Natural history of liver disease in cystic fibrosis

The median age of the population with cystic fibrosis (CF) has increased wo rldwide, which has led to the suggestion that the prevalence of liver disea se would increase. The aim of this study was to evaluate the natural histor y of CF-associated liver disease over a 15-year period in a well-controlled population of patients with CF. During the years 1976 through 1993, 124 pa tients were followed up by yearly liver function tests (LFTs). Fifteen pati ents were followed up with liver biopsies throughout the whole study period . More than 50% of the patients had pathological LFTs in infancy, later bei ng normalized. Approximately 25% of children 4 years of age or older had bi ochemical markers of liver disease during the study period. In about 10% of the patients, cirrhosis or advanced fibrosis was confirmed at biopsy and 4 % of patients had cirrhosis with clinical liver disease. Severe liver disea se developed mainly during prepuberty and puberty. Of the 15 patients prosp ectively followed up with liver biopsies, only 3 had progressive fibrosis. No specific risk factor was identified, but deficiency of essential fatty a cids was found more often in patients with marked steatosis (P < .05). No p atient developed clinical liver disease in adulthood and the histological c hanges in the liver biopsies were usually not progressive. Liver disease wa s no more frequent at the end of the study period although the median age o f the patient population had increased. Modern treatment might positively i nfluence liver disease because it seemed less common, less progressive, and less serious than previously reported.