Cystic fibrosis: evolution from a fatal disease of infancy with a clear phenotype to a chronic disease of adulthood with diverse manifestations.
Cystic fibrosis (CF) is the most common life-threatening genetic condition in the Caucasian population. Since the complete pathologic description of the disease in 1938, tremendous progress has been made in identifying its genetic basis, the pathophysiology of CF lung disease, and the development of new therapies. This issue of CRAI will review several areas of progress in our understanding and treatment of CF.
Ren
Clement L
CL
Division of Pediatric Pulmonology, University of Rochester, 601 Elmwood Avenue, Rochester, NY 14642, USA. Clement_ren@urmc.rochester.edu
D000328:Adult; D002908:Chronic Disease; D003550:Cystic Fibrosis / Q000209:etiology / Q000235:genetics / Q000503:physiopathology / Q000628:therapy; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics*; D018450:Disease Progression; D006801:Humans; D007231:Infant, Newborn; D009154:Mutation; D044465:White People
C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator