabolic disease and demyelinating diseases. sanofi pasteur Research Award, 2008. insights gained through the study of childhood multiple and neurointensive care. The Division of Neurology has a Royal College accredited training program in Child Neurology, as well sclerosis. Multiple Sclerosis 2008: 14(1): pp 4-5. In addition, there are a number of graduate students and postdoctoral fellows in clinical neurophysiology, Ochi A: William A. Hawke Award for Excellence in neurogenetics, molecular and cellular neurobiology. Clinical Teaching, Division of Neurology, The Hospital Banwell B: Editorial. The long (itudinally extensive) for Sick Children, 2008. and the short of it: transverse in children. Neurology 2007: 68(18): pp 1447-1449. The research foci of the Division of Neurology is epilepsy (both clinical and basic research), Taylor M: Annual Josephine Mills Research Award magnetoencephalography, stroke, dyslexia, burden for Excellence in MEG Research in Developmental of disease and quality of life, muscular dystrophy, Neuroscience, 2008. Becker DJ, Dosch HM: Abnormal T cell reactivities in multiple sclerosis, fatty acid oxidation disorders and molecular and cellular neurobiology. Banwell B, Bar-Or A, Cheung R, Kennedy J, Krupp LB, childhood inflammatory demyelinating disease and Type PUBLICATIONS 1 diabetes. Annals of Neurology 2008: 63(1): pp 98-111. Amin R, Ford-Jones L, Richardson SE, MacGregor D, HONOURS AND AWARDS Tellier R, Heurter H, Fearon M, Bitnun A: Acute Banwell B, Ghezzi A, Bar-Or A, Mikaeloff AY, Tardieu M: Akiyama T: Prichard Day Prize Winner, Movie of ictal childhood encephalitis and encephalopathy Multiple sclerosis in children: invited review. high-frequency oscillations combined with intracranial associated with influenza: a prospective 11-year Lancet Neurology 2007: 6: pp 887-902. video EEG in Jacksonian seizures, 2008. review. Pediatric Infectious Disease Journal 2008: April 4 [Epub ahead of print]. Dr. Mary Lou Smith Dr. Margot Taylor Dr. Robyn Westmacott SUBSPECIALTY RESIDENTS Dr. Reem Al-Khater Dr. Cyrus Boelman Dr. John Brandsema Dr. Ryan Hung Dr. Naila Makhani Dr. Jorina Elbers Dr. Emily Tam DEPARTMENTAL FELLOWS Dr Juan Pablo Appendino Dr. Klaus Werner Dr. Zarazuela Zolkipli EPILEPSY FELLOWS Dr. Tomoyuki Akiyama Dr. Derrick Chan Dr. Cristina Go Dr. Zulma Tovar-Spinoza RESEARCH FELLOWS Dr. Ayataka Fujimoto Dr. Ichiro Sugiyama STROKE FELLOWS Dr. Lori Billinghurst Dr. Marilyn Tan ADMINISTRATIVE STAFF Cecilia Amorim Erin Crawford Maureen Hodge Fatima Ledo Michelle Lo Marilyn McLaughlin Susy O’Neill Wendy Ricketts Barbara Zimnowodzki The Hospital for Sick Children 2007-08 ANNUAL REPORT 123 Banwell B, Krupp L, Kennedy J, Tellier R, Tenembaum Barbeau EJ, Taylor MJ, Regis J, Marquis P, Chauvel P, Bismilla Z, Sell E, Donner EJ: Hashimoto’s S, Ness J, Belman A, Boiko A, Bykova O, Waubant E, Liégeois-Chauvel C: Spatio-temporal dynamics of face encephalopathy responding to Risperidone. Journal Mah J, Stoian C, Kremenchutzky M, Bardini MR, recognition. Cerebral Cortex 2008: 18(5): pp 997-1009. of Child Neurology 2007: 22(7): pp 855-857. Yeh EA, Farrell K, Freedman M, Iivanainen M, Sevon M, Becker WJ, Gladstone JP, Aube M: Migraine prevalence, Cheyne D, Bostan AC, Gaetz W, Pang EW: Bhan V, Dilenge ME, Stephens D, Bar-Or A: Clinical diagnosis and disability. Canadian Journal of Event-related beamforming: a robust method for features and viral serologies in paediatric multiple Neurological Sciences 2007: 34(4): S3-S9. presurgical functional mapping using MEG. Clinical Ruggieri M, Rensel M, Hahn J, Weinstock-Guttman B, Neurophysiology 2007: 118: pp 1691-1704. sclerosis: A multinational observational study. Lancet Neurology 2007: 6: pp 773-781. Bercovici E, Pang EW, Sharma R, Mohamed IS, Banwell B, Sled J: Invited editorial. Starting early: S, Chuang SH, Kemp SM, Rutka JT, Snead OC III, Holowka S, Rutka J, Weiss S, Chuang S, Snead OC III, MRI evidence of gray matter atrophy in children with Otsubo H: Somatosensory evoked fields on Otsubo H: Extra-operative somatosensory evoked multiple sclerosis. Neurology 2008: 70: pp 1065-1066. magnetoencephalography for epilepsy infants younger potentials confirm the accuracy of MEG SEF Imai K, Fujimoto A, Ochi A, Viljoen A, Chu B, Holowka Chu B, Pang EW, Ochi A, Sharma R, Hunjan A, than four years with total intravenous anaesthesia. localizations. International Congress Series 2007: Clinical Neurophysiology 2008: 119: pp: 1328-1334. 1300: pp 637-640. Pittock SJ: Neuromyelitis optica-IgG in childhood Bernard T, deVeber G, Benke T: Athletic participation Clement EM, Godfrey C, Tan J, Brockington M, Torelli S, inflammatory demyelinating CNS disorders. after acute ischemic childhood stroke: a survey of Feng L, Brown SC, Jimenez-Mallebrera C, Sewry CA, paediatric stroke experts. Journal of Child Neurology Longman C, Mein R, Abbs S, Vajsar J, Schachter H, Banwell B, Tenembaum S, Lennon VA, Ursell E, Kennedy J, Bar-Or A, Weinshenker BG, Lucchinetti CF, Neurology 2008: 70(5): pp 344-352. 2007: 22(8): pp 1050-1053. Muntoni F: Mild POMGnT1 mutations underlie a novel limb-girdle muscular dystrophy variant. Archives of Neurology 2008: 65(1): pp 137-141. Datta AN, Hahn CD, Sahin M: Clinical presentation Dr. Jiri Vajsar and team and diagnosis of tuberous sclerosis complex in infancy. Journal of Child Neurology 2008: 23(3): pp 268-273. 124 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Jackson A, Khademian Z, Moon R, Berger E, Kirton A, Elliott IM, Lach L, Kadis DS, Smith ML: Psychosocial Hamani C, Hodaie M, Chiang J, del Campo M, outcomes in children two years after epilepsy surgery: Andrade DM, Sherman D, Mirski M, Mello L, Lozano A: deVeber G, Chandran L, During A, Balsam D: Index has anything changed? Epilepsia 2008: 49(4): Deep brain stimulation in the anterior nucleus of of suspicion. Pediatrics in Review 2007: 28(8): the thalamus: effects of electrical stimulation on pp 305-311. pp 634-641. pilocarpine-induced seizures and status epilepticus. Epilepsy Research 2008: 78(2-3): pp 117-123. Katzir T, Kim YS, Wolf M, Morris RD, Lovett MW: The of epileptiform activity. International Journal of Herdman AT, Pang EW, Ressel V, Gaetz W, Cheyne D: subtypes of children with dyslexia at letter, word and Bifurcation and Chaos 2008: 18(2): pp 429-439. Task-related modulation of early cortical responses connected text levels of reading. Journal of Learning during language production: an event-related synthetic Disabilities 2008: 41(1): pp 47-66. Garcia Dominguez L, Wennberg R, Perez Velazquez JL, aperture magnetometry study. Cerebral Cortex 2007: Guevara Erra R: Enhanced measured synchronization 17: pp 2536-2543. García Domínguez L, Guevara Erra R, Wennberg R, varieties of pathways to dysfluent reading: comparing Perez Velazquez JL: On the spatial organization Kirton A, Chen R, Friefeld S, Gunraj C, Pontigon A, deVeber G: Contralesional repetitive transcranial of unsynchronized sources: inspecting the physiological significance of synchronization analysis of whole brain Imai K, Otsubo H, Sell E, Mohamed I, Ochi A, magnetic stimulation for chronic hemiparesis in electrophysiological recordings. International Journal RamachandranNair R, Snead OC III: MEG source subcortical paediatric stroke: a randomized trial. estimation from mesio-basal temporal areas in Lancet Neurology 2008: 6: pp 507-513. of Physical Sciences 2007: 2(11): pp 305-317. a child with a porencephalic cyst. ACTA Neurologica Scandinavica 2007: 116: pp 263-267. Kirton A, deVeber G, Pontigon A, MacGregor D, Shroff isolated syndromes. Annals of Neurology 2008: Isackson PJ, Bennett MJ, Lichter-Konecki U, Willis M, classification predicts outcomes. Annals of Neurology 63(3): pp 401-405. Nyhan WL, Sutton VR, Tein I, Vladutiu GD: CPT2 gene 2008: (63)4: pp 436-443. Ghassemi R, Antel S, Francis SJ, Banwell B, Arnold M: Presumed perinatal ischemic stroke: vascular DL: Lesion distribution in children with clinically mutations resulting in lethal neonatal or severe Gladstone JP: Dopamine and migraine: infantile carnitine palmitoyltransferase II deficiency. Kirton A, Tan M, Mikulis D, Fisher J, Han J, deVeber G, trigeminovascular nociception, genetics and Molecular Genetics and Metabolics 2008: June 10 Rubin B: Recurrent, reversible coma in an adolescent. therapeutics. Cephalalgia 2007: 27(11): pp 1315–1320. [Epub ahead of print]. The Lancet Neurology 2008: 7(1): pp 110-112. Hahn CD: The ARX story: a new twist. Kirton A, Westmacott R, deVeber G: Pediatric stroke: Neurology 2007: 69(5): pp 421-422. rehabilitation of focal injury in the developing brain. Neurorehabilitation 2007: 22(5): pp 371-382. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 125 Knerr I, Pearl PL, Bottiglieri T, Snead OC III, Jakobs C, Luca P, Laurin N, Misener VL, Wigg KG, Anderson B, Mirabella G, Morong S, Buncic JR, Snead OC III, Gibson KM: Therapeutic concepts in succinate Cate-Carter T, Tannock R, Humphries T, Lovett MW, Logan WJ, Weiss SK, Abdolell M, Westall CA: Pediatric semialdehyde dehydrogenase (SSADH;ALDH5a1) Barr CL: Association of the dopamine receptor D1 neurology emergencies. Canada International deficiency (gamma-hydroxybutyric aciduria). Hypotheses gene, DRD1, with inattention symptoms in families Scientific Exchange Program, University of Toronto. evolved from 25 years of patient evaluation, studies selected for reading problems. Molecular Psychiatry Contrast sensitivity is reduced in children with in Ald5a1-/- mice, and characterization of gamma- 2007: 8: pp 776-785. infantile spasms. Investigative Ophthalmology and Visual Science 2007: 48(8): pp 3610-3615. hydroxybutyric acid pharmacology. Journal of Inherited Metabolic Disease 2007: 30: pp 279-294. MacGregor DL: Shaken Baby Syndrome: the quest for evidence. Developmental Medicine and Child Kolski H, Hawkins C, Zatz M, de Paula F, Biggar D, Neurology 2008: 50(1): p 8. Mohamed I, Cheyne D, Gaetz WC, Otsubo H, Logan WJ, Snead OC III, Pang EW: Spatiotemporal patterns of oscillatory activity during auditory word recognition in Alman B, Vajsar J: Diagnosis of limb-girdle muscular children: a synthesis aperture magnetometry study. dystrophy 2A by immunohistochemical techniques. Maguire JL, deVeber G, Parkin P: Association between Neuropathology 2008: 28(3): pp 264-268. iron-deficiency anemia and stroke in young children. International Journal of Psychophysiology 2008: 68: Pediatrics 2007: 120(5): pp 1053-1057. pp 141-148. Lamhonwah AM, Hawkins C, Tam C, Wong J, Mai L, Tein I: Expression patterns of organic cation/carnitine Mahant S, Peterson R, Campbell M, MacGregor DL, Mohamed IS, Otsubo H, Imai K, Shroff M, Sharma R, transporter family in adult murine brain. Brain and Friedman JN: Reducing inappropriate hospital use Chuang SH, Donner EJ, Drake J, Snead OC III: Surgical Development 2008: 30(1): pp 31-42. on a general pediatric inpatient unit. Pediatrics 2008: treatment for acute symptomatic refractory status 121(5): pp e1068-e1073. Retrospective study of serum protein status in epilepticus: a case report. Journal of Child Neurology 2007: 22(4): pp 435-439. Liu YC, Lowe HL, Donner EJ, Kobayashi J: McLinden A, Baird AD, Westmacott R, Anderson PE, children fed the ketogenic diet via gastrostomy tube. deVeber G: Early cognitive outcome after neonatal Mohamed IS, Otsubo H, Ochi A, Elliott I, Donner EJ, Journal of American Dietetic Association 2007: stroke. Journal of Child Neurology 2007: 22(9): pp Chuang SH, Sharma R, Holowka S, Rutka JT, Snead 107(8): pp 686-695. 1111-1116. OC III: Utility of magnetoencephalography in the Lovett MW, Lacerenza L, De Palma M, Steinbach KA, Menascu S, Donner EJ: Linear Nevus Sebaceous Frijters JC: Preparing teachers to remediate reading Syndrome: case reports and review of the literature. disabilities in high school: what is needed for effective Pediatric Neurology 2008: 38: pp 207-210. evaluation of recurrent seizures after epilepsy surgery. professional development? Teaching and Teacher Education 2008: 24(4): pp 1083-1097. 126 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Epilepsia 2007: 48: pp 2150-2159. Nilsson D, Rutka JT, Snead OC III, Raybaud CR, Otsubo H, Ochi A, Imai K, Fujimoto A, Go C, Dirks P, Perez Velazquez JL, Garcia Dominguez L, Guevara Erra Widjaja E: Preserved structural integrity of white Donner EJ: High-frequency oscillations of ictal muscle R: Fluctuations in neuronal synchronization in brain matter adjacent to low-grade tumours. Child’s Nervous activity and epileptogenic discharges on intracranial activity correlate with the subjective experience of System 2008: 24: pp 313-320. EEG in a temporal lobe epilepsy patient. Clinical visual recognition. Journal of Biological Physics 2007: Neurophysiology 2008: 119(4): pp 862-868. 33: pp 49-59. Nofech-Mozes S, Blaser S, Roifman C, Grunebaum E, Kobayashi J: Neurological abnormalities in patients Pang EW, Drake J, Otsubo H, Martineau A, Strantzas Perez-Velazquez JL, Huo JZ, Dominguez LG, Leschenko Y, with adenosine deaminase deficiency. Pediatric S, Cheyne D, Gaetz W: Intraoperative confirmation Snead OC III: Typical versus atypical absence Neurology 2007: 37(3): pp 218-221. of hand motor area identified preoperatively by seizures: Network mechanisms of the spread of magnetoencephalography: case report. Pediatric paroxysms. Epilepsia 2007: 48: pp 1585-1593. Nylen K, Perez Velazquez JL, Likhodii SS, Cortez MA, Neurosurgery 2008: 44: pp 313-317. RamachandranNair R, Ochi A, Benifla M, Rutka JT, Shen L, Leshchenko V, Adeli K, Gibson KM, Burnham WM, Snead OC III: A ketogenic diet rescues the Pedersen CB, Kølvraa S, Kølvraa A, Stenbroen V, Snead OC III, Otsubo H: Benign epileptiform murine succinic semialdehyde dehydrogenase Kjeldsen M, Ensenauer R, Tein I, Matern D, Rinaldo P, discharges in Rolandic region with mesial temporal deficient phenotype. Experimental Neurology 2008: Vianey-Saban C, Ribes A, Lehnert W, Christensen E, lobe epilepsy: MEG, scalp and intracranial EEG 210: pp 449-457. Corydon TJ, Andresen BS, Vang S, Bolund L, Vockely J, features. Acta Neurologica Scandinavica 2007: Bross P, Gregersen N: The ACADS gene variation 13: pp 505-516. O’Shea AM, Wilson GJ, Ling SC, Minassian BA, spectrum in 114 patients with short-chain acyl-CoA Turnbull J, Cutz E: Lafora-like ground-glass inclusions dehydrogenase (SCAD) deficiency is dominated by RamachandranNair R, Ochi A, Imai K, Benifla M, in hepatocytes of pediatric patients: a report of two missense variations leading to protein misfolding Akiyama T, Holowka S, Rutka JT, Snead OC III, Otsubo cases. Pediatric Developmental Pathology 2007: at the cellular level. Human Genetics 2008: June 4 H: Epileptic spasms in older pediatric patients: MEG 10(5): pp 351-357. [Epub ahead of print]. and ictal high-frequency oscillations suggest focal- Ochi A, Otsubo H: Magnetoencephalography-guided Perez Velazquez JL, Galán, RF, Garcia Dominguez L, onset seizures in a subset of epileptic spasms. Epilepsy Research 2008: 78: pp 216-224. epilepsy surgery for children with intractable focal Leshchenko Y, Lo S, Belkas J, Erra RG: Phase epilepsy: SickKids experience. International Journal response curves in the characterization of epileptiform Sadeghnia HR, Cortez MA, Liu D, Hosseinzadhe H, of Psychophysiology 2008: 68: pp 104-110. activity. Physical Review. E, Statistical, Nonlinear, and Snead OC III: Anti-absence seizure activity of safranal: Soft Matter Physics 2007: 76(6 Pt 1): p 061912. an autoradiographic study. Journal of Pharmacy and Pharmaceutical Science 2008: 11(3): pp 1-14. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 127 Santoshkumar B, Turnbull J, Minassian BA: Unverricht- Sugiyama I, Iida K, Akiyama T, Otsubo H: Surgical Tein I, Elpeleg O, BenZe’ev B, Korman S, Lossos A, Lundborg progressive myoclonus epilepsy in Oman. treatment for intractable epilepsies in children. Lev D, Lerman-Sagie T, Leshinsky-Silver E, Vockley G, Pediatric Neurology 2008: 38(4): pp 252-255. Brain Medical 2008: 20: pp 57-65. (Japanese) Berry G, Lamhonwah AM, Matern D, Roe C, Gregersen Siintola E, Topcu M, Aula N, Lohi H, Minassian BA, Sutcliffe TL, Gaetz WC, Logan WJ, Cheyne DO, Fehlings (319 C>T) presents with clinical heterogeneity and is Paterson AD, Liu XQ, Wilson C, Lahtinen U, Anttonen D: Developmental disregard predicts improvement candidate founder mutation in individuals of Ashkenazi AK, Lehesjoki AE: The novel neuronal ceroid after constraint-induced movement therapy in Jewish origin. Molecular Genetics and Metabolism lipofuscinosis gene MFSD8 encodes a putative hemiplegic children. Journal of Developmental and 2008: 93(2): pp 179-189. lysosomal transporter. American Journal of Human Behavioural Pediatrics 2007: 28(4): p S3. N: Short-chain acyl-CoA dehydrogenase gene mutation Thomas T, Banwell B: Multiple sclerosis in children. Genetics 2007: 81(1): pp 136-146. Tagliabracci VS, Turnbull J, Wang W, Girard JM, Snead OC III, Donner EJ: A new generation of Seminars in Neurology 2008: 28(1): pp 69-83. Zhao X, Skurat AV, Delgado-Escueta AV, Minassian BA, Turnbull J, Kumar S, Ren Z, Shanmugakonar M, anticonvulsants for the treatment of epilepsy in DePaoli-Roach AA, Roach PJ: Laforin is a glycogen children. Paediatrics and Child Health 2007: 12(9): phosphatase, deficiency of which leads to elevated Naranian T, Ackerley CA, Minassian BA: Lafora pp 741-744. phosphorylation of glycogen in vivo. Proceedings of progressive myoclonus epilepsy: disease course the National Academy of Science USA 2007: 104(49): homogeneity in a genetic isolate. The Journal of Stewart LS, Nylen KJ, Persinger MA, Cortez MA, pp 19262-19266. Child Neurology 2008: 23(2): pp 240-242. Gibson KM, Snead OC III: Circadian distribution of generalized tonic-clonic seizures associated Taylor MJ: MEG in paediatrics: preface to special Wang CH, Finkel RS, Bertini ES, Schroth M, Simonds with murine succinic semialdehyde dehydrogenase issue. International Journal of Psychophysiology A, Wong B, Aloysius A, Morrison L, Main M, Crawford deficiency, a disorder of GABA metabolism. Epilepsy 2008: 68(2): pp 83-84. TO, Trela A, Vajsar J: Consensus statement for standard of care in spinal muscular atrophy. Journal of Child and Behaviour 2008: May 29 [Epub ahead of print]. Taylor MJ, Mills T, Smith ML, Pang EW: Face processing Striano P, Zara F, Turnbull J, Girard JM, Ackerley CA, in adolescents with and without epilepsy. International Cervasio M, DeRosa G, Del Basso-De Carro ML, Striano Journal of Psychophysiology 2008: 68: pp 94-103. Neurology 2007: 22(8): pp 1027-1049. Wedderburn L, Varsani H, Li C, Newton K, Amato A, S, Minassian BA: Typical progression of myoclonic Banwell B, Bove, Corse A, Emslie-Smith A, Harding P, epilepsy of the lafora type: a case report. National Hoogendijk J, Lundberg I, Marie S, Minetti C, Clinical Practice. Neurology 2007: 4(2): 106-111. Nennesmo I, Rushing E, Sewry C, Allen E, Charman S, Pilkington C, Holton J (the International Consensus Group on JDM Biopsy), and on behalf of the UK 128 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Juvenile Dermatomyositis Research Group: Wise JC, Sevcik RA, Morris RD, Lovett MW, Wolf M: International consensus on a proposed score system The relationship among receptive and expressive infectious, toxic and metabolic headaches. In: Wolff’s for muscle biopsy evaluation in patients with JDM, vocabulary, listening comprehension, pre-reading Headache and Other Head Pains (Silberstein, Dodick for potential use in clinical trials. Arthritis Care skills, word identification skills, and reading and Lipton, eds.). Oxford University Press, 2007. and Research 2007: 57(7): pp 1192-1201. comprehension by children with reading disabilities. Wells GD, Noseworthy MD, Hamilton J, Tein I: Bigal ME, Gladstone JP: Headaches attributed to Journal of Speech, Language, and Hearing Research Chu B, Pang EW, Ochi A, Sharma R, Hunjan A, Holowka 2007: 50(4): pp 1-17. S, Rutka J, Weiss S, Chuang S, Snead OC III, Otsubo Yamazaki M, Ochi A, Akiyama T, Otsubo H: Epileptic confirm the accuracy of MEG SEF locations. In: New H: Extra-operative somatosensory evoked potentials Skeletal muscle metabolic dysfunction in obesity and metabolic syndrome. Canadian Journal of Neurological Sciences 2008: 35(1): pp 31-40. high frequency oscillations. Epilepsy 2008: 2: Frontiers in Biomagnetism (Cheyne D, Ross B, Stroink pp 53-60. (Japanese) G, Weinberg H, eds.). Elsevier, Amsterdam, 2007. Westmacott R, McAndrews MP, Silver F: Altered effective connectivity within language Yoon G, Westmacott R, MacMillan L, Quercia N, Hahn C, Banwell B: Diseases of the peripheral networks following left hemisphere stroke. Koutsou P, Georghiou A, Christodoulou K, Banwell B: nervous system. In: Pediatric Critical Care Medicine: Brain and Cognition 2007: 63: p 1999. Complete deletion of the aprataxin gene: ataxia with Basic Science and Clinical Evidence (Wheeler DS, oculomotor apraxia type 1 with severe phenotype and Wong HR, Stanley TP, eds.). Springer-Verlag, Westmacott R, McAndrews MP, Silver FL: cognitive deficit. Journal of Neurology, Neurosurgery, London, England, 2007. Understanding medial temporal activation in memory and Psychiatry 2008: 79: pp 234-236. Jones-Gotman M, Smith ML, Wieser HG: Intra-arterial tasks: evidence from fMRI of encoding and recognition in a case of transient global amnesia. Hippocampus Zhang Y, Minassian BA: Will my Rett Syndrome patient amobarbital procedures. In: Epilepsy: A Comprehensive 2008: 18: 317-325. walk, talk and use her hands? Neurology 2008: Textbook, 2nd Edition (Engel J, Pedley T, eds.). 70(16): pp 1302-1303. Lippincott Williams & Wilkins, New York, 2008. BOOKS AND BOOK CHAPTERS Kerr M, Smith ML: Learning disorders. In: Epilepsy: A Widjaja E, Blaser S, Miller E, Kassner A, Shannon P, Chuang SH, Snead OC III, Raybaud CR: Evaluation of subcortical white matter and deep white matter tracts Bayle DJ, Bostan AC, Taylor MJ: Attention to the Comprehensive Textbook, 2nd Edition (Engel J, Pedley in malformations of cortical development. Epilepsia person or the emotion: underlying brain activations in T, eds.). Lippincott Williams & Wilkins, New York, 2008. 2007: 48: pp 1460-1469. MEG. In: New Frontiers in Biomagnetism (Cheyne D, Ross B, Stroink G, Weinberg H, eds.). Elsevier, Amsterdam, 2007. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 129 Martín del Campo C, Pérez Velázquez JL, Cortez MA: Sharma R, Pang EW, Mohamed I, Chu B, Hunjan A, FUNDING EEG recording in rodents. In: Current Protocols in Ochi A, Holowka S, Gaetz W, Chuang S, Snead OC, An investigation of the neural mechanisms underlying Neuroscience (Gerfen C, Rowgawski MA, eds.). Otsubo H: Magnetoencephalography in children: amblyopia. Wong A, Taylor MJ, Cheyne DO. Canadian John Wiley & Sons Inc., New Jersey, 2008. routine clinical protocol for intractable epilepsy Foundation for Innovation Leadership Fund at The Hospital for Sick Children. In: New Frontiers ($1,015,761 2007-2011) Otsubo H, Imai K: Clinical neurophysiology of cortical in Biomagnetism (Cheyne D, Ross B, Stroink G, malformations: magnetoencephalography and Weinberg H, eds.). Elsevier, Amsterdam, 2007. electroencephalography. In: Malformations of the Atypical absence epilepsy: GABA B receptor-mediated mechanisms. Snead OC III, Francis J, Cortez MA. Nervous System, Handbook of Clinical Neurology (Sarnet Stefan H, Olofsson OE, Snead OC III: Typical and Canadian Institutes of Health Research ($315,376 HB, Curatolo P, eds.). Elsevier Ltd., Edinburgh, 2007. atypical absence seizures, myoclonic absences, and 2000-2008) eyelid myoclonias. In: Epilepsy: A Comprehensive Pang EW, Otsubo H, Sharma R, Hunjan A, Chu B, Textbook, 2nd Edition (Engel J, Pedley T, eds.). Autism Treatment Network: Co-operative multicentre Snead OC III: Unilateral abnormalities on MEG Lippincott Williams & Wilkins, New York, 2008. program for research and treatment of autism. Syndrome. In: New Frontiers in Biomagnetism (Cheyne Tein I: Primary carnitine transporter defect. In: Neurobase, Goldfarb C, Berall G, Bradley E, Brian J, Freeman N, auditory evoked fields in children with Landau-Kleffner Roberts W, Loh A , Feigenbaum A, Weiss S, Marcon M, D, Ross B, Stroink G, Weinberg H, eds.). Elsevier, 14th Edition (Gilman N, Goldstein GW, Waxman SG, eds.). Atkinson A, Taylor MJ, Stinson J, Mitchell S, Green P. Amsterdam, 2007. Arbor Publishing Corp., La Jolla, CA, 2008. Autism Speaks ($1,000,000 2008-2012) Perez Velazquez JL, Garcia Dominguez L, Wennberg R: Thomas P, Snead OC III: Absence status epilepticus. Carnitine-responsive cardiomyopathy and the different- The fluctuating brain: dynamics of neuronal activity. In: In: Epilepsy: A Comprehensive Textbook, 2nd Edition affinity carnitine transporters. Tein I. Heart and Stroke Nonlinear Phenomena Research Perspectives (Wang (Engel J, Pedley T, eds.). Lippincott Williams & Wilkins, Foundation of Ontario ($267,144 2004-2007) CW, ed.). Nova Science Publishers, Inc., New York, 2008. New York, 2007. Centre for the Investigation of Neuroplasticity and Weiss SK: Sleep disorders. In: Adolescent Healthcare: Developmental Disorders. Snead OC III, Taylor MJ, RamachandranNair R, Weiss SK, MacGregor D: Sleep A Practical Guide, 5th Edition (Katzman D, ed.). Salter M, Josselyn S, Westall C, Dennis M, Tannock R, and metabolism. In: Head Injury in Children and Lippincott, Williams & Wilkins, Philadelphia, PA, 2008. Sled J, Cheyne D, Campbell M. Canadian Foundation Adolescents (MacGregor D, Kulkarni A, Dirks P, Rumney P, eds.). MacKeith Press, London, 2007. 130 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT for Innovation ($15,709,086 2006-2011) Centre for the Study of Complex Childhood Diseases. Genetics of reading disabilities. Barr CL, Kerr E, Investigation of an epilepsy epidemic in Cameroon. Post M (Tein I, CFI Team). Canada Foundation Lovett MW. Canadian Institutes for Health Research Snead OC III, Weiss SK, Elliott I, Smith ML, for Innovation (CFI) ($21,000,000 2007-2012) ($1,125,785 2008-2013) Stephen D, Keystone J, Cockburn L, Tsang V, Characterization of factors and mechanisms that Globus pallidus deep brain stimulation in children with Children’s Hospital Foundation Chair in explain the association between physical activity and dystonia. Soman T, Moro E, Lozano AM, Hodaie M, Paediatric Neuroscience ($20,000 2007-2008) lung health in cystic fibrosis: does muscle function Westmacott R, Gorman D. SickKids Foundation New play a role? Ratjen F, Coates A, Tein I. Canadian Investigator’s Grant ($118,617 2008-2010) Fongwa M, Takougang I, Akeneck J. Bloorview Krembil Neuroscience Centre Seeding Grant. Andrade D. Krembil Neuroscience Centre ($39,000 2007-2008) Cystic Fibrosis Foundation Grant ($158,000 2006-2008) Health-related quality of life in children with epilepsy: the first two years after diagnosis through parents’ Making a better brain: an Ontario neuroimaging CIHR Team in Childhood Obesity Research. McCrindle eyes. Smith ML, Nixon-Speechley K, Camfield C, consortium. Menon RS, Bartha R, Chronik BA, B, Anderson H, Hamilton J, Parkin P, Pencharz P Wiebe S, Zou G, Levin S. Canadian Institutes of Henkelman M, Kassner A, Lee TY, Mikulis D, Moller HJ, (Tein I, co-applicant). Canadian Institutes of Health Health Research ($393,692 2003-2008) Rutt BK, Sled JG, Taylor MJ. Ontario Research Foundation ($4,161,595 2008-2011) Research ($2,452,522 2008-2013) Identification of genetic causes of paediatric Cognitive behavioural therapy as a novel approach to neurological conditions and using these to uncover the MEB diseases. Vajsar J. SickKids Foundation the management of dystonia in children: a pilot study. cellular pathways involved and to determine the ways ($37,000 2006-2007) Soman T, Logan W, Chen R, O’Driscoll H. SickKids in which they are disturbed. Minassian BA. Canada Foundation ($4,500 2007-2008) Research Chairs Program ($478,585 2006-2011) Molecular basis of Lafora bodies (starch formation in the brain) and Lofora’s progressive myoclonus epilepsy. Minassian BA, Ackerley CA, Scherer SW, CREMS – Comprehensive Research Experience for Immune regulation and biomarker development for Medical Students. Weiss SK, Zak M, Mulligan J. The paediatric and adult autoimmune diseases. Bar-Or A, Lohi H. Canadian Institutes of Health Research Hospital for Sick Children, Division of Neurology; Banwell B, Piccirillo P, Sherman P, Polychronakos C. ($1,146,830 2006-2011) CREMS-University of Toronto ($5,000 2008) Canadian Institutes of Health Research New Emerging Team Grants ($2,018,445 2007-2012) Curing adult polyglucosan body disease by eliminating Multimodal neuroimaging of frontal lobe cognitive function in children. Taylor MJ, Donner, EJ, Pang EW. the polyglucosan bodies. Minassian BA. Adult Canadian Institutes of Health Research ($466,335 Polyglucosan Body Disease Research Foundation 2006-2011) ($59,000 2007-2008) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 131 Multimodal neuroimaging and neurobehavioural Neuroimaging measures of brain function in preterm Prospective study of the clinical epidemiology, outcomes in preterm neonates. Taylor MK, Whyte H, neonates. Taylor MJ, Sled JG, McNamara P, Whyte pathology, and neuroimaging features of Canadian Shroff M, Donner E, Moore A, Soman T. Canadian HEA, Moore AM, Donner EJ, Shroff MM. Collaborative children with clinically isolated demyelinating Institutes of Health Research ($925,000 2007-2012) Health Research Program ($361,860 2008-2011) syndromes. Banwell B, Bar-Or A, Sadovnick D, Arnold Multiple-component remediation for struggling Neuroimaging measures of brain function in preterm ($4,300,000 2004-2009) D. Multiple Sclerosis Scientific Research Foundation middle school readers. Morris R, Lovett MW, neonates. Taylor M, Donner EJ, McNamara PJ, Sevcik R, Frijters JC. Institute of Education Sciences Moore AM, Shroff MM, Sled JG, Whyte HEA: Natural Randomized controlled trial to improve sleep in children ($2,882,630 US 2006-2010) Sciences and Engineering Research Council who have both epilepsy and neurodevelopmental of Canada ($361,860 2008-2011) disabilities. Weiss SK. The Hospital for Sick Children National Grants Program ($121,328 2007-2009) Multi-site paediatric network for fMRI mapping in childhood epilepsy. Smith ML, Gaillard WD. Neuroprotection by inhibitors of apoptosis proteins American Epilepsy Society ($60,000 2005-2007) following neonatal stroke. Askalan R. Clinician- Role of the carnitine/organic cation transporters in Scientist Award (Phase I), Heart and Stroke the mammary gland and implications for the suckling Foundation of Ontario ($50,000 2007-2010) infant. Tein I. Mead-Johnson Grant ($28,500 2007-2008) Murine knockout model of 4-hydroxybutyric aciduria. Snead OC III, Gibson KM. National Institute of Non-invasive decompression of the superior sagittal Shared epitopes of functional variants of the organic sinus to prevent perinatal brain injury in neonatal cation/carnitine transporter family (OCTN1 and OCTN2) Murine treadmill (6-lane) with electric shocker. Tein I. cerebral sinovenous thrombosis. deVeber G, Kirton A, and campylobacter jejuni may underlie susceptibility to SickKids Foundation Equipment Grant ($6,500 2008) Tan M, Shroff M, Moore A. Ontario Federation for Crohn’s disease and Guillain-Barre syndrome. Tein I. Cerebral Palsy ($12,875 2007-2008) SickKids Foundation ($5,000 2007-2008) with autism. Taylor M, Donner EJ, Roberts SW, Predicting neurobehavioural outcome in preterm Spatio-temporal patterns of synchronized brain activity Bayless S, Shroff M. National Alliance for Autism neonates. Taylor M.J, Whyte HE, Raybaud C, Moore Perez Velazquez JL. National Science and Engineering Research ($155,907 2006-2008) AM, Donner EJ, Shroff MM, Frisk VA. Canadian Research Council of Canada Discovery Grant Institutes of Health Research ($929,129 2007-2012) ($172,165 2007-2011) Health ($587,500 USD 2006-2011) Neuroimaging and frontal lobe function in children 132 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT The Children’s Stroke Project. deVeber G. Anonymous TIPS (Tips for Infant and Parent Sleep). Stremler R, Validation of accelerometry as a measure of physical Children’s Foundation ($2,070,000 2001-2009) Weiss SK, Hodnett E, Lee K, MacFarlane J, Willan A. activity and inactivity in children with chronic disease. Canadian Institutes of Health Research ($274,846 Feldman B, Stephens S, Takken T, Van der Net J, 2007-2009) Tremblay M, Eslinger D, Bigger W, Wright V, The practice of paediatric movement disorders amongst Schneiderman-Walker J, Longmuir P, Banwell B, paediatricians. Soman TB, Lamba D, Logan WJ, Beyenne J. Canadian Institutes of Health Research, Lang AE. SickKids Foundation ($500 2006-2008) Towards the correction of Rett-like behaviour in MeCP2-deficient mice by exogenous gene delivery. Institute of Musculoskeletal Health and Arthritis - The role of muscle carnitine deficiency and the Snead OC III. Canadian Institutes of Health Research Seed Grant: Physical Activity, Sport and Health carnitine/organic cation transporter family in ($117,137 2004-2009) Competition ($64,985 2007-2008) Duchenne muscular dystrophy. Tein I. SickKids Traumatic brain injury in children and youth: the Validation of the NIH stroke scale in children. deVeber complexities of co-morbidities. Perez Velazquez JL, G, Ichord R. National Institute of Neurological The role of vigabatrin in the treatment of infantile Hutchison J, Schachar R. Ontario Neurotrauma Disorders and Stroke ($45,000 2006-2009) spasms. Carmant L, Weiss, SK. Canadian Institutes Foundation ($250,000 2007-2010) Foundation ($7,650 2007-2008) Variability of brain coordination dynamics as a of Health Research ($860,000 2003-2009) Unravelling the pathogenesis of the autophagic prognostic tool in traumatic brain injury. Perez The Ts65Dn mice model. Cortez MA. SickKids vacuolar myopathies. Minassian BA, Ackerley C, Velazquez JL. SickKids Foundation ($48,500 Foundation ($10,000 2005-2008) Manolson MF. Canadian Institutes of Health 2007-2008) Research ($779,395 2007-2012) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 133 Division of Dr. Angelo Mikrogianakis DIVISION HEAD Dr. William Mounstephen FULL-TIME Dr. Kathy Boutis Dr. Kevin Chan Dr. Seen Chung Dr. Dr. Dr. Dr. Dr. Dr. Dr. Yaron Finkelstein Stephen Freedman Shauna Jain Anna Jarvis Kelly Keogh Luba Komar Amina Lalani Dr. Sanjay Mehta Dr. Angelo Mikrogianakis Dr. Bruce Minnes Dr. Trent Mizzi Dr. Jonathan Pirie Dr. Savithiri Ratnapalan Dr. Susan Schneeweiss Dr. Suzanne Schuh Dr. Dennis Scolnik Dr. Jennifer Thull-Freedman PART-TIME Dr. Sherry Baine Dr. Susanna Benseler Dr. Carolyn Calpin Dr. Joseph Copeland Dr. Anh Do Dr. Janine Flanagan Dr. John Freedman Dr. Claudio Fregonas Dr. Sabine George Dr. Cara Gilman Dr. Peter Glazer Dr. Donna Goldenberg Dr. Massoud Grouhi Dr. Shuja Hafiz Dr. Mark Handley-Derry Dr. Leah Harrington Division of Paediatric Emergency Medicine OVERVIEW paediatric emergency medicine knowledge and skills. HONOURS AND AWARDS The Emergency Department (ED) at The Hospital for We have also worked with the Canadian Armed Forces Chan K: Division of Paediatric Emergency Medicine, Sick Children provides acute care to children who are ill to provide paediatric training to their physician The Hospital for Sick Children Research Recognition or who have been injured. We are both the primary care assistants. We provide telephone consultative support Award in recognition of excellence in outstanding centre for children in Toronto, plus the tertiary referral to the Canadian Armed Forces in Afghanistan for any effort and contribution to research, 2007-2008. centre for the Greater Toronto Area (GTA), in addition to paediatric concerns they may encounter. The division being the designated Paediatric Trauma Centre for the continues to run Paediatric Life Support Courses (PALS Chan K: Fellow, Munk Centre for International Studies, GTA. The division has 21 full-time staff, the majority of and APLS). We also have a well-developed weekly University of Toronto, 2007. whom are recertified in both paediatrics and paediatric half-day of educational activities for trainees and all emergency medicine, plus a cadre of part-time health care providers in the Emergency Department. Dr. Leah Harrington: RHA Haslam, Pre-clerkship paediatricians. Over the next two to three years we will In the fall, the division will present the fifth annual Teaching Award, Department of Paediatrics, The be redeveloping the Emergency Department’s physical Paediatric Emergency Medicine Update which in the Hospital for Sick Children, University of Toronto, 2008 plant. This redevelopment will ensure that the ED at past four years has been an overwhelming success. SickKids remains a leader in paediatric emergency care. In addition, we have recently introduced an electronic Mehta S: RHA Haslam Pre-clerkship Teaching Award for Research in the division remains clinically focused, Excellence in Pre-clerkship Teaching (Runner-up), 2008. tracking system that replaces the old white board. covering a wide range of topics from respiratory These changes will allow us to provide more efficient disease acute care management, orthopaedic injuries, Mikrogianakis A: The Hospital for Sick Children, and effective care to our patients. pain and sedation, to drug errors. Research done Department of Paediatrics, the Richard Rowe Award within the division continues to be presented for Clinical Excellence in Paediatric Medical Care The division has an active three-year academic nationally and internationally with publications in (Junior Award), 2008. fellowship program. The Royal College of Physicians peer-reviewed journals. Overall, a substantial increase and Surgeons has fully accredited our fellowship in clinical research activities has resulted, putting Mikrogianakis A: Clinical Recognition Award. In program. In addition, we have a number of clinical us on par with the other North American leaders in recognition of outstanding effort and contribution departmental fellows from around the world spending paediatric emergency medicine. to the Emergency Department, Division of Paediatric a year or more with us further developing their Emergency Medicine Awards Day, 2008. Mikrogianakis A: Postgraduate PERLS Teaching Award (Runner-up), 2008. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Nicole Inch Edward Jin Eddy Lau Yen Le Patricia Linders Steven Moss Patricia Neelands Dr. Carole O’Beirne Dr. Linda Pedder Dr. Christina Ricks Dr. Jennifer Riley Dr. Lillian Rojnica Dr. Domenick Shelton Dr. Heshy Sturm Dr. Dr. Dr. Dr. Dr. Rahim Valani Charmaine Van Schaik Jeff Weisbrot Sheldon Wise Hirotaka Yamashiro CROSS-APPOINTEES Dr. Eyal Cohen Dr. Astrid Guttmann Dr. Ronik Kanani Dr. Christoph Licht Dr. Jonathan Tolkin Dr. Hilary Whyte Dr. Jamie Hutchison CLINICAL FELLOWS Dr. Amir Ahmad Dr. Samim Al-Qadhi Dr. Miguel Glatstein Dr. Alexandra Hernandez Dr. Maria Oliva Dr. Charles Stewart Dr. Klaus Werner ACADEMIC FELLOWS Dr. Abdullah Al-Dhafian Dr. Nesrin Alharthy Dr. Ahmed Al-Saleh Dr. Alawi Baaboud Dr. Vered Gazit Dr. April Kam The Hospital for Sick Children 2007-08 ANNUAL REPORT 135 Mikrogianakis A: Lionel Weinstein Clerkship Teaching PUBLICATIONS Dubnov-Raz G, Fogelman R, Juurlink D. N, Merlob P, Award for Excellence in Teaching, Department of Al-Ansari K, Sulowski C, Ratnapalan S: Analgesia and Ito S, Koren G, Finkelstein Y: Prolonged QT interval in Paediatrics, The Hospital for Sick Children, University sedation practices for incarcerated inguinal hernias neonates exposed to selective serotonin reuptake of Toronto (Runner-up), 2008. in children. Clinical Pediatrics (Philadelphia) 2008: inhibitors in late pregnancy. Clinical Pharmacology May 19 [Epub ahead of print]. and Therapeutics 2008: 83: p S42. Pirie J: Award for Excellence in Postgraduate Medical Education in Teaching Performance/Mentorship/ Avner M, Finkelstein Y, Hackam D, Koren G: Finkelstein Y, Schechter T, Garcia F, Nurmohamed L, Advocacy, University of Toronto, 2008. Establishing causality in pediatric adverse drug Juurlink D, Blanchette V, Koren G: Is morphine reactions: use of the Naranjo Probability Scale. exposure associated with acute chest syndrome in Pirie J: Continuing Education Award. For excellence Pediatric Drugs 2007: 9: pp 267-270. Paediatrics, The Hospital for Sick Children, 2008. children with vaso-occlusive crisis of sickle cell disease? A 6-year case-crossover study. Clinical in long-term contribution to CE in the Department of Boutis K, Willison D: To pay or not to pay: is that the Therapeutics 2007: 29: pp 2738-2743. right question? Student BMJ 2008: 16: pp 118-119. Finkelstein Y, Garcia-Bournissen F, Schechter T, Pirie J: Clinical Teaching Award, Division of Paediatric Nurmohamed L, Koren G: Is morphine administration Emergency Medicine, The Hospital for Sick Children Brandao L, Harrington D. J, Finkelstein Y, Cohen E, (Runner-up), 2008. Thompson M, Verjee Z: Superwarfarin exposure and to children with sickle cell disease: and vaso-occlusive severe coagulopathy in two infants: novel biomarkers crisis associated with an increased risk for the Schuh S: Article selected as one of the Best Articles for diagnosis and prolonged therapy. Clinical development of acute chest syndrome: a case- Relevant to Pediatric Immunology by the American Toxicology 2007: 45: pp 637. fluticasone does not replace oral prednisolone in children with mild to moderate acute asthma. Scolnik D: Global Health Scholar, Peter A. Silverman Chan KJ: A clinical trial gone awry: the Chocolate Happiness Undergoing More Pleasantness (CHUMP) Finkelstein Y, Nava-Ocampo AA, St. Pierre E, study. Canadian Medical Association Journal 2007: Schechter T, Walker S, Koren G: Population 177: pp 4-5. pharmacokinetics of doxorubicin in infants and children with malignant diseases. Clinical Centre for International Health, Mount Sinai Hospital, University of Toronto, 2007. Dubnov G, Finkelstein Y, Koren G: Recommend n-3 fatty acids in pregnancy? Canadian Family Physician 2007: 53: pp 1299-1300. Dr. Dr. Dr. Dr. Melissa Parker Tania Principi Ayelet Rimon-Babad Hayat Saddeq ADMINISTRATIVE STAFF Majda Savaglio Andrew Serrano Sabrina Dubeau Nicole Winters Maggie Stevanovic cross-over study. Clinical Pharmacology and Therapeutics 2007: 81: pp S78-S79. Academy of Pediatrics. Title: High-dose inhaled Deborah Atchison Joanna Barnes Dana Doll Ritsa Iriotakis Patricia Nedanovski Pauline Smith Viki Dukic Traci Peggie Sandra Miller Pharmacology and Therapeutics 2007: 81: p S78. Finkelstein Y, Fogelman R, Juurlink D. N, Merlob P, Ito S, Goldman RD, Mounstephen W, Langer J: Prospective Mikrogianakis A, Osmond MH, Nuth JE, Shephard A, Koren G, Dubnov-Raz G: Antenatal exposure to selective validation of pediatric appendicitis score. Journal of Gaboury I, Jabbour M: Evaluation of a multidisciplinary serotonin reuptake inhibitors prolongs the QT Interval in Pediatrics 2008: March 19 [Epub ahead of print]. pediatric mock trauma code educational initiative: a pilot study. Journal of Trauma 2008: 64(3): 761-767. Neonates. Clinical Toxicology 2007: 45: p 624. Goldman RD, Narula N, Klein-Kremer A, Finkelstein Y, Freedman SB, Adler M, Seshadri R, Powell EC: Rogovik AL: Predictors for opioid analgesia Osmond MH, Gazarian M, Henry RL, Clifford T, Tetzlaff Antiemetics for acute gastroenteritis: a never ending administration in children with abdominal pain J, Mikrogianakis A, and the PERC Spacer Study Group: story. Journal of Pediatric Gastroenterology and presenting to the emergency department. Clinical Barriers to metered-dose inhaler/spacer use in Nutrition 2007: 45: pp 132-133. Journal of Pain 2008: 24(1): pp 11-15. Canadian pediatric emergency departments. Academic Freedman SB: Acute infectious pediatric gastroenteritis: Goldman RD, Scolnik D, Chauvin-Kimoff L, Farion KJ, Ali beyond oral rehydration therapy. Expert Opinion on S, Lynch T, Gouin SM, Osmond M, Johnson DW, Klassen Pirie J: Pre- and post-paediatric emergency care: Pharmacotherapy 2007: 8: pp 1651-1665. TP: Practice variation among paediatric emergency where do we go from here? Paediatrics and Child physicians in the management of febrile neonates. Health 2007: 12: p 451. Emergency Medicine 2007: 14(11): pp1106-1113. Freedman SB, Thakkar VA: Easing the strain on a Paediatric Child Health 2007: 12 Suppl: p 61A. Ratnapalan S: Cutting the vagus nerve: clinical pediatric tertiary care center: the use of a redistribution system. Archives of Pediatrics and Harrington DJ, Dominic J, Brandao LR, Leonardo R, practice and research in medicine. Canadian Family Adolescent Medicine 2007: 161(9): pp 870-876. Cohen E, Finkelstein Y, Thompson M, Verjee Z: Physician. 2008: 54(5): p 748. Superwarfarin exposure and severe coagulopathy in Freedman SB, Haladyn JK, Floh A, Kirsh J, Taylor G, two infant immigrants: unequivocal diagnosis using Ricks C, Ratnapalan S, Jain S, Tait G: Evaluating Thull-Freedman J: Pediatric myocarditis: emergency novel biomarkers. Therapeutic Drug Monitoring 2007: computer-assisted learning for common pediatric department clinical findings and diagnostic evaluation. 29: p 466. emergency procedures. Pediatric Emergency Care 2008: 24(5): pp 284-286. Pediatrics 2007: 120(6): pp 1278-1285. Mehta SV: Neuroimaging for pediatric minor closed Freedman SB, Thull-Freedman J: Pediatric dehydration head injuries. Paediatrics and Child Health 2007: assessment and oral rehydration therapy. Pediatric 12(6): pp 482-484. Emergency Medicine Reports 2008: 13(2): pp13-28. Schechter T, Finkelstein Y, Williams S, Chan A K, Ali M, Brandao LR, Leonardo R: Dosing and safety of unfractionated heparin in infants with thrombotic complications. Pediatric Blood and Cancer 2007: 48: pp 642-643. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 137 Schecheter T, Afzal S, Finkelstein Y, Doyle J, Dupuis L: BOOKS AND BOOK CHAPTERS Finkelstein Y: Poisoning in pregnancy. In: Medication Daclizumab therapy for pediatric steroid-resistant Chan K: Economics and global health. In: Understanding Safety in Pregnancy and Breastfeeding (Koren G, ed.). graft-versus-host disease. Paediatric Transplantation Global Health (William Markle, Melanie Fisher, Ray McGraw Hill Publication, New York, 2007. 2008: May 11 [Epub ahead of print]. Smego, eds.). McGraw Hill Press, New York, 2007. Scolnik D, Lovinsky R, Aronson L, Toledano K, Glazier Chan K, Rosenberg M, Stewart CC, Hall T: Hurdles and Mehta SV: Smoke inhalation. In: The Hospital for Sick Children Manual of Pediatric Trauma (Cheng A, R, Eisenstadt J, Eisenberg P, Wilcox L, Rowsell R, opportunities in global health program. In: Developing Mikrogianakis A, Valani R, eds.). Lippincott, Williams Silverman M: Efficacy of a targeted, oral penicillin- Residency Training in Global Health: A Guidebook & Williams, Toronto, 2007. based yaws control program among children living in (Evert J, Stewart C, Chan K, Rosenberg M, Hall T, rural South America. Clinical Infectious Disease 2003: eds.). Global Health Education Consortium, San Pirie J, Hutchison C: Pediatric procedures. In: The 36(10): pp 1232-1238. Francisco, 2008. Hospital for Sick Children Handbook of Pediatrics, 11th Edition (Dipchand A, Friedman J, eds.). Elsevier Canada, Toronto, 2008. Tallett S, Lingard L, Hellmann J, Hilliard R, Hurley J, Chan K, Stewart CC, Hall T: Resources for teaching in Leslie K, Jefferies A, Mian M, Pirie J, Rosenfield J, global health. In: Developing Residency Training in Schneider R, Spero L: Measuring educational Global Health: A Guidebook (Evert J, Stewart C, Chan Scolnik D: Health as a bridge to peace: CISEPO workload: a pilot study of paper-based and PDA tools. K, Rosenberg M, Hall T, eds.). Global Health as a case study. VDM Verlag Dr. Muller, Saarbrucken, Medical Teacher 2008: 30: pp 296-301. Education Consortium, San Francisco, 2008. Germany, 2007. Stewart CC, Dillibaugh L, Chan K: Examples of global Warren DW, Jarvis DA, LeBlanc L, Gravel J, and Creery D, Mikrogianakis A: Sudden infant death the CTAS National Working Group (NWG): Revisions syndrome, BMJ Clinical Evidence Handbook, BMJ health curricular components. In: Developing to the Canadian Triage and Acuity Scale Paediatric Publishing Group Ltd., London, UK, 2007. Residency Training in Global Health: A Guidebook Evert J, Stewart C, Chan K, Rosenberg M, Hall T: eds.). Global Health Education Consortium, San Developing Residency Training in Global Health: Francisco, 2008. (Evert J, Stewart C, Chan K, Rosenberg M, Hall T, Guidelines (PaedCTAS) ED administration. Canadian Journal of Emergency Medicine 2008: 10(3): p 224. A Guidebook. Global Health Education Consortium, San Francisco, 2008. 138 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT FUNDING Children with fractures suspicious for abuse. Why are Predictors for diagnostically accurate ultrasound A randomized controlled trial of casting versus wrist they missed in the emergency department? Boutis K, in children with suspected appendicitis. Doria A, splint in children with minimally angulated distal radius Alharty N, Cory E, Au H, Ravichandiran N. Canadian Schuh S, Cheng A, Langer J, Tomlinson G. fractures in children. Boutis K, Howard A, Babyn P, Hospitals Injury Reporting and Prevention Program SickKids Foundation ($128,000 2007-2009) Willan A. SickKids Foundation ($129,775 2006-2008) ($4,000 2006-2007) Palatability of oral rehydration solutions (PORS). Barriers on the use of paediatric ankle X-ray decision Global health education consortium global health Freedman SB, Boutis K, Schuh S. Paediatric rules. Boutis K, Constantine E, Narayanan U. The modules. Hall H, Chan K, Neusy A-J, Jones E. Consultants Partnership’s Grant ($8,210 2007-2009) Hospital for Sick Children Paediatric Consultants Margaret Kendrick Blodgett Foundation Partnership’s Grant ($6,650 2007-2008) ($275,000 2006-2008) Emergency department rapid intravenous rehydration Funding for the position of a research co-ordinator Roifman C, Richardson S, Schuh S. The Hospital for (RIVR) for paediatric gastroenteritis: a randomized in the emergency department. Schuh S. Trudell Sick Children Research Institute ($33,800 2007-2008) controlled trial. Freedman S, Parkin P, Willan A, Medical ($50,000 2006-2008) Severe influenza in children: genetic determinants and related epidemiology. Tran D, Paterson A, Beyene J, Severe influenza in children: genetic determinants and Schuh S. Physicians’ Services Incorporated Foundation ($165,000 2006-2008) Evaluate run exercises trained versus non-trained to related epidemiology. Tran, D, Paterson A, Beyene J, Impact of oximetry on hospitalization in acute bronchiolitis. Schuh S, Freedman SB, Coates A, Roifman C, Richardson S, Schuh S. University Parkin P, Allen U, Willan A, Ungar W, Da Silva Z. of Toronto Dean’s Fund ($10,000 2007-2008) evaluate the impact of the curriculum on performance. Physicians’ Services Incorporated Foundation Komar L. National CBRNE ($2.8 million 2008-2010) ($163,000 2007-2009) The MAST project: magnesium for sickle cell trial. Goldman RD, Kirby-Allen M, Mounstephen W, Exploring novel methods to improve our diagnostic Impact of oximetry on hospitalization in acute Friedman J. Physician’s Services Incorporated accuracy of childhood bacterial pneumonia. Lynch T, bronchiolitis. Schuh S, Freedman SB, Coates A, Foundation ($157,000 2006-2008) Klassen T, Boutis K, Johnson. Canadian Institutes Parkin P, Allen U, Willan A, Ungar W, Da Silva Z. of Health Research ($75,000 2006-2009) Thrasher Research Fund ($125,000 2007-2010) Young infants with acute recurrent wheezing: to puff or not to puff? A randomized controlled trial. Grassemann Isotonic versus hypotonic IV maintenance fluids H, Schuh S, Willan A, Dick P, Coates A, Ungar W. in children: a randomized controlled trial. Geary D, SickKids Foundation ($169,500 2008-2010) Freedman SB, Beck C, Freidman JN. Paediatric Consultants Partnership’s Grant ($9,956 2007-2008) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 139 Division of Dr. Zia Bismilla 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. Robert Hilliard Dr. Sanjay Mahant Dr. Charlotte Moore Dr. Patricia Parkin Dr. Elena Pope Dr. Adam Rapoport Dr. Michelle Shouldice Dr. Susan Tallett Dr. Michael Weinstein MAJOR PART-TIME Dr. Mark Feldman Dr. Saul Greenberg Dr. Moshe Ipp Dr. Sheila Jacobson Dr. Michael Peer Dr. Miriam Weinstein PART-TIME Dr. Tony Barozzino Dr. Salvatore Cammisuli Dr. Nhung Ho Dr. Alex Levin Dr. Michael Sgro Dr. Christine Webster ASSOCIATE Dr. Nirit Bernhard Dr. Mariana Boragina Dr. Ronik Kanani Dr. Sanjeev Luthra Dr. Ivor Margolis Dr. Sharon Naymark Division of Paediatric Medicine OVERVIEW The divisional complex care program continues to section. Dr. Jeremy Friedman is an associate editor Faculty in the the Division of Paediatric Medicine had a develop under the leadership of Dr. Eyal Cohen, whose of the Paediatrics and Child Health Journal. The two successful and productive year in 2007-08. In 2008, efforts were recognized by his selection to join the books for parents written by divisional members we welcomed one new full-time faculty, Dr. Zia Bismilla. Ontario MOH expert panel on complex care in children. (Caring for Kids, 2006 and Canada’s Baby Care, 2007) After completing her residency with her final year as On the clinical side, a dedicated inpatient complex and edited by Drs. Friedman and Saunders, are both associate chief resident, Zia was accepted into the care team now operates four to six beds and is staffed Canadian bestsellers and on sale internationally as well. Paediatric Medicine fellowship. During her two-year by a hospitalist with complex care expertise and a fellowship, she successfully completed her Masters of nurse practitioner. The ambulatory arm of the complex This has been a successful year with regards to Education at OISE and was also resident editor of the care team has just begun taking referrals through research productivity. There were over 20 abstracts 11th Edition of the SickKids Handbook. Her major ARMs, although capacity remains limited at this time. from the division at the annual PAS and CPS meetings, academic interest is in the area of simulation, and she The hospital has helped fund a proposed pilot project including many platform presentations. Funding from presented her research at the CPS and PAS meetings. to study a new model of care, whereby a medical home both internal and external agencies was secured, for the most complex multi-system patients with high including a number of CIHR grants. PORT, under the Our part-time inpatient hospitalist group has grown resource requirements would be developed in their leadership of Dr. Patricia Parkin, was funded by the and now includes Drs. Ronik Kanani (NYGH), Yehuda community hospitals in collaboration with the SickKids SickKids Foundation for a further five-year term. Many Mozes (SCH), Ivor Margolis and Sanjeev Luthra program. The Norman Saunders Complex Care manuscripts were published in top general paediatric (WOMC), Tony Barozzino, Mike Sgro and Ethel Ying Initiative will award its first research grants after journals (see below). (St. Mike’s). These individuals, who function as the inaugural annual competition this summer. Once again, the residents identified their general hospitalists/consultants at their ‘home’ hospitals, each provide us with two to four weeks of inpatient Faculty members continued to excel and gain paediatric ward rotation as their best experience of attending every six months, connecting our tertiary recognition as leaders in the field of paediatric medicine. the year, and many faculty members were recognized care group more closely to the community and the Dr. Robert Hilliard was elected to the position of VP with teaching awards (see below). Special mention care provided outside of SickKids. Their contribution of the Canadian Paediatric Society, and will serve as to Dr. Stacey Bernstein, who won the prestigious is much appreciated. A formal fellowship in president in two years time. Dr. Sanjay Mahant was University of Toronto WT Aikins Award, and Dr. Eyal Community Paediatrics has been developed under elected president of the Hospital Medicine section Cohen for winning the Department of Paediatrics the leadership of Dr. Mark Feldman, with the support at the CPS, while Dr. Mark Feldman is the current Harry Bain Award. of the Department of Paediatrics at SickKids, and president of the Community Paediatrics the first fellow will start her training in 2009. Dr. Yehuda Nofech-Mozes Dr. Gary Sibbald Dr. Ethel Ying ADJUNCT Dr. Peter Azzopardi Dr. Ranjit Baboolal Dr. Anna Banerji Dr. Bert Cellupica Dr. Patricia Colangelo Dr. Hilary de Veber Dr. Paul Dick Dr. Suhid (Tony) D’Souza Dr. Michelle Farine Dr. Dr. Dr. Dr. Dr. Dr. Dr. Claudio Fregonas Gerald Friedman Marvin H. Gans Hartley A. Garfield Morton Goldbach Leslie Goodis Stuart Gordner Dr. Dr. Dr. Dr. Dr. Dr. Dr. Michelle Gordon Masoud Grouhi James D. Hilton Tommy Ho Alan P. Hudak Anthony Y.T. Hui Edward G. Jin Dr. Dr. Dr. Dr. Dr. Dr. Dr. Beverley Kupfert Eddy Lau Cinda B.S. Lee Maurice Levy Aubrey Maze Anilkumar Menon Saba Merchant Dr. Dr. Dr. Dr. Dr. Dr. Dr. Steven Moss Larry B. Pancer John Philpott S. Joseph Porepa Vladimir Pucholt Percy Rajchgot Harkamal Randhawa The Hospital for Sick Children 2007-08 ANNUAL REPORT 141 HONOURS AND AWARDS Weinstein M: James C. Fallis Teaching Award, Invited Beck C, Parkin P, Friedman JN: Pediatric hospitalist Bernstein S: WT Aikins Award for Individual Teaching Speaker, Division of Paediatric Emergency Medicine, medicine: an overview and a perspective from Toronto, Performance, University of Toronto, 2008. The Hospital for Sick Children, 2008. Canada. Clinical Paediatrics 2008: 47(6): pp 546-548. Cohen E: Harry Bain Award for excellence in teaching PUBLICATIONS Beck CE: Hypotonic versus isotonic maintenance by a full-time member of the Department of Agha M, Glazier R, Guttmann A: Social inequalities intravenous fluid therapy in hospitalized children: Paediatrics, University of Toronto, 2008. affect ambulatory care sensitive hospitalizations a systematic review. Clinical Pediatrics 2007: 46: for up to nine years among children born in a major pp 764-770. Feldman M: Co-winner of the St. Joseph’s Health Centre Canadian urban centre. Ambulatory Pediatrics interdepartmental Best Clinical Teacher Award, 2007. 2007: 7: pp 258-262. Bismilla Z, Sell E, Donner E: Hashimoto’s encephalopathy. Journal of Child Neurology 2007: 22(7): pp 341-346. Lansang P, Pope E, Weinstein M: Alvin Jacobs’ Aljaser F, Weinstein M: A one-week-old newborn with Resident/Fellow Research Award. Growth outcomes hypercalcemia and palpable nodules: subcutaneous Chiu PL, Azzie G, Hilliard RI, Fecteau A: Experience of infants with hemangiomas treated with systemic fat necrosis. Canadian Medical Association Journal of moral distress among pediatric surgery trainees. steroids. Society for Pediatric Dermatology Annual 2008: 178(13):1653-4. Journal of Pediatric Surgery 2008: 43(6): pp 986-993. Meeting, Chicago, IL, 2007. Au H, Harrison M, Ahmet A, Orsino A, Beck CE, Cohen E, Friedman J, Nicholas D, Adams S, Lau E: Undergraduate Community Paediatric Teaching Tallett S, Gans M, Birken CS: Residents as Health Rosenbaum P: A home for medically complex children: Award, Department of Paediatrics, University of advocates: the development, implementation the role of hospital programs. Journal for Healthcare Toronto, 2008. and evaluation of a child advocacy initiative at the Quality 2008: 30: pp 7-15. University of Toronto. Paediatrics and Child Health Pope E: Promoted to Associate Professor, Department Cohen E, Navarro O: Silent aspiration. Pediatrics 2007: 12: pp 567-571. in Review 2007: 28: pp 419-425. of Paediatrics, University of Toronto, 2008. Barrera M, D’Agostino N, Schneiderman G, Tallett S, Tallett S: The Mickles Fellowship Award, Spencer L, Jovcevska V: Patterns of parental Cohen E, Weinstein M, Fisman DN: Cost effectiveness University of Toronto, 2007. bereavement following the loss of a child and related of competing strategies for the treatment of pediatric factors. OMEGA 2007: 55(2): pp 145-167. empyema. Pediatrics 2008: 121: pp e1250-e1257. Dr. Douglas Campbell Dr. Carlos E. Contreras Dr. Leona Fishman Dr. John S. Freedman Dr. Lee Ann Gallant Dr. Beth D. Gamulka Dr. Rose Geist Dr. Golda Milo-Manson Dr. Jay D. Rosenfield Dr. Suzanne Schuh Dr. Jeffrey J. Weisbrot Dr. Sheldon A. Wise Dr. Rahul Saxena Dr. Clive J. Schwartz Dr. David H. Shawn Dr. Angelo Simone Dr. William Gary Smith Dr. Joseph Telch Dr. Guy Widrich Dr. Peter D. Wong Dr. Heather Yang CROSS-APPOINTEES Dr. Glen B. Berall Dr. Alan Berzen Dr. Bonnie J. Cameron 142 The Hospital for Sick Children 2007-08 ANNUAL REPORT Dr. Sabine R. George Dr. Nicole G. Inch Dr. D. Anna Jarvis Dr. Ian C. Kitai Dr. Leo Levin Dr. Patricia M. Linders Dr. John S. Lovering HONORARY Dr. Lionel B.H. Boxall Dr. Andrew Chlebus Dr. William Davidson Dr. Till A. Davy Dr. Gordon J. Donsky Dr. James Dorey Cohen E, Zlotnik-Shaul R: Beyond the therapeutic Ipp M, Taddio A, Sam J, Goldbach M, Parkin PC: Mahant S, Peterson R, Campbell M, MacGregor DL, orphan: children and clinical trials. Pediatric Health Vaccine-related pain: randomised controlled trial Friedman JN: Reducing inappropriate hospital days on (invited submission) 2008: 2(2): pp 151-161. of two injection techniques. Archives of Diseases a general paediatric inpatient unit. Pediatrics 2008: of Childhood 2007: 92: pp 1105-1108. 121:(5): e1068-e1073. Kanani R, Mohamed M: Case 1: the case of the Metry DW, Siegel DH, Cordisco MR, Pope E, Finan E, Mak W, Bismilla Z, McNamara PJ: Early discontinuation of intravenous prostaglandin E(1) after balloon atrial septostomy is associated with irritable nephritic. Paediatrics and Child Health 2008: Prendiville J, Drolet BA, Horii KA, Stein SL, Frieden IJ: an increased risk of rebound hypoxemia. Journal 13(2): pp121-122. A comparison of disease severity among affected male versus female patients with PHACE syndrome. of Perinatology 2008: 28(5): pp 341-346. Lam J, Pope E: Pityriasis lichenoides and cutaneous The Journal of the American Academy of Dermatology Guttmann A, Manuel D, Stukel T, DeMeules M, Cernat T-cell lymphoma in paediatrics. Current Opinion in 2008: 58(1): pp 81-87. G, Glazier R: Immunization coverage among young Pediatrics 2007: 19(4): pp 441-445. O’Brodovich H, Beyene J, Tallett S, MacGregor D, children of immigrant mothers in Ontario, Canada. Ambulatory Pediatrics 2008: 8(3): pp 205-209. Guttmann A, Zagorski B, Austin P, Schull M, Razzaq A, Maguire J, DeVeber G, Parkin PC: The association Rosenblum N: Performance of a career development between iron deficiency anaemia and stroke in young and compensation program at an academic health children. Pediatrics 2007: 120: pp 1053-1057. science centre. Pediatrics 2007: 119: pp e791-e797. To T, Anderson G: Effectiveness of emergency department asthma management strategies on return visits in children: a population-based study. Pediatrics 2007: 120: pp e1402-e1410. Hawkes M, Kitai I, Blaser S, Cohen E, Bitnun A, Dr. Stacey Bernstein and team Fluss J, Tran D: Neuroimaging findings in isoniazid toxicity, presumed intramyelinic edema. European Journal of Pediatric Neurology 2008: February 1 [Epub ahead of print]. Dr. Katy Driver Dr. Andrew D. Duic Dr. Anthony S.R. Duke Dr. Thomas J. Egan Dr. Robert Farber Dr. Hyder H. Fazal Dr. William Feldman Dr. Adelaide L. Fleming Dr. Raphael Folman Dr. Katerina Haka-Ikse Dr. William B. Hanley Dr. Dirk W. Huyer Dr. Jaya Islur Dr. Martin H. Kosoy Dr. Howard M. Langer Dr. Michael Lester Dr. David C. Maresky Dr. Marcellina Mian Dr. Gilbert Miller Dr. Janette A. Milne Dr. Paul Munk Dr. John D.M. Murray Dr. Jack Newman Dr. Raymond C.W. Ng Dr. Evangelia Papadouris Dr. Helene A. Richards Dr. Chandrakant P. Shah Dr. Donald Stewart Dr. Peter G. Strachan Dr. Robert E. Sutton Dr. Martin G. Wolfish FELLOWS Dr. Sara Abed Dr. Alexandra Balma-Mena Dr. Zia Bismilla Dr. Malini Dave Dr. Irene Lara Corrales Dr. Perla Lansang Dr. Jonathon Maguire Dr. Michael McNeely Dr. Ma. Lourdes Palmero The Hospital for Sick Children 2007-08 ANNUAL REPORT 143 O’Connor D, Khan S, Vaughan J, Weishuhn K, Jefferies Tallett S, Lingard L, Leslie K, Pirie J, Jefferies A, Spero Friedman JN, Saunders NR: Canada’s Baby Care Book. A, Campbell D, Asztalos E, Feldman M, Rovet J, L, Schneider R, Hilliard R, Rosenfield J, Hellmann J, Robert Rose Publishing, Toronto, 2007. Westall C, Whyte H: Growth and nutrient intakes Mian M, Hurley J: Measuring educational workload. of human milk-fed premature infants provided with Medical Teacher 2008: 30(3): pp 296-301. Friedman JN: Technologic devices in the medically complex child. In: Comprehensive Pediatric Hospital extra energy and nutrients after hospital discharge. Pediatrics 2008: 121: pp 766-776. Wendtlandt K, Beck CE: A child with gastrointestinal Medicine (Zaoutis LB, Chiang VW, eds.). Elsevier bleeding. Paediatrics and Child Health 2007: 12: Science, Philadelphia, 2007. Seltz LB, Kanani R, Zamakhshary M, Chiu PPL: A pp 869-870. Landrigan C, Friedman JN: Patient safety and medical newborn with chylous ascites caused by intestinal malrotation associated with heterotaxia syndrome. BOOKS AND BOOK CHAPTERS errors. In: Comprehensive Pediatric Hospital Medicine Pediatric Surgical International 2008: 24: pp Adams SL, Mahant S: Introduction to the medically (Zaoutis LB, Chiang VW, eds.). Elsevier Science, 633-636. complex child. In: Comprehensive Pediatric Hospital Philadelphia, 2007. Medicine (Zaoutis LB, Chiang VW, eds.). Elsevier Inc, Serletis D, Bouffet E, Parkin P, Rutka J, Drake J, Shroff Weinstein M: Lower respiratory tract infections. Philadelphia, 2007. M: Massive plexiform neurofibromas in childhood: In: Comprehensive Pediatric Hospital Medicine natural history and management issues. Journal of Bernstein S: Systemic lupus erythematosus. In: (Zaoutis LB, Chiang VW, eds.). Elsevier Science, Neurosurgery 2007: 106(5 Suppl): pp 363-367. Comprehensive Pediatric Hospital Medicine (Zaoutis L, Philadelphia, 2007. Chiang V, eds.). Elsevier Inc., Philadelphia, 2007. RESEARCH FUNDING Taddio A, Manley J, Potash L, Ipp M, Sgro M, Shah V: Routine immunization practices: use of topical Friedman JF, Beck CE: Parenteral fluid therapy for An exclusively community-based clerkship in anaesthetics and oral analgesics. Pediatrics 2007: infants and children. In: Conn’s Current Therapy paediatrics compared with a paediatric academic 120: pp 630-631. 2008 (Rakel RE, Bope ET, eds.). Elsevier Inc., health science centre. Feldman M. Paediatric Philadelphia, 2008. Consultants Partnership’s Grant ($5,000 2007-2008) Dr. Adam Rapoport Dr. Leonard Barry Seltz Dr. Navneet Sharma Research Associates Deepa Dalal Spring Holter Vesna Jovcevska Amina Khambalia Kayi Li Noorulain Malik Magda Mekky Dr. Jyothi Sehgal Kathryn (Kate) Smith Vineet Syan 144 The Hospital for Sick Children 2007-08 ANNUAL REPORT Catherine Wong Ming Yang ADMINISTRATIVE STAFF Sarah Ahmad Tiziana Altobelli Josie Marcantonio Marg Mather Daffodil Morrison Kathryn Robertson Pauline Smith Jan White Donna Whitely A prospective study on the age of onset, incidence, CIHR Emerging Team in Childhood Obesity Research. Evaluation of the efficacy and safety of imiquimod 5% geographic distribution and risk factors associated McCrindle B, Parkin P, Pencharz P, Hamilton J, topical cream in plaque morphea: a prospective, with atopic dermatitis. Pope, E, Krafchik BR, Pelletier J. Anderson G, Birken C, Wells G, Riddell M, Hanley A, multiple baseline, open label pilot study. Pope E, Laxer Canadian Dermatology Foundation ($35,000 O’Connor D, Dettmer E, Bellisimo N, Langer J, Tein I. R, Doria A, Feldman B, Babyn P: 3M Inc/Graceway 1996-2007) Canadian Institutes of Health Research ($2,452,522 Pharmaceuticals Canada ($29,500 2005-2008) 2008-2013) Evaluation of the emergency department general A randomized, prospective, double-blind placebocontrolled clinical study assessing the efficacy Complementary medicine in Canadian paediatric internal medicine patient flow toolkit. Schull M, of topical tacrolimus 0.1% ointment and topical patients with atopic dermatitis: a cross-sectional Guttmann A. Ministry of Health and Long-Term Care clobetasol propionate 0.05% ointment in the survey. Weinstein M, Pope E, Redlick F. Canadian ($1,133,400 2007-2011) treatment of vitiligo. Ho N, Krafchik BR, Pope E, Dermatology Foundation ($10,000 2006-2008) Examining the impact of primary care reform in Greenberg S, Webster C, Weinstein M. Fujisawa Developing quality and safety indicators for Canada’s Ontario on access to and quality of care. Glazier R, emergency departments. Schull M, Guttmann A. Guttmann A. Canadian Institutes of Health Research Canadian Institutes of Health Research ($20,000 2008) ($833,400 2008-2011) Birken CS, Beck CE, McCrindle B, Parkin PC. Does the order in which vaccines are administered Genetic analysis of PHACE(S) association. An Paediatric Consultants Partnership’s Grant ($9,975 affect pain response? A randomized, double-blind, international registry based out of the University of clinical trial of Pentacel versus Prevnar. Ipp M, Taddio California, San Francisco. Frieden IJ, Pope E, Siegel D: Canada Inc. ($62,425 2004-2007) An office based intervention to improve media use in preschool children: a randomized controlled trial. 2007-2008) Career enhancement. Guttmann A Canadian Child A, Lear N, Goldbach M, Parkin PC. Sanofi Pasteur University of Southern California at San Francisco ($17,250 2006-2007) ($20,000 2006-2009) Health Clinician Scientist Program ($25,000 2007-2012) Emergency department rapid intravenous rehydration Image guided GJ-tube versus laparoscopic nissen (RIVR) for paediatric gastroenteritis: a randomized fundoplication with gastrostomy in neurologically controlled trial. Freedman SB, Parkin P, Willan AR, impaired children with gastroesophageal reflux: a Schuh S. Physicians’ Services Incorporated study examining health-related quality of life. Langer Foundation ($156,000 2006-2008) JC, Mahant S, Connolly B, Friedman JN, Nicholas D, Wales P, Ungar W, Pastor A. Ontario Federation for Cerebral Palsy ($29,097 2007-2009) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 145 Impact of oximetry on hospitalization in acute Low versus high fidelity simulation in neonatal Nutritional education and the prevention of iron bronchiolitis. Schuh S, Freedman S, Coates A, resuscitation training. Whyte H, Bismilla Z, Finan E, depletion in children nine months to two years: Parkin PC, Allen U, Willan A, Ungar W, Da Silva Z. McNamara P. Paediatric Consultants Partnership’s a randomized controlled trial. Parkin PC, Maguire J, Physicians’ Services Incorporated Foundation Grant ($5,000 2007-2008) Khambalia A, Jacobson S, Peer M. Danone Institute ($61,148 2006-2008) ($163,000 2007-2009) Magnesium for Sickle Cell Trial (MAST). Goldman R, International registry of paediatric cutaneous T-cell Mounstephen W, Kirby M, Friedman JN. Physicians’ Open label extension study to determine safety lymphoma: a longitudinal, prospective, observation Services Incorporated Foundation ($163,846 of etanercept in paediatric subjects with plaque study of cutaneous T-cell lymphoma in the paediatric 2005-2008) psoriasis. Pope E, Weinstein M. Amgen Inc. ($43,000 2006-2007) population. Pope E, Weinstein M, Weitzman S, Taylor G. Canadian Dermatology Foundation ($10,000 Measuring the quality of hospital care for children 2004-2009) and youth in Canada. Guttmann A, Matlow A, Moore C, Parshuram C, Stukel T, To T. Canadian Institutes NF1-associated malignancy. Lewis S, Parkin P. International registry of paediatric cutaneous T-cell of Health Research ($25,000 2007-2010) The Hospital for Sick Children Research Institute Palindromy at the NF1 locus as a risk factor for New Ideas Grant ($45,023 2006-2009) lymphoma: a longitudinal, prospective, observation study of cutaneous T-cell lymphoma in the paediatric Measuring the quality of hospital care for children population. Pope E. University of Toronto Dean’s and youth in Canada. Guttmann A. Ontario Ministry of Paediatric outcomes research. Parkin PC, Macarthur C. Fund New Staff Grants ($10,000 2004-2009) Health and Long-Term Care ($300,000 2007-2010) SickKids Foundation ($750,000 2008-2012) Is prolonged bottle feeding an independent risk for Measuring the quality of hospital care for children Pilot study of a lifestyle program in children receiving overweight in young children? Birken CS, Parkin PC, and youth in Canada. Guttmann A. Canadian treatment for acute lymphoblastic leukaemia (ALL). O’Connor D, Jacobson S, Peer M, Mekky M. University Institutes of Health Research ($75,000 2008-2011) Nathan P, Wright M, Collins L, Birken CS, Dettmer E. Modifiable risk factors for acute chest syndrome in Grant ($14,000 2008-2009) Paediatric Oncology Group of Ontario Seed Funding of Toronto Dean’s Fund New Staff ($9,972 2008-2013) Isotonic versus hypotonic IV maintenance fluid in children with sickle cell disease admitted to hospital children: a randomized controlled trial. Friedman JN., with a painful crisis. Birken CS, Khambalia A, Dekker Freedman S, Beck C, Geary D. Paediatric Consultants A, Pastor A, Lee M, Padavattan K, Parkin PC. Paediatric Partnership’s Grant ($9,956 2007-2008) Consultants Partnership’s Grant ($3,750 2007-2008) 146 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT The efficacy of trimethoprim in wound healing of Presentation, management and outcomes evaluation Teaching intubation to residents: standard mannequin of children with vascular tumours. (Vascular versus high fidelity simulation. Whyte H, Bismilla Z, patients with epidermolysis bullosa: a randomized Tumour Registry). Pope E, Mahant S, Blanchette V, Finan E, Jefferies A, LeBlanc V. Physicians’ Services double-blinded, placebo-controlled, cross-over pilot Weitzman S, Baruchel S, John P, Brandao L. Paediatric Incorporated Foundation ($17,000 2007-2008) study. Pope, E, Lara-Corrales I, Weinstein M, Parkin P, Sibbald G. Canadian Dermatology Foundation Consultants Partnership’s Grant ($9,900 2006-2009) The effectiveness of the paediatric sedation device: ($10,000 2007) Genetic determinants of influenza severity in children: pilot study for randomized controlled trial. Brown SC, A pre-pandemic community-based feasibility study. Schneeweiss S, McGrath PA, Pope E. National Treatment choices for children with typical acute Tran D, Beyene J, Bitnun A, Paterson A, Richardson S, Institutes of Health ($159,062 2005-2008) immune thrombocytopenic purpura: development of a decision aid. Beck C, Birken CS, Blanchette V, Ipp M. Canadian Institutes of Health Research ($99,919 2008-2009) The efficacy of trimethoprim in wound healing of Boydell K, Llewellyn-Thomas H, Parkin PC. University patients with epidermolysis bullosa: a randomized, of Toronto Dean’s Fund New Staff Competition ($10,000 2006-2011) Short- and long-term outcomes of complicated double-blinded, placebo-controlled, cross-over pilot paediatric pneumonia. Weinstein M, Cohen E, Dell S, study. Pope E, Lara-Corrales I, Weinstein M, Parkin P, Mahant S, Traubici J, Mekky M. University of Toronto Sibbald G. Dystrophic Epidermolysis Bullosa Dean’s Fund New Staff Grant ($10,000 2007-2008) Waiting with an emergency. Schull M, Guttmann A. Recessive Association of Canada (dEBra Canada) Canadian Health Services Research Foundation ($10,000 2006-2008) ($400,000 2007-2010) Sickle cell disease in children: osteomyelitis or vaso-occlusive crises? Berger E, Saunders N, Khambalia A, Friedman JN. SickKids Trainee Start-up Fund ($3,000 2007-2008) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 147 Division of Dr. Theo Moraes DIVISION HEAD Dr. Felix Ratjen FULL-TIME Dr. Allan L Coates Dr. Sharon Dell Dr. Hartmut Grasemann Dr. Indra Narang Dr. Melinda Solomon Dr. Padmaja Subbarao Dr. Neil B Sweezey PART-TIME Dr. Peter Bikangaga Dr. James MacFarlane Dr. Theo Moraes Dr. Charlotte D’E. Miller ADJUNCT SCIENTIST Dr. Hiran Selvadurai CROSS-APPOINTEES Dr. T. Douglas Bradley Dr. Theodore Marras THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT HONORARY FACULTY Dr. Dennis Gurwitz Dr. Elizabeth Tullis FELLOWS Dr. Dimas Mateos-Corral Dr. Harutai Kamalaporn Dr. Mustafa Osman Dr. Reshma Amin Dr. Sintra Phumethum Dr. Suhail Al-Saleh SUBSPECIALTY RESIDENTS Dr. Joanna Swinburne Dr. Tania Samanta Dr. Tara Allen ADMINISTRATIVE STAFF Tara Beattie Jennifer Chay Victoria Snell Division of Respiratory Medicine OVERVIEW As part of the Toronto Lung Transplantation Program, inflammation and infection in cystic fibrosis, effect of The Division of Respiratory Medicine continues to the division provides pre- and post-transplantation sex hormones in airway inflammation, defining the role provide comprehensive care for the full range of care for children with end-stage lung disease. The of novel genes in lung development, studies in aerosol paediatric pulmonary diseases such as asthma, cystic lung transplant program continues to be the largest therapy and exercise physiology. Dr. Subbarao has fibrosis (CF), interstitial lung fiseases, primary ciliary paediatric lung transplant program in the country. become the co-principal investigator of a large birth cohort study to elucidate the factors important for the dyskinesia (PCD), hereditary hemorrhagic telangiectasia (HHT), sleep-related disorders of breathing and complex The training program has independent Royal College development of asthma in early childhood that is respiratory care requiring ventilatory support, as well as of Physicians and Surgeons of Canada accreditation supported by CIHR and partners such as AllerGen. children with congenital malformations involving the and is the largest training program for paediatric respiratory tract. We provide consultation support for respiratory medicine in the country. It provides the full the hospital in all areas of paediatric respiratory range of training that is required for board certification NIH-funded childhood asthma management program disease. The division provides diagnostic services in paediatric pulmonology in the United States. As well continuation study (Dr. Grasemann, Dr. Subbarao). including flexible bronchoscopy. as offering training to Royal College of Physicians and There has also been a growing interest in assessing Over the last year, we have broadened our diagnostic The division also continues to be involved in the Surgeons of Canada eligible candidates, the division the pulmonary sequelae of sickle cell disease that are also trains physicians from abroad. currently being addressed in a number of research studies (Dr. Grasemann, Dr. Narang, Dr. Subbarao). spectrum of tests to assess diseases with airway inflammation, including testing of nitric oxide in Dr. Theo Moraes, who completed his PhD in Dr. Gregory Dr. Allan Coates and Laura Seed have been very active exhaled air as well as gas mixing techniques as more Downey’s laboratory and subsequently worked with on both the provincial and national level with the Ontario Lung Association and the Canadian Thoracic sensitive tests for obstructive airway diseases. The Dr. Tanja Watts, has joined our division as a clinician- addition of an infant pulmonary function laboratory scientist. His research is directed towards a better Society by promoting training of spirometry in children has broadened the potentials for diagnostic tools to understanding of processes driving lung inflammation, and acting in advisory roles for research studies using objectively assess lung disease in infants and young as well as immunological mechanisms involved in lung function testing with Statistics Canada. children, and is available for the assessment of both immunity against respiratory viruses. There is a broad inpatients and outpatients. The infant pulmonary spectrum of both basic science and clinical research function lab is the only laboratory in the country that bridges the laboratory to the clinic in many offering this service. We are currently in the process disease areas. The major research foci include clinical of closing the gap by developing pulmonary function and epidemiological studies in patients with asthma tests for preschool children as well. and cystic fibrosis, translational studies on airway THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 149 Dr. Dell is involved in an international program to Dr. Ratjen and the CF group are involved in multiple For a more complete description of our clinical activities, develop a new classification for interstitial lung studies in a leading role, including multicentre studies educational events and research interests, please visit diseases and a NIH-sponsored study on primary ciliary on azithromycin in Pseudomonas negative CF patients. the division of respiratory medicine website as well as dyskinesia. Dr. Dell, in collaboration with researchers This is the first Canada-U.S. study funded by the U.S. the website CF centre. from the University of California in Berkeley, is also CF foundation on the role of aspergillus infection in CF. leading a study examining the association between Funded by the Canadian CF foundation and Physicians’ PUBLICATIONS asthma and traffic-related air pollution in Toronto Services Incorporated Foundation and on the use of Al-Saleh S, Grasemann H, Cox P: Necrotizing pneumonia school-age children. Dr. Solomon is studying the hypertonic saline as an early intervention strategy complicated by early and late pneumatoceles. Canadian cognitive-behavioural aspects of teaching communication funded by the US CF foundation and the NIH. Dr. Respiratory Journal 2008: 15: pp 129-132. supported by an educational development grant from Grasemann is leading a series of studies to elucidate the Centre of Faculty Development. the role of arginase and NO deficiency in CF lung disease Amin R, Ratjen F: MRI: the next frontier for cystic sponsored by CIHR and the Canadian CF foundation. fibrosis clinicians? Expert Review of Respiratory In addition, members of the division are key partners of Medicine 2008: 2(2): pp 179-182. the CF team that has formed the Cystic Fibrosis Centre, The division is also involved in developing new highlighting the longstanding and ongoing excellence in outcomes measures to assess improvement in CF Belik J, Shehnaz D, Pan J, Grasemann H: both clinical care and research at SickKids in this area. lung disease within the BREATHE program, a study Developmental changes in arginase expression Newborn screening for cystic fibrosis was started this to develop CFTR corrector therapy (Dr. Ratjen). Other and activity in the lung. American Journal year and the CF team at SickKids was a driving force studies supported by research grants, as well as of Physiology–Lung, Cellular and Molecular Physiology 2008: 294: pp L498-L504. in getting this off the ground. Dr. Ratjen acts as a generous support by individuals such as the Irwin medical advisor to the screening group in Ottawa and family, are ongoing, in addition to industry sponsored a collaborative study with Dr. Durie as the principal studies. One is an 80-site study on denufosol, a P2Y Brenden C, Danziger-Isakov LA, Astor T, Aurora P, investigator will be started to evaluate the clinical receptor agonist that activates chloride conductance Bluemchen K, Boyer D, Conrad C, Eichler I, Elidemir O, course of patients with an unclear diagnosis within through alternative chloride channels (Dr. Ratjen, PI). Goldfarb S, Michaels MG, Mogayzel PJ, Mueller C, the Newborn Screening Program. The CF group is also involved in other clinical studies Parakinikas D, Oberkfell D, Solomon M, Boehler A: of drugs targeting the basic defect in CF. A close Variability in immunization guidelines in children collaboration exists with the adult CF program at St. before and after lung transplantation. Pediatric Michael’s Hospital led by Dr. Tullis, in both clinical Transplantation 2007: 11: pp 882-887. care and many areas of research, and with the Lung Transplant Team at Toronto General led by Dr. Keshavjee. 150 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Grasemann H, Gerard NP, De Sanctis GT: Ventilatory Coates AL, Green M, Leung K, Louca E, Tservistas M, Dell SD, Becker A, Chapman K, Foty R, Franssen E: Chan J, Ribeiro N, Charron M: The challenges of Are symptom-based criteria enough to define asthma responses to acute hypoxia in neurokinin-1 receptor quantitative measurement of lung deposition using control in children? A population-based cross- deficient mice. Respiratory Physiology and Neurobiology 2007: 159: pp 227-231. 99mTc-DTPA from delivery systems with very different sectional analysis comparing adult and pediatric delivery times. Journal of Aerosol Medicine 2007: asthma outcomes. Pediatric Pulmonology 2007: 20: pp 320-330. 42: pp 1117-1124. Lakeman P, Gille JJP, Dankert-Roelse JE, Heijerman Coates AL: Guiding aerosol deposition in the lung. Deutsch GH, Young LR, Deterding RR, Fan LL, Dell SD, Cornel MC, ten Kate LP: CFTR mutations in Turkish New England Journal of Medicine 2008: 358: Bean JA, Brody AS, Nogee LM, Trapnell BC, Langston and North African cystic fibrosis patients in Europe: HGM, Munck A, Iron A, Grasemann H, Schuster A, pp 304-305. C, and the Pathology Co-operative Group: Diffuse lung implications for screening. Genetic Testing 2008: disease in young children: application of a novel 12: pp 25-35. Coates AL, Green M, Leung K, Chan J, Ribeiro N, Louca classification scheme. American Journal of Respiratory E, Ratjen F, Charron M, Tservistas M, Keller M: Rapid and Critical Care Medicine 2007: 176: pp 1120-1128. Nikolaizik WH, Vietzke D, Ratjen F: A pilot study to compare tobramycin 80 mg injectable preparation pulmonary delivery of inhaled tobramycin for Pseudomonas infection in cystic fibrosis: a pilot project. Duong M, Subbarao P, Adelroth E, Otis J, Obminski G, with 300 mg solution for inhalation in cystic fibrosis Pediatric Pulmonology 2008: 43(8): pp 753-759. Strienich T, Inman M, Pedersen S, O’Byrne PM: patients. Canadian Respiratory Journal 2008: 15(5): Sputum eosinophils and the response of exercise- pp 259-262. Corral DM, Coates AL, Yau YC, Tellier R, Glass M, induced bronchoconstriction to corticosteroid in Jones SM, Waters VJ: Burkholderia pseudomallei asthma. Chest 2008: 133(2): pp 404-411. Narang I, Rosenthal M, Bush A: Reproducibility of cardioventilatory measurements using a respiratory infection in a cystic fibrosis patient from the Caribbean: a case report. Canadian Respiratory Grasemann C, Ratjen F, Reutershahn E, Vester U, mass spectrometer. Respiratory Physiolology & Journal 2008: 15(5): pp 237-239. Schnabel D, Grasemann H: Effect of growth hormone Neurobiology 2007: 157: pp 310-315. therapy on nitric oxide formation in patients with Davis SD Ratjen F: Editorial: Reduced lung function in cystic fibrosis. European Respiratory Journal 2008: Nemati B, Atmodjo W, Kaplan F, Sweezey NB: cystic fibrosis: a primary or secondary phenotype? 4: pp 815-821. Glucocorticoid receptor disruption delays structural American Journal of Respiratory and Critical Care maturation in the lungs of newborn mice. Pediatric Medicine 2008: 178(1): pp 2-3. Pulmonology 2008: 43(2): pp 125-133. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 151 Quinlan J, Kaplan F, Sweezey NB, Goodyer P: LGL1, Stanojevic S, Wade A, Stocks J, Hankinson J, Coates Waters V, Ratjen F: Combination antimicrobial a novel branching morphogen in developing kidney, AL, Pan H, Rosenthal M, Corey M, Lebecque P, Cole susceptibility testing for acute exacerbations in is induced by retinoic acid. American Journal of TJ: Reference ranges for spirometry across all ages: chronic infection of Pseudomonas aeruginosa in Physiology - Renal Physiology 2007: 293(4): a new approach. American Journal of Respiratory and cystic fibrosis. Cochrane Database Systematic pp F987-F993. Critical Care Medicine 2008: 177: pp 253-260. Reviews 2008: 3: CD006961. Ratjen F: Aerosol therapy in cystic fibrosis: Subbarao P, Balkovec S, Solomon M, Ratjen F: Pilot Wells G, Wilkes D, Schneiderman-Walker J, Elmi M, weighing the evidence. Pediatric Pulmonology study of safety and tolerability of inhaled hypertonic Tullis E, Ratjen F, Coates AL: Reliability and validity 2008: 43(S9): pp S3-S4. saline in infants with cystic fibrosis. Pediatric of the habitual activity estimation scale (HAES) in Pulmonology 2007: 42: pp 471-476. Current Opinion in Pulmonary Medicine 2007: 13(6): pp 541-546. Ratjen F: Recent advances in cystic fibrosis. Paediatric patients with cystic fibrosis. Pediatric Pulmonology 2007: 43: pp 345-353. Ratjen F: New pulmonary therapies for cystic fibrosis. To T, Dell S, Dick P, Cicutto L: The burden of illness experienced by young children associated with BOOKS AND BOOK CHAPTERS asthma: a population-based cohort study. Journal Narang I, Balfour-Lynn I: Cardiopulmonary function of Asthma 2008: 45(1): pp45-49. testing–paediatrics. In: Physiotherapy for Respiratory Respiratory Reviews 2008: 9(2): pp 144-148. and Cardiac Problems, 4th Edition (Pryor J, Prasad A, To T, Gershon A, Wang C, Dell S, Cicutto L: Persistence Ridder-Schaphorn S, Ratjen F, Hübbers A, Hüberte J, and remission in childhood asthma: a population-based Falk S, Küster P, Schuster A, Mellies U, Löwe B, asthma birth cohort study. Archives of Pediatrics and Reintjes R, Peters G, Kahl BC: Nasal staphylococcus Adolescent Medicine 2007: 161(12): pp 1197-1204. aureus carriage is not a risk factor for lower airway eds.). Churchill Livingstone, Oxford, UK, 2008. Ratjen F, Tullis E: Cystic fibrosis. In: Clinical Respiratory Medicine, 3rd Edition (Albert RK, Spiro SG, Jett JR, eds.). Mosby Elsevier, St. Louis, MO, 2008. infection in young cystic fibrosis patients. Journal of Clinical Microbiology 2007: 45(9): pp 2979-2984. Solomon M, Ross H, Delgado D. Pediatric lung transplantation. In: Lung Transplantation Manual (Chaparro Mutis C, Hutcheon M, Singer L, Keshavjee S. eds.). Elsevier, Toronto, 2007. 152 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT FUNDING CAMP-CS3: The Hospital for Sick Children Clinical Effect of increased L-arginine bioavailability on A multi-stage population based study to determine Centre for Study of a Childhood Asthma Management cystic fibrosis lung disease. Grasemann H, Ratjen F, the association between air pollution and asthma. Program - continuation study, part 3. Subbarao P, Pencharz P, Scott J. Canadian Cystic Fibrosis Dell S. Health Canada ($79,000 2007-2009) Grasemann H, Solomon M. John Hopkins University Foundation ($39,869 2007-2009) Centre for Clinical Trials/National Institutes of A pilot study to evaluate the tolerability of inhaled Effect of increased L-arginine bioavailability on Health National Heart, Lung and Blood Institute 7% hypertonic saline in infants with cystic fibrosis cost-reimbursement contract grant (US$370,633 Pseudomonas aeruginosa infection of the lung in (ISIS-001). Ratjen F. US CF Foundation ($10,878 2008-2011) cystic fibrosis. Grasemann H, Ratjen F, Pencharz P, Scott J. Canadian Institutes of Health Research 2007-2008) Canadian birth cohort: Indoor air and the development ($100,000 2007-2009) A randomised double-blind, placebo-controlled of asthma and allergy (CHILD study). Sears M, four-way cross over study comparing montelukast, Subbarao P. Canadian Institutes of Health Research Effect of Ontario’s universal influenza immunization inhaled budesonide and their combination to placebo ($12,000,000 2007-2013) program on influenza-related health-care outcomes. Characterization of factors and mechanisms affecting ($82,163 2007-2009) Manuel D, Dell S. Canadian Institutes of Health on exercise-induced bronchoconstriction. Grasemann H, Subbarao P, O’Bryne P. Merck-Frosst RCT ($207,580 2007-2008) Breathe program–a randomized-controlled trial of physical activity, muscular function and lung health in cystic fibrosis. Coates AL, Ratjen R. Canadian Cystic Effect of rhDNase of ventilation in homogeneity in Fibrosis Foundation ($180,000 2006-2008) patients with cystic fibrosis. Ratjen F, Subbarao P. Hoffman-La-Roche ($45,000 2007-2008) inhaled hypertonic saline (7%) to detect a treatment effect using the lung clearance index in CF patients Diagnosing cystic fibrosis in neonates and adults: with normal lung function. Ratjen F. Canadian Cystic where do we draw the line? Durie P, Tullis E, Ratjen F. Functional analyses of two novel, developmentally- Canadian Cystic Fibrosis Foundation ($360,000 regulated glucocorticoid responsive genes in fetal Fibrosis Foundation ($61,654 2007-09) 2008-2011) CAMP-CS2: The Hospital for Sick Children Clinical lung. Kaplan F, Sweezey N. Canadian Institutes of Health Research ($655,595 2004-2009) Centre for Study of a Childhood Asthma Management Program – continuation study, part 2. Grasemann H, Solomon M, Subbarao P. John Hopkins University Centre for Clinical Trials/National Institutes of Health/National Heart, Lung and Blood Institute ($631,133 2003-2007) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 153 Glucocorticoid receptor and glucocorticoid-responsive Modulation by sex hormones of inflammation and Multicentre, multi-national, randomized, placebo- genes in developing lung. Sweezey N, Kaplan F. susceptibility to Pseudomonas aeruginosa in cystic controlled trial of azithromycin in participants with Canadian Institutes of Health Research ($563,500 fibrosis airways. Sweezey N. Canadian Cystic Fibrosis cystic fibrosis, six to 18 years old, culture negative for 2004-2009) Foundation ($140,000 2007-2009) Pseudomonas aeruginosa. Ratjen F, Solomon M. Cystic Fibrosis Foundation Therapeutics Inc. Inhaled saline for infants study (ISIS-002). Davis S, Modulation by sex hormones of inflammation and Rosenfeld M, Ratjen F. US CF Foundation (continued susceptibility to Pseudomonas aeruginosa in cystic ($5,200,464 2006-2009) funding guaranteed for the complete study duration of fibrosis airways (salary support for technican). Pharmacogenomics of P2Y2 receptor activation. four years through CFF) (US$1,794,674 2007-2008) Sweezey N. The Lynne and Arnold Irwin Foundation Ratjen F. Inspire Inc. ($51, 030 2007-2009) ($40,000 2007-2009) Respiratory population-based outcomes network: Lab technician salary support. Grasemann H. The Lynne and Arnold Irwin Foundation ($25,000 2007-2008) Mucociliary Clearance Consortium, Rare Diseases studies and evaluations (RESPONSE). To T, Dell S. Centre. Site PI: Dell S (main PI: Knowles M, primary Canadian Institutes of Health ($150,000 2007-2009) site: Chapel Hill). National Institutes of Health Long-term outcomes after paediatric complicated ($76,958 2007-2008) and modulates NO synthesis in the cystic fibrosis lung. pneumonia. Cohen E, Dell S. University of Toronto Dean’s Fund ($10,000 2007-2009) Surfactant protein D controls inflammatory response Multicentre, multi-national, randomized, placebo- Grasemann H (David Douda, PhD student). Ontario controlled trial of azithromycin in participants with Student Opportunity Trust Fund/Restracomp ($60,000 2008-2010) Lung allograft rejection gene expression observational cystic fibrosis, six to 18 years old, culture negative study. Solomon M. XDx Expression Diagnostics for Pseudomonas aeruginosa (PI salary support). ($7,500 2005-2007) Ratjen F. Cystic Fibrosis Foundation Therapeutics Inc. (US$36,546 2007-2008) Mini-child lung function and biomarkers - program A–gene environment interactions. Subbarao P. AllerGen NCE Inc. ($215,000 2007-2009). 154 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT VX06-770-101: A phase 2a, randomized, double-blind, The cognitive-behavioural model for teaching Treatment of aspergillus fumigatus in patients with communication skills: a pilot project. Solomon M. cystic fibrosis: a randomized, double-blind, placebo- placebo controlled study of VX770 in patients with Educational Development Fund, University of Toronto controlled trail. Ratjen F, Aaron S. Canadian Cystic cystic fibrosis having g551d mutations to evaluate ($5,000 2007-2008) Fibrosis Foundation ($220,800 2007-2010) safety, phamacokinetics and biomarkers of CFTR activity. Accurso F, Durie P, Ratjen F, Tullis E. Vertex Pharmaceuticals, Inc. (US$30,000 2007-2008) The Hospital for Sick Children Centre for the Study Treatment of aspergillus fumigatus in patients with of Complex Childhood Diseases (CSCCD). Post M. cystic fibrosis: A randomized, double-blind, placebo- Canadian Foundation for Innovation ($20,571,919 controlled trail. Ratjen F, Aaron S. Physicians’ Young infants with acute recurrent wheezing: to puff 206-2011) Services Incorporated Foundation ($122,000 or not to puff? A randomized controlled trial. 2007-2009) Grasemann H, Schuh S, Coates A, Willan A, Ungar W, Training program in clinical nutrition. Pencharz P, Dick P. SickKids Foundation/Institute of Human Anderson H, Coates A, Darling P, O’Connor D, Archer Development, Child and Youth Health/Canadian M, Wolever T. Canadian Institutes of Health Institutes of Health Research ($130,000 2008-2010) ($1,590,000 2002-2008) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 155 Bonnie Cameron DivisionDr.of DIVISION HEAD Dr. Brian Feldman FULL-TIME Dr. Susanne Benseler Dr. Ronald Laxer Dr. Rayfel Schneider Dr. Shirley Tse Dr. Rae Yeung MAJOR PART-TIME Dr. Earl Silverman Dr. Lynn Spiegel PART-TIME Dr. Bonnie Cameron CROSS-APPOINTEES Dr. Vivian Bykerk Dr. Hans-Michael Dosch Dr. Paul Fortin CLINICAL FELLOWS Dr. Clare Hutchinson Dr. Linda Hiraki Dr. Roman Jurencak Dr. Sathish Kumar Dr. Lily Lim Dr. Marcela Pizana-Serna Dr. Heinrike Schmeling Dr. Elizabeth Stringer ADMINISTRATIVE STAFF Sandra Bergeron Shona Magee Roseanne Richard Division of Rheumatology OVERVIEW Singapore. Paediatric rheumatology graduates have Blankier S: First place winner, University of Toronto The Division of Rheumatology provides comprehensive done extremely well in obtaining academic placements Ogryzlo Research Day for his poster presentation, The care for children with rheumatic diseases. The across Canada and around the world and trainees role of atorvastatin in modulating pathogenesis in an ambulatory clinics have approximately 5,000 patient have been productive in the research arena. animal model of kawasaki disease, June 2008. well as at Bloorview Kids Rehab and the outreach Research foci include the development of trial design Cameron B: Claus Wirsig Humanitarian Award clinic in Sudbury. In addition to the general rheumatology for the study of rare diseases, outcome, prognosis and winner, 2008. clinics, there are unique multidisciplinary subspecialty treatment studies in juvenile arthritis, SLE, neonatal clinics for children with dermatomyositis, morphea, lupus, scleroderma, dermatomyositis and vasculitis. Hutchison C: First prize winner, University systemic JIA and systemic lupus erythematosus. New Basic research focuses on the immunopathogenesis of Toronto Ogryzlo Research Day for her podium clinics for children with central nervous system of Kawasaki disease using an animal model. visits per year at The Hospital for Sick Children, as presentation, Open label study for treatment of angiography-negative, biopsy-positive primary vasculitis and juvenile spondyloarthropathy have been initiated. The division makes extensive use of the In July 2007, Dr. Rayfel Schneider was promoted to medical day care unit, where children receive joint Associate Chair, Education, Department of Paediatrics injections under anaesthesia, therapeutic infusions, and continues to work part-time as a staff rheumatologist. Hutchinson C: Winner, American College of or undergo investigations which would otherwise Dr. Brian Feldman was promoted to Head of the Rheumatology Pediatric Rheumatology Fellow Award, require inpatient admission. An increasing number of Division of Rheumatology and Dr. Shirley Tse was in recognition of outstanding abstract and research children have benefited from treatment with biologic appointed Director of the Rheumatology Residency accomplishments. Keystone, CO, 2008. agents on the medical day care unit. Training Program. The Division of Rheumatology has the largest HONOURS AND AWARDS Canadian Network for Improved Outcomes in Systemic paediatric rheumatology fellowship training program Ardelean D: ACR REF/Abbott Medical and Lupus Erythematosus, 2008 (Declined). in North America and is fully accredited by the Royal Paediatric Resident Research Award, 2007 for College of Physicians and Surgeons of Canada. This her research on VEGFB expression in a mouse Hiraki L: Clinician Scientist Training Program (CSTP), academic year, our fellows originated from Canada, model of Kawasaki disease. SickKids, Toronto, 2008 (Declined) Benseler S: Clinical Recognition Award, Division Hiraki L: Frank Knox Memorial Fellowship to conduct of Paediatric Emergency Medicine, The Hospital postgraduate studies in clinical epidemiology at for Sick Children, 2007. Harvard University. CNS vasculitis in children, 2008. Hiraki L: CaNIOS Kirkland Scholarship Award, the Czech Republic, Germany, India, Mexico and THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 157 Hiraki L: Second place winner, University Schmeling H: Winner, German Society of Pediatric PUBLICATIONS of Toronto Ogryzlo Research Day for her poster Rheumatology (GKJR) Research Award at the Annual Abrahamyan L, Feldman BM, Johnson SR, Beyen J, presentation, Predicting macrophage activation Meeting in Neuss, Germany, for her project, Nailfold Shah PS: Quality of randomized clinical trials in juvenile syndrome (MAS) in paediatric systemic lupus capillary density is strongly associated over time idiopathic arthritis. Rheumatology 2008: 1: p 366. erythematosus (pSLE) parents at diagnosis, 2008. with muscle and skin disease activity in juvenile dermatomyositis (supervisor Dr. Brian Feldman), 2007. Akikusa JD, Feldman BM, Gross GJ, Silverman ED, Gladman Award for sustained and outstanding Silverman E: Ontario Lupus Association pulse methyprednisolone. Pediatrics 2007: 119(3): contributions to the University of Toronto Division HOPE Award, 2008. pp e778-e782. Schneider R: University of Toronto, 2007 Dafna Schneider R: Sinus bradycardia after intravenous of Rheumatology as Program Director, Paediatric Rheumatology Residency Training Program. Silverman E: Department of Paediatrics, Akikusa JD, Schneider R, Harvey EA, Hebert D, Nominated by Rheumatology Trainees, 2007. University of Toronto, The Hospital for Sick Children, Thorner PS, Laxer RM, Silverman ED: Clinical features Subspecialty Education Award for Excellence and outcome of pediatric Wegener’s granulomatosis. in Clinical Teaching, 2007-2008. Arthritis and Rheumatism 2007: 57(5): pp 837-844. pharmacogenetics of juvenile idiopathic arthritis and Stringer E: Completing her masters in clinical Avcin A, Singh-Grewal D, Silverman ED, Schneider R: juvenile dermatomyositis–The influence of single- epidemiology and has accepted a staff position Severe digital ischemia in a child with Wegener’s nucleotide polymorphisms in genes coding for at Dalhousie University, IWK Health Centre granulomatosis. Scandinavian Journal of metabolism pathway enzymes on the efficacy and in the Division of Paediatric Rheumatology, 2008. Rheumatology 2007: 36(1): pp 78-80. Yeung R: The Arthritis Society Investigator Award, Avcin T, Benseler SM, Tyrrell PN, Cucnik S, Silverman Schmeling H: Postgraduate Medical Education Research Award, the Chisholm Memorial Fellowship: toxicity of methotrexate, 2008. Schmeling H: Selected to participate in the Clinical 2007-2012. ED: A follow-up study of antiphospholipid antibodies Immunology Society (CIS) School in Systemic and associated neuropsychiatric manifestations in Autoimmune Diseases, Boston, 2008. 