PORTAL-HYPERTENSION IN PATIENTS WITH CYSTIC-FIBROSIS AND CHRONIC LIVER-DISEASE

To evaluate the presence of chronic liver disease and portal hypertension in patients with cystic fibrosis, we studied 231 cystic fibrosis subjects over age 1 year. In patients with chronic liver disease, clinical and echographic signs suggestive of portal hypertension were evaluated. Patients with suspected portal hypertension underwent esophagogastric endoscopy to detect esophageal varices. Twenty-one percent of cystic fibrosis patients had chronic liver disease. Thirty-six of 48 (75%) subjects with chronic liver disease had clinical or echographic signs of portal hypertension. Thirty-four patients with portal hypertension underwent esophagogastric endoscopy: 11 subjects had esophageal varices and 5 of them experienced at least one episode of bleeding. In the present study, clinical splenomegaly was the most sensitive (100%) marker of the presence of esophageal varices.