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About Sickkids
About SickKids

Peter Durie, MD, FRCP(C)

The Hospital for Sick Children
Gastroenterologist
Gastroenterology, Hepatology and Nutrition

Research Institute
Senior Scientist Emeritus
Translational Medicine

University of Toronto
Professor
Paediatrics


Phone: 416-813-6185
Fax: 416-813-6531
Email: peter.durie@sickkids.ca

Brief Biography

Peter Durie is a Professor, Department of Paediatrics, and Member of the Institute of Medical Sciences, Faculty of Medicine, University of Toronto. He is a Staff Gastroenterologist, the Division of Gastroenterology and Nutrition, the Department of Paediatrics, and Senior Scientist, Program in Physiology & Experimental Medicine, the Research Institute, The Hospital for Sick Children. Dr. Durie is also Director of Cystic Fibrosis Research, which is the largest disease based research program in the Research Institute.

Dr. Durie is an expert in the diagnosis and care of children with disorders of the exocrine pancreas. He has maintained a focused research interest in the genetic and pathophysiologic basis of inherited disorders of the exocrine pancreas including cystic fibrosis, and Shwachman Diamond syndrome. During more than 20 years, his laboratory has been continuously funded, from highly competitive extramural sources including the National Institutes of Health, The Canadian Institutes of Health Research and the Canadian Cystic Fibrosis Foundation.

Research Interests

  • Pancreatic Disorders
  • Cystic Fibrosis

Research Activities 

Our studies focus on understanding the pathogenesis of exocrine pancreatic diseases in infancy and childhood. Much of this work focuses on cystic fibrosis, the most common inherited cause of exocrine pancreatic disease. One goal of our current studies is to understand the clinical, biological and functional consequences of mutations in the cystic fibrosis transmembrane conductance regulator (CFTR), the gene defective in cystic fibrosis. A second goal is to understand the relationship between the functional classes of CFTR-gene mutations and the expression of the basic defect in vivo. In a collaborative effort, we are evaluating the genetic basis of Schwachman-Diamond syndrome, the second most common genetic cause of exocrine pancreatic dysfunction in childhood.

External Funding

Cystic Fibrosis Foundation Therapeutics, Inc. (CFFT) 2007/01-2007/12
Principal Investigator (PR Durie)

Title of project: Clinical Research Facilitation Award: Understanding CFTR folding, trafficking and membrane stability and applying this knowledge towards development of drug discovery assays.

Shwachman Diamond Syndrome Foundation 2006/11-2007/10
Co-PI with Dr. Johanna Rommens, Dr. Akiko Shimamura
Title of project: SBDS Protein Expression in Peripheral Blood Leukocytes

Genome Canada 2005-2009
Co-Leader (with Julian Zielenski)
Title of project: The contribution of genetic modulators of disease severity in cystic fibrosis to other diseases with similarities of clinical phenotype

Canadian Cystic Fibrosis Foundation 2005/04/01–2008/03/31
Principal Investigator(PR Durie)
Title of project: Determining the Threshold of CFTR Function that Causes CF Disease

National Institutes of Health – 1 R01 HL079573-01 2004-2009
Co-Investigator (Principal Investigator: Johanna Rommens
Title of Project: Molecular Genetics, SBDS Function and Deficiency in SDS

Canadian Cystic Fibrosis Foundation - BREATHE Program 2004-2009
Co-Applicant (Director: Christine Bear)
Title of project: Targeted Small Molecule Therapies for Cystic Fibrosis

Publications

Kalnins D, Durie P, Pencharz P. Nutritional management of cystic fibrosis patients. Curr Opin Clin Nutr Metab Care 10(3):348-354, 2007.

Sweezey NB, Smith D, Corey M, Ellis L, Carpenter S, Tullis DE, Durie P, O’Brodovich H. Amiloride-insensitive nasal potential difference varies with the menstrual cycle in cystic fibrosis. Pediatric Pulmonology, 42:519-524, 2007.

Ho W, Cheretakis C, Durie P, Kulkarni G, Glogauer M. Prevalence of oral diseases in Shwachman-Diamond syndrome. Spec Care Dentist 27(2):52-58, 2007.