137 children with systemic lupus erythematosus. Arthritis Care and Research 2008: 59(2): pp 206-213. 158 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Avcin T, Silverman ED: Review: antiphospholipid Elbers J, Benseler SM: Central nervous system Hiraki LT, Benseler SM, Tyrrell PN, Hebert D, Harvey E, antibodies in pediatric systemic lupus erythematosus vasculitis in children. Current Opinion in Rheumatology Silverman ED: Clinical and laboratory characteristics and the antiphospholipid syndrome. Lupus 2007: 2008: 20(1): pp 47-54. and long-term outcome of pediatric systemic lupus erythematosus. Journal of Pediatrics 2008: 152(4): 16(8): pp 627-633. pp 550-556. Feldman BM, Aledort L, Bullinger M, Delaney FM, Benseler SM, Silverman ED: Neuropsychiatric Doria AS, Funk S, Giangrande P, Lundin B, Manco- involvement in pediatric systemic lupus Johnson M, Miners A, Scriba PC, Sricvastava A, Hiraki LT, Hamilton J, Silverman ED: Measuring erythematosus. Lupus 2007: 16(8): pp 564-571. Blanchette VS: The economics of haemophilia permanent damage in pediatric systemic lupus prophylaxis: governmental and insurer perspectives. erythematosus. Lupus 2007: 16(8): pp 657-662. Benseler SM, Silverman ED: systemic lupus Haemophilia 2007: 13(6): pp 1-5. Huber AM, Dugan ED, Lachenbruch PA, Feldman BM, erythematosus. Rheumatic Disease Clinics Perez MD, Zemel LS, Lindsley CB, Rennebohm RM, of North America 2007: 33(3): pp 471-498. Feldman BM, Funk S, Lundin B, Doria AS, Ljung R, Blanchette V: Musculoskeletal measurement tools Wallace CA, Passo MH, Reed AM, Bowyer SL, Ballinger Boros C, Silverman ED, Spence D, Blaser S: from the International Prophylaxis Study Group (IPSG). SH, Miller FW, Rider LG, Juvenile Dermatomyositis Hydrocephalus and macrocephaly: new manifestations Haemophilia 2008: 14(Suppl): pp 162-169. Disease Activity Collaborative Study Group: Preliminary Gong G, Barrera M, Beyene J, Bhat S, Carcao M, assessment tool in juvenile dermatomyositis. Lord S, Narayanan U, Sung L, Young N, Feldman BM: Arthritis and Rheumatism 2008: 59(2): pp 214-21. validation and clinical meaning of the cutaneous of neonatal lupus erythematosus. Arthritis and Rheumatism 2007: 57(2): pp 261-266. Brunner HI, Gladman DD, Ibanez D, Urowitz MD, The gap study (GapS) interview - developing a process Silverman ED: Difference in disease features to determine the meaning and determinants of quality Huber AM, Dugan EM, Lachenbruch PA, Feldman BM, between childhood-onset and adult systemic lupus of life in children with arthritis and rheumatic diseases. Perez MD, Zemel LS: The cutaneous assessment tool: erythematosus (SLE). Arthritis and Rheumatism Clinical and Experimental Rheumatology 2007: 25(3): development and reliability in juvenile idiopathic 2008: 58(2): pp 556-562. pp 486-493. inflammatory myopathy. Rheumatology 2007: 46(10): pp 1606-1611. Compeyrot-Lacassagne S, Tyrrell PN, Atenafu E, Doria Gong G, Young N, Dempster H, Porepa M, Feldman AS, Stephens D, Gilday D, Silverman ED: Prevalence BM: The quality of my life (QOML) questionnaire: Huber AM, Tomlinson GA, Koren G, Feldman BM: and etiology of low bone mineral density in juvenile The minimal clinically important difference (MCID) Amitriptyline to relieve pain in juvenile idiopthic systemic lupus erythematosus. Arthritis and for pediatric rheumatology. Journal of Rheumatology arthritis: a pilot study using Bayesian meta-analysis Rheumatism 2007: 56(6): pp 1966-1973. 2007: 34(3): pp 581-587. of multiple N-of-1 clinical trials. Journal of Rheumatology 2007: 34(5): pp 1125-1132. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 159 Ota S, Cron RQ, Schanberg LE, O’Neil K, Mellins ED, Jacob CO, Reiff A, Armstrong DL, Myones BL, Compeyrot-Lacassagne S, Rosenberg AM, Babyn P, Silverman ED, Klein-Gitelman M, McCurdy D, Wagner- Laxer RM: Pamidronate in the treatment of chronic Fuhlbrigge RC, Feldman BM: Research priorities in Weiner L, Nocton JJ, Solomon A, Zidovetzki R: non-infectious inflammatory lesions of the mandible pediatric rheumatology: the Childhood Arthritis and Identification of novel susceptibility genes in childhood- in children. Journal of Rheumatology 2007: 34(7): Rheumatology Research Alliance (CARRA) consensus. onset systemic lupus erythematosus using a uniquely pp 1585-1589. Pediatric Rheumatology 2008: 6(1): p 5. designed candidate gene pathway platform. Arthritis Manlhiot C, Liang C, Tran D, Bitrum A, Tyrrell PN, Ruperto N, Lovell DJ, Cuttica R, Wilkinson N, Woo P, Feldman BM: Assessment of an infectious disease Espada G, Wouters C, Silverman ED, Balogh Z, Johnson S, Feldman BM, Hawker G: Classification history preceeding juvenile dermatomyositis symptom Henrickson M, Apaz MT, Baildam E, Fasth A, Gerloni V, criteria for systemic scolerosis subsets. Journal onset. Rheumatology 2008: 47(4): pp 526-529. and Rheumatism 2007: 56(12): pp 4164-4173. Lahdenne P, Prieur AM, Ravelli A, Saurenmann RK, Gamir ML, Wulffraat N, Marodi L, Petty RE, Joos R, of Rheumatology 2007: 34: pp 1855-1863. Manlhiot C, Tyrrell P, Liang L, Atkinson A, Lau W, Zulian F, McCurdy D, Myones BL, Nagy K, Reuman P, Johnson SR, Goek O-N, Singh-Grewal D, Vlad SC, Feldman BM: Safety of intravenous immunoglobulin Szer I, Travers S, Beutler A, Keenan G, Clark J, Feldman BM, Felson DT: Classification criteria in in the treatment of juvenile dermatomyositis: adverse Visvanathan S, Fasanmade A, Raychaudhuri A, rheumatic diseases: a review of methodologic reactions are associated with immunoglobulin A Mendelsohn A, Martini A, Giannini EH, for the PRINTO properties. Arthritis and Rheumatism 2007: 57 7): content. Pediatrics 2008: 121(3): pp e626-30. and PRCSG: A randomized, placebo-controlled trial of Nicholson A, Berger K, Bohn R, Carcao M, Fischer K, polyarticular course juvenile rheumatoid arthritis. Lau A, Duong TT, Ito S, Yeung RSM: Matrix Gringeri A, Hoots K, Mantovani L, Schramm W, Van Arthritis and Rheumatism 2007: 56(9): pp 3096-3106. Metalloproteinase-9 activity leads to elastin Hout BA, Willan AR, Feldman BM: Recommendations breakdown in an animal model of Kawasaki disease. for reporting economic evaluations of haemophilia Saurenmann R, Rose JB, Tyrrell PN, Feldman B, Laxer prophylaxis: a normal group’s consensus statement RM, Schneider R, Silverman ED: Epidemiology of infliximab plus methotrexate for the treatment of pp 1119-1133. Arthritis and Rheumatism 2008: 58: 3: 854-63. on behalf of the Economics Expert Working Group juvenile idiopathic arthritis in a multi-ethnic cohort: Lau A, Rosenberg H, Duong TT, McCrindle BW, Yeung of the International Prophylaxis Study Group. ethnicity as a risk factor. Arthritis and Rheumatism RSM: Elastolytic matrix metalloproteinases and Haemophilia 2007: 14: pp 127-132. 2007: 56(6): pp 1974-1984. coronary outcome in children with Kawasaki disease. Pediatric Research 2007: 61(6): pp 710-715. 160 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Singh-Grewal D, Schneiderman-Walker J, Wright V, Sung L, Tomlinson GA, Greenberg ML, Koren G, Judd P, BOOKS AND BOOK CHAPTERS Bar-Or O, Beyene J, Selvadurai H, Cameron B, Laxer Ota S, Feldman BM: Serial controlled N-of-1 trials of Benseler SM: Kawasaki disease. In: Handbook of RM, Schneider R, Silverman ED, Spiegel L, Tse S, topical vitamin E as prophylaxis for chemotherapy- Pediatric Emergency Medicine (Lalani A, Schneeweiss LeBlanc C, Wong J, Stephens S, Feldman BM: Physical induced oral mucositis in paediatric patients. European S, eds.). Jones and Bartlett, Sudbury, MA, 2007. exercise and activity in kids (PEAK): a randomized Journal of Cancer 2007: 43(8): pp 1269-1275. controlled single blinded trial of the effects of vigorous Benseler SM: Koerperlische untersuchung. In: exercise training on physical function in children with Sung L, Tomlinson GA, Greenberg ML, Koren G, Judd P, Paediatrische Rheumatologie (Wagner N, Dannecker arthritis. Arthritis and Rheumatism 2007: 57(7): Ota S, Feldman BM: Validation of the oral mucositis GE Hrsg, eds.). Springer Medizin Verlag, Auflage, 2007. pp 1202-1210. assessment scale in pediatric cancer. Pediatric Blood and Cancer 2007: 49(2): pp 149-153. Benseler SM: Rheumatologic emergencies. In: Tyrrell P, Beyene J, Benseler S, Sarkissian T, Silverman Schneeweiss S, eds.). Jones and Bartlett, 2007. Stephens S, Singh-Grewal D, Bar-Or O, Beyene J, Cameron B, LeBlanc C, Schneider R, Schneiderman- Handbook of Pediatric Emergency Medicine (Lalani A, Walker J, Selvadurai H, Silverman E, Spiegel L, Tse S, ED: Predictors of lipid abnormalities in children with Wright V, Feldman BM: The reliability of exercise new onset systemic lupus erythmatosus. Journal Benseler SM: Seltene formen der vaskulitis. In: testing and functional activity questionnaires in of Rheumatology 2007: 34(10): pp 2112-2118. Paediatrische Rheumatologie (Wagner N, Dannecker children with juvenile arthritis. Arthritis and Rheumatism 2007: 57(8): pp 1446-1452. GE Hrsg, eds.). Springer Medizin Verlag, Auflage, 2007. Uziel Y, Feldman BM, Krafchik BR, Laxer RM, Yeung RSM: Increased serum levels of TGF>1 in children Benseler SM, Kuemmerle-Deschner J: Tagayashu Stinson J, Toomey P, Stevens B, Kagan S, Duffy C, with localized scleroderma. Pediatric Rheumatology arteritis. In: Paediatrische Rheumatologie ((Wagner N, Huber A, Malleson P, McGrath P, Yeung RSM, Feldman, 2007: 5: p 22. Dannecker GE Hrsg, eds.). Springer Medizin Verlag, B: Asking the experts: exploring the self-management Auflage, 2007. needs of adolescents with arthritis. Arthritis Care Yeung RSM: Lessons learned from an animal model and Research 2008: 59: pp 65-72. of Kawasaki disease. Clinical and Experimental Feldman BM, Ravelli A: Maintaining flexibility: joints Rheumatology 2007: 25: (1 Suppl 44): pp S69-S71. and other considerations. In: Myositis & You: A Guide Stringer E, Yeung RSM: Pathogenesis of Kawasaki to Juvenile Dermatomyositis for Patients, Families and disease: rhe central role of TNFλ. Future Yeung RSM: Clinical phenotype and coronary outcome Health-care Providers (Rider L, Pachman L, Miller F, Rheumatology 2008: 3: pp 69-77. in Kawasaki disease. The Lancet 2007: 369: pp 85-88. Bollar H, eds.). The Myositis Association, Washington, DC, 2007. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 161 Laxer RM: Rheumatic Disease Clinic of North America, Determinants of risks and outcomes in inflammatory Feasibility and impact of qigong as compared to Volume 33, Issue 3, Pediatric Rheumatology. Elsevier arthritis. Siminovitch K, Yeung RSM, Bernatsky S, aerobic exercise in the treatment of childhood chronic Saunders Co., Philadelphia, 2007. Bombardier C, Boillais L, MacPherson A, Oen K. musculoskeletal pain: a pilot randomized controlled Canadian Institutes of Health Research trial. Tse SML, Feldman BM, Fairbairn J, O’Brien C, Schneider R, Laxer RM: Systemic juvenile idiopathic ($2,000,000 2007-2012) Bar-Or O, Schneiderman-Walker J, Singh-Grewal D, Stephens S, Stinson J, Tyrell P, Whitney K, Wright V, arthritis. In: Handbook of Systemic Autoimmune Diseases (Cimaz R, Lehman T, eds.). Elsevier, Development and usability and feasibility testing of a Cameron B, Lefebvre A, Laxer R, Schneider R, Oxford, UK, 2008. web-based self-management program for adolescents Silverman ED, Spiegel L, Wu H, Bustamante K. with arthritis. McGrath PJ, Stinson J, Hodnett E, SickKids Foundation Complementary and Alternative Woo P, Laxer RM, Sherry DD. Pediatric Rheumatology Feldman B, Duffy C, Huber A, Tucker L, Hetherington Health Care and Paediatrics Research Grant in Clinical Practice. Springer-Verlag, London, 2007. R, Yeung R. Canadian Arthritis Network ($445,500 ($128,223 2005-2007) 2007-2009) FUNDING Feasibility of an interactive web-based rheumatology Biologically-based outcome predictors in juvenile Development and usability and feasibility testing of a teaching module for paediatric residents. Tse SM, idiopathic arthritis. Rosenberg A, Baxter-Jones AD, web-based self-management program for adolecents co-investigators: Feldman BM, Schneider R, Hyman A, Gordon J, Muhajarine N, Oen K, Pahwa P, Scherer S, with arthritis. Feldman B. Canadian Institutes of research team: Stringer E, Bassilious E, Batthish M, Turvey S, von Baever C, Whiting SJ, Yeung RSM, Health Research ($69,350 2007-2009) Bendiak G, Gupta S, Bradley N, Cameron T, Tait G, Shouldice M. Educational Dean’s Funds matched with Bernatsky S, Duffy C, Silverman ED. Canadian Institutes of Health Research/Canadian Arthritis Development of a feasible tool to measure oral Network ($1,500,000 2006-2011) mucositis in children with cancer. Sung L, Judd P, Department of Paediatrics Funds ($18,300 2007-2008) Tomlinson D, Doyle J, Hendershot E, Maloney AM, Inflammation and atherosclerosis in paediatric Childhood Arthritis and Rheumatology Research Feldman B. Pediatric Oncology Group of Ontario rheumatic diesase. Bradley T, Beyene J, Schneider R, Alliance. Sandborg C, Brewer E, Feldman B, Hirsch R, ($13,334 2007-2008) Feldman B, Silverman ED. Heart and Stroke Ilowite N, Jung L, Lovel D, Malleson P, Mellins E, Foundation of Ontario ($125,488 2007-2009) O’Neil K, Schanberg L, Wallace C. Arthritis Foundation ($525,000 2004-2007) International study: the efficacy of monoclonal anti-TNF antibody in JRA. Extension phase. Silverman ED (Site PI). Centocor Corporation ($110,000 2004-2010) 162 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT The role of chronic inflammation and systemic Kawasaki disease: on our way to unravelling the Pilot study of once-a-day prophylaxis for youth and pathogenesis. Yeung RSM. Canadian Institutes young adults with severe haemophila. Feldman BM, lupus erythematosus-related factors in the of Health Research ($318,873 2005-2008) Rivard G, Carcao M, St-Louis J, Teitel J, Pulleneyegum E, development of premature atherosclerosis in Blanchette V. Bayer Partnership Fund ($250,000 paediatric SLE. Silverman ED, Bargman J, New onset juvenile idiopathic arthritis: a Canadian 2007-2008) Bradley T, Beyene J. The Heart and Stroke Foundation of Canada ($275,000 2006-2009) multicentre collaborative outcome study. Yeung RSM, Duffy C, Oen K, Tucker L, Feldman B, Feldman D, Research to improve outcomes for inflammatory Young N, Spiegel L. Canadian Institutes of Health arthritis. Inman R, Gladman D, Maksymowych W, Towards a better understanding of Kawasaki disease. Research ($998,000 2004-2009) Rahman P (co-principal investigators), Davis A, Tsui F, Yeung RSM. The Heart and Stroke Foundation of Greenwood C, Badley E, Scherer S, Reeve J, Cook R Canada ($160,565 2006-2008) Novel biomarkers in inflammatory arthrits. Siminovitch K, (project co-investigators), Boonen A, Choquette D, Bernatsky S, Bombardier C, Boillais L, Yeung RS. Colbert R, Frank J, Ghahary A, Gignac M, Husted J, Canadian Arthritis Network ($150,000 2007-2009) Keen K, Lambert R, Laxer R, Marra C, Mosher D, heart block. Hamilton RH, Gross G, Silverman ED. Mulholland K, Nair B, Poole R, Richards C, Salanen D, Canadian Institutes of Health Research ($197,000 Pain in child health (PICH): an innovative, Shojania K, Thomson G, Tse S. The Arthritis Society, 2006-2009) transdisciplinary, cross-Canada, research training National Research Initiative ($2,500,000 2005-2010) Towards understanding the mechanism of congenital Validation of accelerometry as a measure of physical consortium. McGrath P, Craig K, von Baeyer C, Stevens B, Johnston C, Finley A (co-principal Rituximab therapy in refractory adult and juvenile IIM. activity and inactivity in children with chronic disease. investigators), Schellinck H, Unruh A, Kenny N, Oddis CV, Reed AM, Pestronk A, Rider L, Miller F, Feldman B, Stephens S, Takken T, Wright V, Tramblay Camfield C, Harmon K, Frager G, Rathwell T, Howlett A, Eisenberg R, Feldman BM, Ytterberg S, Levine T. M, Beyenne J, Biggar W, Esliger D, Longmuir P, Otley T, Connolly J, Feldman BM, Langer J, Taddio A, National Institutes of Health ($7,732,309 2004-2009) McCrindle B, Schneiderman-Walker J, Van Der Net J. Hetherington R, Grunau R, Oberlander T, Chambers C, Superantigens in autoimmunity. Yeung RSM. Canadian 2007-2008) Dube L, Ohlsson A, Murray M, Barr R, Koren G Institutes of Health Research ($172,553 2006-2008) Canadian Institutes of Health Research ($64,985 Baruchel S, Brown S, Gibbins S, Salter M, (co-investigators). Canadian Institutes of Health Training Grant ($300,000 2002-2008) THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 163 Dr. Golda Milo-Manson VICE PRESIDENT, MEDICINE & ACADEMIC AFFAIRS Dr. Golda Milo-Manson PHYSICIAN DIRECTORS Dr. Peter Rumney, Rehabilitation & Complex Continuing Care Dr. Darcy Fehlings, Child Development Program Dr. Rob Carmichael, Chief of Dental Services STAFF PHYSICIANS Dr. Doug Biggar Dr. Elizabeth Jimenez Dr. Tamarah Kagan-Kushnir Dr. Anne Kawamura Dr. Jeffrey Kobayashi 164 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Dr. Dr. Dr. Dr. Dr. Laura McAdam Gilbert Miller Angela Orsino Jonathan Tolkin Wendy Roberts Bloorview Kids Rehab OVERVIEW MEDICAL EDUCATION Developmental Paediatric Residency Program Director As a provincial facility, we see almost 7,000 children Year 2007 Dr. Golda Milo-Manson and youth on an outpatient basis and we have over Paediatric RII...................................... 380 days 650 patient admissions each year. Neurology........................................... 80 days Resident Coordinator Psychiatry .......................................... 30 days Dr. Anne Kawamura Bloorview Kids Rehab offers both inpatient and PM&R ................................................ 60 days outpatient developmental and rehabilitation services Medical students................................ 300 days Academic Accomplishments to the children and families of greater Toronto and Developmental paediatric see Division of Developmental Paediatrics Report. provides tertiary services in pediatric rehabilitation to subspecialty residents ........................ 980 days the Province of Ontario. TOTAL ................................................ 1830 days HIGHLIGHTS • April 2008 Royal College accreditation – full approval • Third Annual Roszel Developmental Paediatric Resident Research Day, February 2008 Resident Prize Winner: Dr. Kassia Johnson • Dr. Darcy Fehlings: Inaugural holder, Bloorview Children’s Hospital Foundation’s Chair in Developmental Paediatrics, 2007 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 165 Division of Dr. David Grant DIRECTOR Dr. David Grant TRANSPLANT PHYSICIANS Dr. Upton Allen Dr. Darius Bagli Dr. Dr. Dr. Dr. Dr. Dr. Mark Cattral John Coles Anne Dipchand Walid Farhat Annie Fecteau Hartmut Grasemann Dr. Paul Greig Dr. Elizabeth Harvey Dr. Diane Hebert Dr. Nicola Jones Dr. Paul Kantor Dr. Shaf Keshavjee Dr. Dr. Dr. Dr. Dr. Dr. Valerie Langlois Simon Ling Seema Mital Vicky Ng Marc de Perrot Andrew Pierre Dr. Dr. Dr. Dr. Dr. Dr. Lisa Robinson Pipi Salle Mindy Solomon Glen Van Arsdell Thomas Wadell Paul Wales ADMINISTRATOR Wanda Schoonheyt ADMINISTRATIVE SUPPORT Christine Garner Transplant Centre OVERVIEW Our clinical programs were busy during the past year. SickKids has one of the largest paediatric solid A total of 56 organs were transplanted in 52 children including the North American Paediatric Renal organ transplant programs in North America, with and our sphere of clinical activity expanded with the Transplant Co-operative Study Group, the Paediatric We contribute to international research groups, well over 1,000 children in receipt of a solid organ integration of the Group for the Improvement of Heart Transplant Study Group, NIH-funded fulminate graft. We deliver cutting-edge, family-centred patient Intestinal Function and Treatment (GIFT) into the liver failure study group, and the Paediatric Liver care with clinical volumes that are within the top Transplant Centre. To meet growing clinical needs Transplantation Registry. Local research projects with five-10 per cent of transplant centres in North America. and ensure that our patients receive care on the extramural funding include: vaccination strategies; Patient survival rates match or exceed the best appropriate unit, the hospital created surge capacity prevention of Epstein-Barr virus and Respiratory programs worldwide. Our clinical programs are on 6B in 2008 and expanded our interdisciplinary Syncytial virus complications post-transplantation enhanced by interdisciplinary educational programs; team to improve access to rehabilitation services, (Upton Allen); quality of life following heart and liver fellowship training in transplantation; and basic, psychology and transplant pharmacy expertise. transplantation (Anne Dipchand, Stacey Pollack,Vicky Ng); xenotransplantation (David Grant, Thomas translational and clinical research. We were also busy academically. Members of the Wadell); adolescent issues of transition to adult care Formerly known as the Paediatric Academic Multi- Transplant Centre served as advocates for paediatric (Miriam Kaufman); techniques to enhance Organ Transplant Program (PAMOT), we successful solid organ transplantation at the provincial, national lung recovery for transplantation (Shaf Keshavjee and transitioned to become the SickKids Transplant and international levels as members of the Board of colleagues); and leukocyte trafficking in allograft Centre in February 2008. A search for a new executive the International Paediatric Lung Transplant rejection (Lisa Robinson). Our trainees continued to team was completed in June 2008 with the Committee (Mindy Solomon); International Paediatric reflect the international appeal of our fellowship appointment of David Grant as Director; Anne Transplant Association (Anne Dipchand); Trillium Gift program with Dr. Meeta Malik (Chief Fellow 2007-08) Dipchand, Associate Clinical Director; Freda Miller, of Life (Diane Hebert); and as president of the becoming an international ambassador for SickKids Associate Research Director; and Wanda Schoonheyt, International Intestine Transplant Society (David as she assumed her faculty position in the UK. Director, Clinical Programs. Grant). Our basic and clinical research activities continued to focus on optimizing long-term transplant In 2008-09, the new Transplant Centre will focus outcomes in children. on fundraising, improving our educational offering building stronger links with families and referring doctors and enhancing our research programs. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 167 2007-08 Housestaff Paediatric Housestaff CHIEF RESIDENTS - PGY4S Dr. Nirit Bernhard Dr. Sharon Naymark ASSOCIATE CHIEF RESIDENTS - PGY4S Dr. Karoon Danayan Dr. Elizabeth Gold Dr. Krishanthi Gunawardena Dr. Mary Kiaira THIRD-YEAR RESIDENTS PGY3S Dr. Dalia Al-Abdulrazzaq Dr. Fatoumah Al-Abdulrazzaq Dr. Amal Al-Maani Dr. Sowmya Balasubramanian Dr. Ereny Bassilious Dr. Michelle Batthish Dr. Glenda Bendiak Dr. Nicholas Blanchette Dr. Michelle Bridge Dr. Karoon Danayan Dr. Sumit Gupta Dr. Joelene Huber Dr. Sangeet Kathuria Dr. Tarek Khalefih Dr. Melanie Makhija Dr. Shruti Mehrotra Dr. Briseida Mema Dr. Caroline McLaughlin Dr. Michelle Porepa Dr. Ewurabena Simpson Dr. Andrea Wan Dr. Kevin Weingarten Dr. Eitan Weinberg SECOND-YEAR RESIDENTS PGY2S Dr. Dr. Fahed Aljaser Dr. Ahmed Alterkait Dr. Kesete Bezu Dr. Joyce Ching) Dr. Avram Denburg Dr. Alisha Kassam Dr. Rae Koffman Dr. Dianne Lim Dr. Nadia Luca Dr. Arif Manji Dr. Gagandeep Saund Dr. Deena Savlov Dr. Ksenia Slywynska Dr. Gordon Soon Dr. Christopher Sulowski Dr. Mark Tessaro Dr. Nisha Thampi Dr. Alène Toulany FIRST-YEAR RESIDENTS PGY1S Dr. Leila Fazeli Chaghoshi Dr. Amy French Dr. Silvio Gonzalez Dr. Andrea Hoffman Dr. Emilie Jean-St-Michel Dr. Julie Johnstone Dr. Dina Kulik Dr. Ana Marissa LagmanBartolome Dr. Paola Luca Dr. Mary Kate Mennie Dr. Niraj Mistry Dr. Reem Mohammed Dr. Nikhil Pai Dr. Anastasios (Taso) Papadopoulos Dr. Cynthia Popalis Dr. Alisha Rachlis Dr. Audrey Segal Dr. Maher Shalabi Dr. Shawna Silver Dr. Jennifer Smith Dr. Anita Villani Dr. Cyrus Boelman (PGY1 neuro) Dr. John Brandsma (PGY1 neuro) PRACTICE READY ASSESSMENT (PRA) Dr. Mariana Boragina Paediatric Grand Rounds 2007 August 22 – Michaela Cada, Tony Truong, Division October 3 – Dr. Michael Geraghty, Head, Department July 4 – Zia Bismilla, Adam Rapoport, Barry Seltz, of Haematology/Oncology: Childhood cancer survivors of Genetics and Metabolism, Children’s Hospital of Division of Paediatric Medicine: Academic general and late effects: paying the price for cure Eastern Ontario: Expanded newborn screening in Ontario paediatric research: changing the way we practice July 11 – Fotini Kavadas, Elana Lavine, Division August 29 – April Kam, Chris Ricks, Division October 10 – Dr. David Goodman, Centre for Health- of Paediatric Emergency Medicine: Foul play care Research and Reform, the Dartmouth Institute for September 5 – Dr. Fraser Mustard, Head, Founder’s Harry Bain PSI Lecture: Are more paediatricians linked Network, Companion of the Order of Canada, Laureate to better health outcomes for children?” of Immunology/Allergy: Changes in ancient places: new directions in immunodeficiency Health Policy and Clinical Practice, New Hampshire: July 18 – Rachel Spitzer, Divison of Endocrinology - of the Canadian Medical Hall of Fame: Robert Haslam Adolescent Gynaecology: Meeting contraceptive challenges: Lecture: early child development and trajectories October 17 – Dr. Fiona Campbell, Anaesthesia, contraception for the medically complex adolescent in physical and mental health SickKids: Pain matters July 25 – Linda Hiraki, Heinrike Schmeling, September 12 – Dr. Phil Sherman, SickKids, October 24 – Dr. Joseph Flynn, Nephrology, Sathish Kumar, Division of Rheumatology: Division of Gastroenterology, Hepatology and Children’s Hospital of Montefiore: Ambulatory Childhood chronic arthritis: can we do better? Nutrition: Probiotics and gastrointestinal health: blood pressure monitoring in children and ready for prime time? adolescents: where are we in 2007? August 1 – Reshma Amin, Division of Respiratory Medicine: Cystic Fibrosis: from salt licks to heel pricks September 19 – Dr. Friedhelm Hildebrandt, Professor October 31 – Dr. Russell Ware, Chair, Department of Pediatrics and Human Genetics, Frederick Huetwell of Haematology, St. Jude Children’s Research Hospital, Professor for the Cure and Prevention of Birth Defects Memphis Bernard Laski Lecture: The rational use of Division of Gastroenterology, Hepatology and Nutrition: University of Michigan, Ann Arbor: Genetics of inherited hydroxyurea in sickle cell disease Is my baby yellow or is it the new compact fluorescent renal disease August 8 – Catharine Walsh and Eric Benchimol, November 7 – Dr. Norman Fost, Director, Program light bulb? An approach to the jaundiced infant September 26 – Michael Weinstein, David Chiasson, in Bioethics, Vice-Chair, Department of Medical August 15 – Jonathon Costello, Hadi Mohseni-Bod Greg Wilson, SickKids: Clinical Pathology Conference: History and Bioethics, University of Wisconsin Hospital: (Aphrothite Kotsakis), Critical Care Response Team: case review: a case of a child with fever Bioethics Lecture: Parental requests for ‘futile’ treatment Critical Care Response Team (CCRT) at The Hospital for Sick Children: evidence and practice 170 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT November 14 – Dr. Richard Aplenc, Pediatric 2008 February 20 – Dr. Noni MacDonald, former Dean of Oncology/Stem Cell Transplant Program, Children’s January 9 – Dr. Robert Hamilton, Division of Medicine, Dalhousie University, Halifax, Nova Scotia: Hospital of Philadelphia: Daniel Burton Lecture: Cardiology, SickKids: Clinical and research cardiac Sexually-transmitted infections and youth: reality bites Studies of genetic variation and paediatric leukaemia: genetics in high-risk hereditary heart conditions February 27 – Dr. Amitai Ziv, Director, Israel Center if you mix ice cream with cow manure, it won’t hurt the for Medical Simulation, Deputy Director, Chaim Sheba cow manure January 16 – Dr. David Fisman, Child Health Evaluative Sciences, SickKids Making best bets: how modelling Medical Center, Israel: Medical Education Day: Medical November 21 – Dr. Sylvain Chemtob, Pediatrics, can inform vaccine policy simulation changing the face of medical education Hôpital Ste. Justine, Université de Montréal: Barry Smith Lecture: Insights into retinopathy of prematurity: injury invites repair in the 21st century January 23 – Dr. Ben Alman, Head of Orthopaedic Surgery, SickKids: From fibromatosis to fractures: March 5 – Dr. Walter Kahr, Division of Haematology/ new therapies based on modulating beta-catenin Oncology, SickKids: New insights in cell biology November 28 – Dr. Sheldon Kaplan, Chief of Infectious from studies of inherited platelet disorders Disease, Texas Children’s Hospital: Community- January 30 – Dr. Charles Scriver, Alva Professor acquired methicillin resistant Staphylococcus Emeritus of Human Genetics, McGill University, aureus infections - they are everywhere December 5 – Dr. Bruce Gelb, Arthur J and Nellie Z March 19 – Dr. Robert Sundel, Rheumatology, Montreal Children’s Hospital Research Institute: Harvard University, Boston: Kawasaki disease Jacob’s Ladder Lecture: The journey but not the at 40 years old arrival yet. A model for ‘personalized medicine’? Cohen Professor of Pediatrics and Genetics and March 26 – Dr. Paul Kurtin, Vice-President and Chief Genomic Sciences, Mount Sinai Hospital, New York: February 6 – Dr. Jeffrey Whitsett, Chief of Quality and Safety Officer, Center for Child Health Noonan Syndrome and related disorders: RAS Neonatology, Perinatal & Pulmonary Biology, Outcomes, Children’s Hospital, San Diego: Improving Cincinnati Children’s Hospital: Genetic basis care for the children of Ontario signalling – getting it just right of normal adaptation to air breathing December 12 – Dr. Carole Marcus, Pediatric April 2 – Dr. Andrew Redington, Head, Division of Pulmonary Medicine, Joseph Stokes Jr. Research February 13 – Dr. Anne Matlow, Director, Infection Institute, Children’s Hospital of Pennsylvania: Prevention and Control Program and Medical Cardiology, SickKids: Remote ischemic preconditioning: harnessing an innate mechanism of cellular protection Childhood obstructive sleep apnea – implications Director, Patient Safety, SickKids Can we do against ischemic injury in adults and children for future health more to help achieve closure through disclosure? THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 171 April 9 – Dr. Michael Cabana, Director, Division April 30 – Paediatric Update, Dr. Harriet MacMillan, May 21 – Dr. David Hackam, Roberta Simmons of General Pediatrics,Professor of Pediatrics, McMaster University (Host: Dr. Suzan Schneeweiss): Associate Professor of Pediatric Surgery, Cell Biology Epidemiology and Biostatistics, UCSF: Physician Paediatric Update: An overview of family violence: and Physiology, University of Pittsburgh School of asthma care education: results of the PACE study implications for child development and approaches Medicine, Department of Pediatric General and to prevention Thoracic Surgery, Children’s Hospital of Pittsburgh April 16 – Dr. Kevin Chan, Division of Paediatric of UPMC A novel role for the innate immune system Emergency Medicine: Global child health research, May 7 – Dr. Vivien Burt, Professor of Psychiatry, in the pathogenesis and treatment of neonatal from Harvard to Toronto David Geffen School of Medicine, UCLA: Motherisk necrotizing enterocolitis Lecture: Mood disorders in women: focus on reproductive transitions May 28 – ANNUAL RESEARCH DAY building humanitarian projects May 14 – Dr. Lisa Shaffer, CEO and Technical Director, June 4 – Professor Linda DeMeirleir, Head of Pediatric Signature Genomic Laboratories, Spokane, WA: The Neurology, University of Belgium, Brussels Barsky Dr. Robert Hilliard, Division of Paediatric Medicine: use of array CGH in dDiagnosis and syndrome discovery Lecture: The clinical spectrum of mitochondrial Dr. Dennis Scolnik, Division of Paediatric Emergency Medicine: Starting small and diseases in young children and approach to diagnosis The changing scene of medical education in Africa Global Child Health Lecture: What SickKids is, May 21 – Dr. Suzanne Kamel-Reid, Senior Scientist, could and should be doing? Division of Applied Molecular Oncology, Ontario June 11 – Dr. Anne Matlow, Director, Infection Cancer Institute (OCI) Princess Margaret Hospital, Prevention and Control Program and Medical Director, April 23 – Dr. Jay Rosenfield, Vice-Dean, University Health Network: Andrew Sass-Kortsak Patient Safety, SickKids: Patient Safety Symposium Undergraduate Medical Education, University Memorial Lecture: Insights into the biology of acute of Toronto: The future of (undergraduate) medical promyelocytic leukaemia: the role of variant fusion proteins education in Canada 172 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT June 18 – ANNUAL AWARDS DAY Promotions/Recognition PROMOTED TO PROFESSOR OF PAEDIATRICS Dr. Debra Katzman THOSE WHO REACHED NORMAL RETIREMENT AGE The following reached normal PROMOTED TO ASSOCIATE PROFESSOR OF PAEDIATRICS retirement age in June of 2008: Dr. Manuel Carcao Dr. Milton Gold Dr. Jill Hamilton Dr. Andrew Shennan Dr. Hartley Garfield Dr. Meredith Irwin Dr. Kyong-Jin Lee 25 YEARS OF SERVICE AWARDS Dr. Golda Milo-Manson The following were honoured for their Dr. Hiroshi Otsubo 25 years of service to The Hospital Dr. Jose Luis Perez Velasquez for Sick Children in June of 2008: Dr. Jonathan Pirie Dr. Carlos Contreras Dr. Elena Pope Dr. Hyder Fazal Dr. Lisa Robinson Dr. Dennis Gurwitz Dr. Shelly Weiss Dr. Peggy Kirkpatrick Dr. Ken Schelberg Awards TEACHING/EDUCATION AWARDS Dr. Brian Chisamore – Postgraduate Community Dr. Leah Harrington – RHA Haslam Pre-Clerkship Teaching Award in recognition of excellence in the CLINICAL AWARDS Dr. Victor Blanchette – Richard Rowe Award for Teaching Award to the faculty member who has teaching of paediatric residents within community- Clinical Excellence in Paediatric Medical Care to the demonstrated excellence in pre-clerkshop teaching. based hospitals and offices. physician in the Department of Paediatrics who has demonstrated excellence in the delivery of clinical care Dr. Raymond Shu – The Lionel Weinstein Clerkship Dr. Shazhan Amed – PERLS (Paediatric Educational in the broadest sense over a significant period of time. Teaching Award to the faculty member who has Resident Lecture Series) Award to the faculty member demonstrated excellence in clerkship teaching. who has demonstrated commitment to excellence in Dr. Angelo Mikrogianakis – Junior Faculty Award for teaching at the paediatric academic sessions. Clinical Excellence in Paediatric Medical Care to a Teaching Award in recognition of excellence in Dr. Earl Silverman – Subspecialty Teaching Award appointment to an academic health sciences centre, the teaching of medical students within community- to the faculty member who has demonstrated who has demonstrated excellence in the delivery of commitment to excellence in teaching the clinical care. Dr. Eddy Lau – The Undergraduate Community based hospitals and offices. physican within the first five to 10 years of subspecialty residents and clinical fellows. RESEARCH AWARDS Dr. Eyal Cohen – Harry Bain Award to a full-time member of the Department of Paediatrics who Dr. Jonathan Pirie – Continuing Medical Education Dr. Lillian Sung – Junior Physician Researcher Award has demonstrated excellence in teaching as Award to the faculty member in recognition of to recognize outstanding achievements and progress nominated by core paediatric residents. excellence in course co-ordination in an event within the first six years after initial appointment as a sponsored by the Department of Paediatrics/ faculty member at an academic health sciences centre. Dr. Sheila Jacobson – Marvin Gerstein Award to a University of Toronto and/or long-term contribution to part-time member of the Department of Paediatrics Continuing Education in the Department of Paediatrics. PAEDIATRIC ADMINISTRATIVE AWARD who has demonstrated excellence in teaching as Annabel Sousa – In recognition of an administrative nominated by core paediatric residents. 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 2007. 