Cystic fibrosis in adults: the unsuspected pulmonary diagnosis

Abstract

The clinical spectrum of cystic fibrosis is insufficiently appreciated. This disease may occur in adolescents or adults who had minimal or no symptoms in childhood. In three patients the diagnosis was made on the basis of respiratory disease (including bronchiectasis, pneumonia and chronic bronchitis), a family history of respiratory disease, and elevated concentrations of electrolytes in the sweat. Two of the patients had pancreatic insufficiency, but this feature was not considered necessary for the diagnosis. It is important to make the diagnosis of cystic fibrosis in affected adults in order to prescribe appropriate therapy, avoid unnecessary operations, anticipate complications that are not common to other respiratory diseases, provide genetic counselling and initiate appropriate screening procedures to detect the disease in close relatives.