Beharry SA, Ackerely C, Corey M, Kent G, Heng Y-M, Christensen H, Luk C, Yantiss RK, Nasser IA, Zaman M, Freedman SD, Durie PR Long-term docosahexaenoic acid therapy in a congenic murine model of cystic fibrosis. Am J Physiol Gastrointest Liver Physiol 292:839-848, 2007.

Wilschanski M, Dupuis A, Ellis L, Jarvi K, Zielenski J, Tullis E, Martin S, Corey M, Tsui L-C, Durie P. Mutations in the cystic fibrosis transmembrane regulator gene and in vivo transepithelial potentials. Am J Respir Crit Care Med 174:787-794, 2006.

Cantin AM, Hanrahan JW, Bilodeau, G, Ellis L, Dupuis A, Liao J, Zielenski J, Durie P. Cystic fibrosis transmembrane conductance regulator function is suppressed in cigarette smokers. Am J Respir Crit Care Med 173:1139-1144, 2006.

Kalnins D, Ellis L, Corey M, Pencharz PB, Stewart C, Tullis E, Durie, PR. Enteric-coated pancreatic enzyme with bicarbonate is equal to standard enteric-coated enzyme in treating malabsorption in cystic fibrosis. J Pediatr Gastroenterol Nutr 42:256-261, 2006

Schibli S, Corey M, Gaskin KJ, Ellis L, Durie PR. Towards the ideal quantitative pancreatic function test: Analysis of test variables that influence validity. Clin Gastroenterol Hepatol 4:90-97, 2006.

Drumm ML, Konstan MW, Schluchter MD, Handler A, Pace R, Zou F, Zariwala M, Fargo D, Xu A, Dunn JM, Darrah RJ, Dorfman R, Sandford AJ, Corey M, Zielenski J, Durie P, Goddard K, Yankaskas JR, Wright FA, Knowles M, for the Gene Modifier Study Group. Genetic modifiers of lung disease in cystic fibrosis. N Engl J Med. 353(14):1443-1453, 2005.

publ10: Bishop MD, Freedman SD, Zielenski J, Ahmed N, Dupuis A, Martin S, Ellis L, Shea J, Hopper I, Corey M, Kortan P, Haber G, Ross C, Tzountzouris J, Ray PN, Tsui L-C, Durie, PR. The cystic fibrosis transmembrane conductance regulator gene and ion channel function in patients with idiopathic pancreatitis. Human Genetics 118:372-381, 2005.

Zenker M, Mayerle J, Lerch MM, Tagariello A, Zerres K, Zerres K, Durie PR, Beier M, Hulskamp G, Guzman C, Rehder H, Beemer FA, Hamel B, Valieferinghen P, Gershoni-Baruch R, Vieira MW, Dumic M, Auslender R, Fil-da-Silva-Lopes V, Steinlicht S, Rauh M, Salev SA, Thiel C, Winterpacht A, Kwon YT, Varshavsky A, Reis A. Deficiency of UBTR1, a ubiquitin ligase of the N-end rule pathway, causes pancreatic dysfunction, malformations and mental retardation (Johanson-blizzard syndrome). Nature Genetics 37:1345-1350, 2005.

Borowitz D, Durie PR, Clarke LL, Werlin SL, Taylor CJ, Semler J, De Lisle RC, Lewindon P, Lichtman SM, Sinaasappel M, Baker RD, Baker SS, Verkade HJ, Lowe ME, Stallings VA, Janghorbani M, Butler R, Heubi J. Gastrointestinal outcomes and confounders in cystic fibrosis. J Pediatr Gastroenterol Nutr 41:273-285, 2005.

Mei Zahav M, Durie P, Zielenski J, Solomon M, Tullis E, Tsui L-C, Corey M. The prevalence and clinical characteristics of cystic fibrosis in South Asian Canadian immigrants. Arch Dis Child 90:675-679; 2005.

Shammas C, Menne TF, Hilcenko C, Michell SR, Goyenechea B, Boocock GRB, Durie PR, Rommens JM, Warren AJ. Structural and mutational analysis of the SBDS protein family: insigight into the leukemia-associated Shwachman-Diamond syndrome. J Biol Chem, 280:19221-19229, 2005.

Stephenson A, Brotherwood M, Robert R, Durie P, Verjee Z, Chaparro C, Corey M, Tullis E. Increased Vitamin A and E levels in adult cystic fibrosis patients after lung transplantation. Transplantation 78(5):613-615, 2005.