174 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Resident and Fellow Awards and Achievements TEACHING/EDUCATION AWARDS RESEARCH AWARDS Dr. Briseida Mema, PGY3, Dr. Ewurabena Simpson, Core Paediatrics PGY3, Dr. Sharon Naymark, PGY4 – Christopher Ondaatje 1. Sumit Gupta Award to a senior resident for excellence in clinical 2. Anita Villani teaching as nominated by core paediatric residents. SENIOR TRAINEE – CLINICAL SCIENCE CANMEDS AWARD – SUBSPECIALTY FELLOW Dr. Leonard Barry Selz 1. Ghassan Abu Kuwaik 2. Haydi Al Wassia Tie 3. Amy Lee Chong CLINICAL AWARDS Tie 3. Daria Grafodatskaya Dr. Michelle Batthish – The Saunders Prize to a senior resident who has demonstrated SENIOR TRAINEE – BASIC SCIENCE excellence in clinical paediatrics. Tie 1. Aleixo Muise Tie 1. Adam Shlien 176 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 177 Annual Resident and Fellow Research Day (May 28, 2008) ORAL PRESENTATIONS Osman M, Respiratory Medicine Al-Jasmi F, Clinical and Metabolic Genetics Al-Harthy N, Paediatric Emergency Medicine Asthma morbidity: is it peanuts? Osman M, Computer-assisted teaching of mucopolysaccridosis The crying infant: which tests to choose? Foty R, To T, Atkinson A, Stieb D, Dell S by patient management problems Al-Jasmi F, Al-Harthy N, Freedman SB, Thull-Freedman J Moldovan L, Clarke JTR Tole S, Nephrology Alwassia H, Neonatology SLIT-2 inhibits neutrophil chemotaxis. Tole S, Neonatal outcomes in multiple gestations: Huang YW, Rui M, Yan M, Liu GY, Robinson LA does mode of conception have an impact? Alwassia H, Shah K, Shah P, Shah V Aljunaibi A, Endocrinology Factors affecting postsurgical blood sugar levels in children and youth with Type 1 diabetes mellitus Wine E, Gastroenterology, Hepatology and Nutrition (T1D) undergoing anaesthesia/surgery. Aljunaibi A, Osteopontin-null mice are protected from citrobacter Stephens D, Daneman D Appendino J, Neurology rodentium-induced colonic hyperplasia due to a reduction The impact of amplitude integrated in attaching-effacing lesions. Wine E, Shen-Tu G, Ngan AlKhater R, Neurology electroencephalography on neonatal B-Y, Licht C, Sodek J, Zohar R, Sherman PM Toward the identification of paediatric movement intensive care unit practice. Appendino JP, McNamara P, Keyzers M, Hahn C disorders genes. A family with a novel neurological POSTER PRESENTATIONS disorder. AlKhater RA, Rilstone J, Minassian BA Abosoudah I, Haematology/Oncology Bismilla Z, Paediatric Medicine WT1 expression and hemihypertrophy in congenital Al-Murshedi F, Clinical and Metabolic Genetics Evaluation of neonatal endotracheal intubation mesoblastic nephroma. Abosoudah I, Ngan B-Y, Elevated propionylcarnitine on newborn screening skill in a tertiary care NICU. Z Bismilla, E Finan, Grant R, Weitzman S and vitamin B12 levels. Al-Murshedi F, Al-Jasmi F, Jefferies A, Whyte H, LeBlanc V Crushell E, Feigenbaum A Abu Kuwaik G, Developmental Paediatrics Gupta S, Core Paediatrics Immunization uptake of siblings of children with Al-Qadhi S, Paediatric Emergency Medicine Incidence and predictors of treatment related Autism. Abu Kuwaik G, Green PE, Roberts W, A retrospective chart review of nail bed lacerations in mortality in paediatric acute leukaemia in El Salvador. Zwaigenbaum L, Bryson S, Brian J, Szatmari P, Smith I children presenting to ER. Al-Qadhi S, Ratnapalan S Gupta S, Bonilla M, Fuentes SL, Howard SC, Caniza M, Barr R, Greenberg ML, Ribeiro R, Sung L Akiyama T, Neurology Al-Rawas A, Haematology/Oncology Movie of ictal high-frequency oscillations and intracranial Treatment of primary brain sarcoma with Ifosphamide, video EEG: marching high-frequency power correlating Carboplatin, Etoposide (ICE) and focal radiation. with symptoms in Jacksonian seizures. Akiyama T Al-Rawas A, Bouffet E, Laperriere N, Bartels U 178 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Al-Saleh S, Respiratory Medicine Barnett C, Clinical and Metabolic Genetics Brandsema J, Neurology Induced sputum compared with expectorated Loeys-Dietz syndrome: in-vitro restoration of fibrillin The incidence of asymptomatic cerebral arteriopathies sputum to identify respiratory pathogens in children and elastin production after treatment with losartan in neurofibromatosis type 1: the need for MRI with cystic fibrosis. Al-Saleh S, Grasemann H, and dexamethasone. Barnett C, Hinek A, Bradley TJ, screening. Brandsema J, Rea D, Parkin P, Armstrong D, Dell S, Yau Y, Waters V, Martin S, Ratjen F Chitayat D Askalan R Al-Shanteer S, Neonatology Barton-Forbes M, Infectious Diseases Bronte-Tinkew D, Gastroenterology, Response times and severity of illness during the Post-transplantation Lymphoproliferative Disorder Hepatology and Nutrition transport of neonates: is there a relationship? (PTLD) in pediatric solid organ transplant recipients: Helicobacter pylori caga activates the signal Al-Shanteer S, Lee K-S, Tomlinson C, Whyte H A review of Hospital for Sick Children (SickKids) transducer and activator of transcription 3 (STAT3) PTLD Registry: 2000-2007. Barton-Forbes M, pathway in vitro and in vivo. Bronte-Tinkew DM, Amin R, Respiratory Medicine Melbourne T, Heurter H, Dipchand A, Hebert D, Ng V, Terebiznik M, Franco A, Ahn D, Mimuro H, Chronic infection with aspergillus fumigatus Solomon M, Fecteau A, Grant D, Ngan B, Allen UD Sasakawa C, Ropeleski MJ, Peek Jr. RM, Jones NL exacerbations in cystic fibrosis patients. Benchimol E, Gastroenterology, Hepatology and Nutrition Cain J, Nephrology Amin R, Corey M, Tullis E, Ratjen F Toxic megacolon in children with inflammatory bowel Lineage specific functions of sonic hedgehog disease: Clinical and radiographic characteristics. signalling during murine kidney development. Baaboud A, Emergency Medicine Benchimol EI, Turner D, Mann EH, Thomas KE, Cain JE, Nieuwenhuis E, Hui C-C, Rosenblum RD Timing of administration of antibiotic and Gomes T, McLernon RA, Griffiths AM is associated with higher rates of pulmonary Chan D, Neurology clinical outcome in febrile neutropenia with oncology disease. Baaboud A, Ratnapalan S Boelman C, Neurology The relationship between rapid oscillatory activity Cerebrovascular disease in meningitis: Safety and in premature and normal term neonatal EEG and Baker J, Haematology/Oncology efficacy of anticoagulation. Boelman C, Askalan R the effect of phenobarbitone. Chan D, Yamazaki M, Prognostic factors in advanced-stage paediatric Akiyama T, Chu B, Donner E, Otsubo H anaplastic large cell lymphoma (ALCL): analysis of Boskovic R, Clinical Pharmacology and Toxicology patients from two consecutive Pediatric Oncology Long-term outcomes of children prenatally exposed Crushell E, Clinical and Metabolic Genetics Group of Ontario (POGO) studies. Baker JM, to valproic acid and carbamazepine: meta-analysis. The Lebers hereditary optic neuropathy mutation Weitzman S, Breakey VR, Smith JN, Zagorski BM, Boskovic R, Banach R, Einarson T, Moretty M, Koren G T14484C can cause Leigh-like disease. Crushell E, Hutchison RE, Laver JH Robinson B, Blaser S, Murray S, Ainsworth PJ, Feigenbaum A. THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 179 Crystal M, Cardiology Fujimoto A, Neurology Go C, Neurology Coronary stents in children: a bridge to surgery. Total intravenous anaesthesia for MEG sediation, Effects of valproic acid withdrawal on seizures by Crystal MA, Hickey EJ, Lee K-J, Chaturvedi RR, part II. Fujimoto A, Ochi A, Sugiyama I, Akiyama T, analysis of scalp and intracranial video EEG. Go C et al Benson LN Otsubo H, Snead III OC Di Giovanni V, Nephrology Gana J, Gastroenterology, Hepatology and Nutrition The BMP receptor, ALK3, controls murine Non-invasive diagnosis of esophageal varices patients with duplication 17p11.2 syndrome. nephrogenesis. Di Giovanni V, Rosenblum N in children. Gana JC, Roberts E, Ng VL, Ling SC Goh E, Goobie S, Yoon G, Shago M, Sgro M, Goh E, Goobie S, Clinical and Metabolic Genetics Bilateral dysplastic kidneys as a feature in Chitayat D, Mendoza-Londono R Durbin A, Haematology/Oncology Garcia-Bournissen F, A JNK dependent switch determines the Clinical Pharmacology and Toxicology Gonska T, Gastroenterology, Hepatology and Nutrition oncogenic or tumour suppressive activity of ILK Comparison of cocaine levels in hair of children Development of a novel outcome parameter for in rhabdomyosarcoma. Durbin AD, Somers GR, and their parents: a tool to estimate environmental clinical trials in cystic fibrosis. Gonska T, Ip W, Forrester M, Hannigan GE, Malkin D exposure to drugs of abuse. Garcia-Bournissen F, Bear C, Quinton P, Durie P Nesterenko M, Koren G Grafodatskaya D, Clinical and Metabolic Genetics Elbers J, Neurology Small-vessel CNS vasculitis in childhood: clinical Gillis J, Clinical and Metabolic Genetics Search for epigastric changes using agilent CpG- and pathological correlation. Elbers J, Halliday W, A novel karyotype involving a pericentric X island microarrays in children with autism born after Hawkins C, Benseler S chromosome inversion and mosaicism for two assisted conception. Grafodatskaya D, Lou Y, Zhao C, cell lines with different 5p deletions, presenting Scherer S, Weksberg R Faghfoury H, Clinical and Metabolic Genetics as neonatal hyperammonemia. Gillis J, George A, Interstitial deletion of chromosome (10) Shago M, Antinucci D, Feigenbaum A, Rohrbach M Faghfoury H, Summers AM, Li C Guttman O, Gastroenterology, Hepatology and Nutrition Biliary atresia with associated structural (q23.32q24.1) in a mother and her two offspring. Glatstein M, Paediatric Emergency Medicine malformations: the Canadian Multicentre Study from Clinical and radiologic predictors of hyponatremia 1985-2002. Guttman OR, Schreiber RA, Barker CC, Ferreira J, Choufani S, Clinical and Metabolic Genetics in children hospitalized with pneumonia. Glatstein M, Ng VL, Roberts EA, the Canadian Paediatric DNA methylation profiling in placentas of intra-uterine Parkin P, Freedman S, Miller E Hepatology Research Group growth restricted fetuses and normal controls using agilent CPG island microarrays. Ferreira J, Grafodatskaya D, Choufani S, Weksberg R 180 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Hamud O, Neonatology Hutchinson C, Rheumatology Kim J, Nephrology Clinical presentation and management of Open label study for treatment of angiography- The role of suppressor of fused in cerebellar supraventricular tachycardia (SVT) in neonates. negative, biopsy-positive primary CNS vasculitis in development. Kim J, Gill P, Zhang M, Hui CC, Hamud O, Lee K-S children. Hutchinson C, Elbers J, Halliday W, Branson H, Rosenblum ND Laughlin S, Armstrong D, Hawkins C, Benseler SM Hawkes M, Infectious Diseases Klieger-Grossmann C, CD36 deficiency confers relative resistance Hutter D, Cardiology Clinical Pharmacology and Toxicolgy to mycobacterial infection. Hawkes M, Li X, The use of inhaled nitric oxide in the Critical Care Unit, The safety of quinolones in the first trimester Crockett M, Diassiti A, Liu J, Kain KC The Hospital for Sick Children, Toronto: a retrospective of pregnancy: a meta-analysis. Klieger-Grossmann C, review 1994-2006. Hutter D, Sambamoorthi S, Humpl T Boskovic R, Moretti M, Koren G Jain S, Clinical and Metabolic Genetics Koifman A, Clinical and Metabolic Genetics. Hiraki L, Rheumatology Predicting macrophage activation syndrome (MAS) in paediatric systemic lupus erythematosus (pSLE) Gender identity in males with 46XX congenital Diaphragmatic hernia – limb abnormalities patients at diagnosis. Hiraki LT, Tyrrell PN, adrenal hyperplasia. Jain S, Zucker K, Wherrett D syndrome (Froster syndrome). Koifman A, Shannon P, Kingdom J, Chitayat D Benseler SM, Abdelhaleem M, Silverman ED Johnson K, Developmental Paediatrics Huber J, Core Paediatrics Books for siblings of children with autism: children’s Koltin D, Endocrinology An investigation of body composition, metabolic rate assessments. Johnson K, McKeever P, Kawamura A Medication induced diabetes during induction and body mass in children and adults with Prader-Willi therapy in paediatric acute lymphoblastic leukaemia. syndrome enrolled in an obesity intervention program. Jurencak R, Rheumatology Huber J, Balko K, Berall G Expression of interleukin-17 in synovial fluid and peripheral blood in juvenile idiopathic arthritis. Kumar S, Rheumatology Hussey S, Gastroenterology, Hepatology and Nutrition Jurencak R, Duong TT, Chung L, Feldman BM, Yeung RSM B cell depletion for autoimmune thrombocytopenia Prospective study of major upper gastrointestinal hemorrhage in children. Hussey S, Kelleher K, Ling SC Koltin D, Sung L, Naqvi A, Urbach SL and autoimmune hemolytic anaemia in paediatric Kavadas F, Immunology and Allergy systemic lupus erythematosus. Kumar S, High-dose methylprednisolone is effective in Benseler S, Kirby-Allen M, Silverman E the management of acute GvHD. Kovadas FD, Somech R, Atkinson A, Grunebaum E, Roifman CM THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 181 Lara-Corrales I, Paediatric Medicine Maegawa G, Clinical and Metabolic Genetics Mittal A, Neonatology The efficacy of trimethoprim in wound healing Juvenile GM2 gangliosidosis: a model for investigation PDA categorization predicts post-ligation need for of patients with epidermolysis bullosa: a randomized, of small molecule therapies for lysosomal storage cardiotropes. Mittal A, Dos Santos S, Hellmann J, Lee K-S, McNamara PJ double blinded, placebo-controlled, cross-over pilot diseases. Maegawa GHB, Banwell B, Tropak M, study. Lara-Corrales I, Parkin P, Stephens D, Buttner J, Rigat B, Hawkins C, Ackerley C, Weinstein M, Sibbald G, Pope E Hayes J, Tifft C, Tlopak M, Sorge G, Blaser S, van Giersbergen P, Clarke JTR, Mahuran DJ Lau M, Neonatology The role of secondary septation during lung development. Lau M, Tanswell AK Lavine E, Immunology and Allergy Lee Chong A, Haematology/Oncology Maguire J, Paediatric Medicine Is iron deficiency anaemia an additive risk factor Muise A, Gastroenterology, Hepatology and Nutrition for stroke in children who have major risk factors Defects in E-cadherin are associated with its for stroke? Magurie J, deVeber G, Parkin P cytoplasmic accumulation and with NOD2 polymorphisms in Crohn’s disease patients. Makhani N, Neurology Muise AM, Walters T, Glowacka W, Griffiths AM, Safety and efficacy of cyclophosphamide for the Ngan B-Y, Lan H, Xu W, Silverberg MS, Rotin D treatment of paediatric multiple sclerosis. Makhani N Curative cures? Lee Chong A, Grant R, Ahmed B, Thomas K, Connolly B, Greenberg M Is perceived injury risk a barrier to physical activity participation in children? Mistry N, Birken CS, Parkin P CD8 lymphopenia and Omenn syndrome. Lavine E, Kavadas F, Atkinson AR, Grunebaum E, Roifman CM Mistry N, Core Paediatrics Nahum A, Immunology and Allergy Mallik M, Nephrology Chronic mucocutaneous candidiasis clinical and The neuronal guidance cue slit 2 impairs monocyte laboratory classification. Nahum A, Roifman CM Lewis P, Neonatology chemotaxis while preserving macrophage Effects of prolonged exposure to inhaled nitric oxide phagocytosis. Mallik M, Rui-Crow M, Yan H, Nashed A, Respiratory Medicine (INO) on established chronic hypoxic pulmonary Chaukos D, Robinson LA Pulmonary hypertension and sleep disordered Kantores C, Van Vliet T, McNamara PJ, Jankov RP Mateo P, Neonatology A Riekstins, J MacFarlane, T Humpl, I Narang Lim S, Rheumatology of abnormal neurological outcome in severe O’Gorman C, Endocrinology Osteopenia and osteoporosis already present hyperbilirubinemia. Mateo PC, Colantinio G, Appetite-related hormone levels in patients with in newly diagnosed juvenile systemic lupus Blaser S, Moore AM craniopharyngioma and hypothalamic obesity. hypertension in neonatal rats. Lewis P, Xu E, breathing in children. A Nashed, S Al-Saleh, Magnetic resonance imaging (MRI) in prediction erythematosus (JSLE) patients. Lim SH, O’Gorman CS, Simoneau-Roy J, Pencharz P, Tyrrell P, Benseler S, Silverman ED Adeli K, Hamilton J 182 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT Parkash S, Clinical and Metabolic Genetics Principi T, Paediatric Emergency Medicine Seltz LB, Paediatric Medicine Van Allen-Myhre syndrome: report of a new case What happens to oxygen saturation after discharge Do children with complex febrile seizures require with chondrodysplasia punctata. Parkash S, Keating S, in outpatients with acute bronchiolitis? Principi T, routine lumbar puncture to exclude bacterial or Kolomietz E, Toi A, Chitayat D Coates A, Parkin P, Da Silva Z, Schuh S herpes simples virus (HSV) meningitis/encephalitis? Seltz LB, Cohen E, Weinstein M Parker M, Paediatric Emergency Medicine Raju D, Gastroenterology Predictors of major intervention in infants with Expression of fractalkine (CX3CL1) and its bronchiolitis. Parker MJ, Allen UD, Lalani A, involvement in helicobacter pylori infection. Prevalence of microcytic anaemia in children 12 to 38 Stephens D, Schuh S Raju D, Terebiznik M, Tole S, Robinson L, Jones N months of age assessed by suspected child abuse Pasic I, Clinical and Metabolic Genetics Rimon Babad A, Paediatric Emergency Medicine Contribution of DNA copy-number variation (CNV) Paediatric omental infarction. Rimon Babad A, to cancer susceptibility and large-scale genome Gerstle JT, Daneman A, Ratnapalan S Sharma N, Paediatric Medicine and neglect team at The Hospital for Sick Children. alterations in osteosarcoma (OS). Pasic I, Shlien A, Sharma N, Shouldice M, Mekky M, Parkin P Sherlock M, Gastroenterology, Hepatology and Nutrition Infliximab maintenance therapy in paediatric Novokmet A, Pinto D, Scherer SW, Malkin D Scheinemann K, Haematology/Oncology Crohn’s disease: the impact of discontinuation High morbidity but low mortality in childhood spinal of concomitant immunomodulation. Sherlock M, Phongsamart W, Infectious Diseases cord low grade gliomas. Scheinemann K, Bartels U, Benchimol EI, Walters T, Turner D, Zachos M, Griffiths AM Cytomegalovirus in paediatric haematopoietic stem Huang A, Hawkins C, Bouffet E, Tabori U cell transplantation at The Hospital for Sick Children, Shlien A, Haematology/Oncology 2003-2007. Phongsamart W, Doyle J, Gasses A, Schmeling H, Rheumatology Excessive germline copy number variations in the Tellier R, Grunebaum E, Allen U Nail fold capillary density is strongly associated over Li-Fraumeni cancer predisposition disorder. Shlien A, time with muscle and skin disease activity in juvenile Tabori U, Pienkowska M, Marshall CR, Nanda S, Porepa M, Core Paediatrics dermatomyositis. Schmeling H, Stephens S, Goia C, Druker H, Novokmet A, Feuk L, Scherer SW, Malkin D Evaluating the impact of a child-driven health Manlhiot C, Schneider R, Luthra S, Feldman BM magazine with children from an urban, multicultural, Smile S, Developmental Paediatrics low-income elementary school. Porepa M, Huber JF, Schneiderman-Walker J, Respiratory Medicine Randomized control study of melatonin for Lam C, Mekky M, Au H, Birken CS Assessment of body composition in paediatric children with autism and sleep difficulties. patients with cystic fibrosis. Schneiderman-Walker J, Smile S, Roberts W, Weiss S Heale L, Wells GD, Wilkes DL, Ratjen F, Coates AL THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 183 So J, Clinical and Metabolic Genetics Tanaka T, Clinical Pharmacology and Toxicology Wells G, Respiratory Medicine The CYLD tumour suppressor sensitizes cells The incidence of poor neonatal adaptation syndrome Skeletal muscle abnormalities in cystic fibrosis. to microtubule destabilization. So J, Huber M, following exposure to venlafaxine in late pregnancy. Wells GD, Wilkes DL, Schneiderman-Walker J Kohler A, Krauss S, Schweiger S, Schneider R Tanaka T, Choi J, Einarson A, Koren G, Ito S Rayner T, Elmi M, Selvadurai H, Ratjen F, Noseworthy M, Tein I, Coates A Soylu H, Neonatology Truong T, Haematology/Oncology Rupture of membranes before the age of viability Treatment-related complications in a young girl Werner K, Neurology increases adverse outcomes for preterm infants: with acute lymphoblastic leukaemia may be due to A MELAS-like phenotype presenting with ND3 a case control study. Soylu H, Diambomba Y, uncovered mutations in the PKD1 and MTHFR genes. mutations and Rolandic encephalopathy. Werner K Jefferies A, Shah PS Truong TH, Nanda S, Meyn MS, Grant R Sugiyama I, Neurology Villani A, Core Paediatrics Williams V, Neonatology Withdrawal of artificial hydration and nutrition Synthetic aperture magnetometry-kurtosis Inherent diagnostic and treatment challenges in in the NICU: A humane practice in tragic situations. for presurgical evaluation of tuberous germinoma of the basal ganglia: a case report and Williams C, Ives-Baine L, Shah P, Hellmann J sclerosis complex. Sugiyama I review of the literature. Villani A, Bouffet E, Blaser S, Millar B-A, Hawkins C, Bartels U Yaghoobi M, Clinical and Metabolic Genetics Nuclear factor (erythroid 2-related) protein 2 Walsh C, Gastroenterology, Hepatology and Nutrition of gastric cancer: a meta-analysis. Yaghoobi M, counteracts bile acid-induced hepatotoxicity: Simulation training in gastrointestinal endoscopy: Bijarchi R involvement of ABC transporters. Tan KP, low versus high fidelity. Walsh CM, Cooper MA, Wood GA, Yang M, Kosuge K, Ito S Rabeneck L, Carnahan H Tan M, Neurology Waters A, Nephrology loss in 60% oxygen-induced neonatal rat lung injury. Alleviation of cerebral venous obstruction in supine Slit diaphragm defects precede proteinuria Yi M, Masood A, Jankov R, Tanswell AK Tan KP, Clinical Pharmacology and Toxicology Impact of family clustering on the risk of development Yi M, Neonatology Inhibition of neutrophil influx protects against vessel lying neonates with use of a custom-designed pillow. in podocyte-specific notch-ic transgenic mice. Tan M, Kirton A, Miller E, Shroff M, Deverber G Waters A, Wu M, Onay T, Piscione T 184 THE HOSPITAL FOR SICK CHILDREN DEPARTMENT OF PAEDIATRICS 2007-08 ANNUAL REPORT 555 University Avenue Toronto, ON, Canada M5G 1X8 www.sickkids.ca Phone: 416.813.6122 For electronic versions of Department of Paediatrics’ annual reports, please see www.sickkids.ca/paediatrics
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