CYSTIC-FIBROSIS

While the care of cystic fibrosis (CF) patients has been mainly the pr ovince of pediatricians, great improvements in the therapy and life sp an of CF patients often results in their transition to care by adult p hysicians. In this review of CF, we begin with an overview of the epid emiology and genetics of the disease, with a discussion of the recentl y found ion abnormalities that lead to the clinical manifestations. Th is is followed by a discussion of the pathophysiology. Methods of diag nosis, ranging from the gold standard, the sweat test, to recent advan ces based on a greater understanding of the genetics of the disease ar e reviewed. This is followed by a discussion of therapy primarily gear ed to the treatment of the respiratory complications, as they are the most common lethal factors of the disease. We point out controversies where they exist. Newer forms of therapy such as lung transplantation are discussed, and we finish with a discussion about future therapeuti c modalities, some of which are being approved as the paper is in prin t.