The benign course of liver disease in adults with cystic fibrosis and the effect of ursodeoxycholic acid.
OBJECTIVE
The life expectancy of patients with cystic fibrosis (CF) has been increasing and the associated liver disease has emerged as a significant medical issue. Our aim was to describe the clinical features, course and effect of ursodeoxycholic acid (UDCA) on liver disease in an adult CF population.


METHODS
From 1983 to 2005, 278 patients with CF were followed up at the Alfred Hospital, an adult tertiary referral centre. Twenty-seven patients (9.7%) satisfied the criteria for liver disease and their clinico-pathological features were assessed. The effect of UDCA on hepatobiliary symptoms and biochemical parameters was determined.


RESULTS
The mean age at liver disease diagnosis was 23 years (range 8-47 years). Portal hypertension was present in 18 (67%) patients. During a median follow-up of 7 years (range 1.5-15), variceal haemorrhage occurred in two patients and ascites in three (one spontaneously). Nine (33%) patients died and five (19%) underwent lung transplantation. There was no encephalopathy, liver transplantation or liver-related deaths. UDCA was taken by 22 patients and was associated with a significant improvement in hepatobiliary symptoms [11/22 (50%) in the pre-UDCA period vs 1/22 (4%) in the post-UDCA period; P=0.0003] and a significant reduction in aspartate aminotransferase (P=0.005); alanine aminotransferase (P<0.001); gamma-glutamyltranspeptidase (P=0.021); and alkaline phosphatase (P<0.001).


CONCLUSIONS
Liver disease in adults with CF is commonly complicated by portal hypertension, but morbidity and mortality associated with this in our small patient population were low. UDCA is associated with improvement in hepatobiliary symptoms and liver function tests.
Department of Gastroenterology, The Alfred Hospital, Melbourne, Vic., Australia. cpdesmond@hotmail.com
Christopher Paul
CP
Desmond
John
J
Wilson
Michael
M
Bailey
Denise
D
Clark
Stuart Keith
SK
Roberts
D000293:Adolescent; D000328:Adult; D002648:Child; D002756:Cholagogues and Choleretics / Q000627:therapeutic use*; D003550:Cystic Fibrosis / Q000150:complications / Q000503:physiopathology*; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006975:Hypertension, Portal / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D008107:Liver Diseases / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology*; D008111:Liver Function Tests; D008297:Male; D008875:Middle Aged; D012720:Severity of Illness Index; D016896:Treatment Outcome; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use*
D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid