[Cystic fibrosis in adults]

Abstract

In the last two decades the life expectancy of patients with cystic fibrosis (CF) has improved enormously: it is now estimated at around 30 years, but some patients reach an older age and one third of all patients with CF currently reach adulthood. Once considered a pediatric disease, CF now is a disease of adults too. As a consequence, internists must be highly knowledgeable about all the clinical patterns of this multifarious disease, must be better qualified to recognize its complications and must be highly trained to treat them. This review is dedicated to internists and general practitioners and not to the specialists in the research and clinical field of CF, until now only pediatricians; it briefly summarizes the medical history of CF and the current knowledge at the molecular, genetic and clinical levels about this disease; it reports up-to-date epidemiological data and it illustrates the clinical patterns which are more frequently encountered in adult patients. The true target of this review is to stimulate a greater interest and participation in this clinical field by adult caregivers, as a development of a medical care system for adults with CF is now crucial. Therefore, a growing number of internists will be increasingly involved in providing care for patients with this disorder.