Cystic fibrosis--a gastroenterological cornucopia.

Cystic fibrosis -a gastroenterological cornucopia Cystic fibrosis, once the province of the paediatrician, has now entered the realm of the adult physician. The most common lethal Mendelian disease of Caucasians, it affects about 1 in 2000 births in Europe and North America. With recent advances in treatment about 60% of affected children can now expect to survive into adulthood, and many at least into their 30s. At least 1000 new patients are now expected to enter adulthood over the next five years. Adults usually come under the care of a chest physician, as the pulmonary manifestations of the disease usually determine survival. Until recently, therefore, both clinical research and management have tended to concentrate on the lungs. Gastrointestinal manifestations, however, occur in most adults and are increasingly recognised to impair the quality of life and to contribute to mortality. Furthermore, the disease may not be manifest until adulthood when it may present in a variety of guises to the gastroenterologist or even to the general surgeon. Cystic fibrosis (CF) therefore now concerns the gastroenterologist dealing with adults.