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

To evaluate the presence of chronic liver disease and portal hypertens ion in patients with cystic fibrosis, we studied 231 cystic fibrosis s ubjects over age 1 year. In patients with chronic liver disease, clini cal and echographic signs suggestive of portal hypertension were evalu ated. Patients with suspected portal hypertension underwent esophagoga stric endoscopy to detect esophageal varices. Twenty-one percent of cy stic fibrosis patients had chronic liver disease. Thirty-six of 48 (75 %) subjects with chronic liver disease had clinical or echographic sig ns of portal hypertension. Thirty-four patients with portal hypertensi on underwent esophagogastric endoscopy: 11 subjects had esophageal var ices and 5 of them experienced at least one episode of bleeding. In th e present study, clinical splenomegaly was the most sensitive (100%) m arker of the presence of esophageal varices.