Symptomatic hepatic disease in cystic fibrosis: incidence, course, and outcome of portal systemic hunting.
Fifteen (2.2%) of 693 patients with cystic fibrosis seen over an 18-year period developed clinical hepatic disease. In 13 patients all symptoms were secondary to portal hypertension. Ten had hypersplenism and 6 had variceal bleeding, including 3 who developed both conditions. All 5 patients who survived the initial episode of gastrointestinal bleeding underwent portal systemic shunting. A shunting procedure also was performed on 1 patients with hypersplenism but no variceal bleeding. No subsequent deterioration of intellectual function occurred in either the shunted or unshunted patients. Only 1 of the shunted patients showed progression of hepatic disease after surgery. These results suggest that portal systemic shunting is useful in the treatment of bleeding esophageal varices in cystic fibrosis. A sweat test to rule out cystic fibrosis should be included in the evaluation of any teenage or young adult patient with unexplained portal hypertension.
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications*; D004932:Esophageal and Gastric Varices / Q000209:etiology; D005260:Female; D006471:Gastrointestinal Hemorrhage / Q000209:etiology; D006801:Humans; D006971:Hypersplenism / Q000209:etiology; D006975:Hypertension, Portal / Q000150:complications / Q000209:etiology / Q000601:surgery; D007223:Infant; D008107:Liver Diseases / Q000453:epidemiology / Q000209:etiology* / Q000601:surgery; D008297:Male; D009820:Ohio; D011167:Portacaval Shunt, Surgical