Quebec Respiratory Health Network

A lever for research in Quebec

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Cystic Fibrosis

The main cause of morbidity and mortality in cystic fibrosis is upper airway compromise. The pathophysiology of this respiratory disease is complex because it involves : 1) a genetic defect with serious consequences on the expression of the CFTR protein, 2) an excessive pulmonary inflammatory response with major disruption of mucus and defenses against bacteria and; 3) an infection with unique and virulent bacterial pathogens. Lung transplantation remains the only option for those most severely affected, but chronic rejection in the form of bronchiolitis obliterans constantly threatens transplanted patients. Finally, diabetes has been found to be present in more than 40% of adults with cystic fibrosis, adding yet another challenge in the fight against cystic fibrosis. Fortunately, in Quebec, we are privileged to have access to research expertise in each of these areas.

The cystic fibrosis research group of the RHN has given itself the mandate of integrating the work of researchers in these areas in order to create a synergy enabling to accelerate the emergence of new therapies designed specifically for the treatment of respiratory cystic fibrosis. The main integrative element of this group is clinical research. All of the researchers in this group require clinical material such as bacterial strains, epithelial cells, mucus and blood samples. In addition, these researchers are involved in collaborative projects with a therapeutic aim. The group includes experts from the Université de Montréal, McGill University et and the Université de Sherbrooke.

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