The core will provide an overall structure for maintaining and connecting all the available patient data related to mutations in the CFTR gene, and will provide experienced and integrated statistical services to project investigators whose data requires more than routine analysis. Core activities will include: continued development of the Toronto CF Database to further integrate clinical and scientific data on CF patients; support for the development of a database on male infertility patients who comprise the largest identified group of an expanded spectrum of patients affected by mutations in the CFTR gene; creation of data files and analytic strategies to study other atypical phenotypic groups related to CFTR, such as young patients with pancreatitis and adults with chronic lung disease; provision of clinical profiles for individual patients and patient groups to enhance the planning or interpretation of scientific experiments; statistical analysis of core and project data; and timely statistical consulting for the design and interpretation of experiments. The core facility is easily accessible to all investigators and has in place a computer network with state-of- the-art database and statistical software. Core support will ensure that scientists have access to the most appropriate patient information and material to design experiments, and that research hypotheses and results are related as immediately and precisely as possible to patient subgroups, to define the links between mutations, biochemical dysfunction, and disease expression. The patient population will benefit from this core because it encourages the collaboration of basic and clinical investigators, and the result will be more precise definition of patient subgroups that can be used in the evaluation of patient management and in the planning of further studies to examine treatments and other factors that affect prognosis.

Project Start
1998-09-30
Project End
1999-08-31
Budget Start
1997-10-01
Budget End
1998-09-30
Support Year
5
Fiscal Year
1998
Total Cost
Indirect Cost
Name
Hospital for Sick Chldrn (Toronto)
Department
Type
DUNS #
208511808
City
Toronto
State
ON
Country
Canada
Zip Code
M5 1-X8
Vandivier, R William; Richens, Tiffany R; Horstmann, Sarah A et al. (2009) Dysfunctional cystic fibrosis transmembrane conductance regulator inhibits phagocytosis of apoptotic cells with proinflammatory consequences. Am J Physiol Lung Cell Mol Physiol 297:L677-86
Wilschanski, Michael; Durie, Peter R (2007) Patterns of GI disease in adulthood associated with mutations in the CFTR gene. Gut 56:1153-63
Wilschanski, Michael; Dupuis, Annie; Ellis, Lynda et al. (2006) Mutations in the cystic fibrosis transmembrane regulator gene and in vivo transepithelial potentials. Am J Respir Crit Care Med 174:787-94
Moraes, Theo J; Plumb, Jonathan; Martin, Raiza et al. (2006) Abnormalities in the pulmonary innate immune system in cystic fibrosis. Am J Respir Cell Mol Biol 34:364-74
Oh, Ray S; Bai, Xinli; Rommens, Johanna M (2006) Human homologs of Ubc6p ubiquitin-conjugating enzyme and phosphorylation of HsUbc6e in response to endoplasmic reticulum stress. J Biol Chem 281:21480-90
Bishop, Michele D; Freedman, Steven D; Zielenski, Julian et al. (2005) The cystic fibrosis transmembrane conductance regulator gene and ion channel function in patients with idiopathic pancreatitis. Hum Genet 118:372-81
Mei-Zahav, M; Durie, P; Zielenski, J et al. (2005) The prevalence and clinical characteristics of cystic fibrosis in South Asian Canadian immigrants. Arch Dis Child 90:675-9
Durie, Peter R; Kent, Geraldine; Phillips, M James et al. (2004) Characteristic multiorgan pathology of cystic fibrosis in a long-living cystic fibrosis transmembrane regulator knockout murine model. Am J Pathol 164:1481-93
Gilljam, Marita; Moltyaner, Yuri; Downey, Gregory P et al. (2004) Airway inflammation and infection in congenital bilateral absence of the vas deferens. Am J Respir Crit Care Med 169:174-9
Gilljam, Marita; Ellis, Lynda; Corey, Mary et al. (2004) Clinical manifestations of cystic fibrosis among patients with diagnosis in adulthood. Chest 126:1215-24

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