La fibrosis quística en la actualidad (I): aspectos digestivos*

摘要: Title: Cystic fibrosis update time (I): digestive aspects is a genetic disorder characterized mainly by chronic progressive pulmonary disease and pancreatic insufficiency but with wide range of clinical manifestations. Over the last years breakthroughs in understanding its pathogenesis development new therapies have lead to outstanding improvements both survival quality life. Its prognosis mostly depends on severity impairment adequate control gastrointestinal manifestations, ensuring good nutritional condition essential for achieving outcome. The are various they will be examined from most recent points view therapeutical diagnosis. Liver quite common. It often has an early onset patients competitively mild only few developing multifocal cirrhosis conducts portal hypertension hypersplenism. Pancreatic exocrine occurs over 90% requires replacement enzyme therapy. Other complications like unresponsive steatorrhea control, intestinal inflammation distal obstruction syndrome acquired special relevance years.