Retrospective review of cystic fibrosis presenting as infantile liver disease

The mode of presentation, clinical course, and outcome of 12 infants with c ystic fibrosis and Liver disease referred over an 18 year period were inves tigated retrospectively. Median age at presentation was 6.5 weeks (range, 5 -12). Two thirds were boys. Conjugated hyperbilirubinaemia was the presenti ng symptom in 11 patients, and hypoalbuminaemia in one. Jaundice was cleare d over a median period of 7.36 months. Eight patients had bile duct prolife ration on liver biopsy and one required cholangiography to exclude biliary atresia. Classic histological features of cystic fibrosis were only present in two children biopsied at 8 and 18 months. Three patients had meconium i leus, including one infant with concomitant a, antitrypsin deficiency, who required early liver transplantation. All other patients had no signs of si gnificant chronic liver disease during a median follow up of 42 months (ran ge, 10-205). Children with cystic fibrosis and infantile liver disease have a good short and medium term prognosis.