NONSYNDROMIC PAUCITY OF INTERLOBULAR BILE-DUCTS - CLINICAL AND LABORATORY FINDINGS OF 10 CASES

Background: Reports concerning nonsyndromic paucity of the interlobula r bile ducts are not common. Methods: The clinical, biochemical, and h istological features of ten such children were described. Results: All presented with jaundice, starting in the first month in seven and in the fourth, seventeenth, and thirtieth month in the others. Acholic st ools were present intermittently in seven and persistently in three pa tients. Pruritus was a prominent symptom in five. Liver function tests were abnormal in all but one. Liver biopsies were performed at ages o f 20 days to 3 years (median 5 months). In addition to a paucity of in terlobular bile ducts, histology revealed intracellular cholestasis in all, portal fibrosis in four, and regenerative nodules in two patient s. Complications of fat-soluble vitamin deficiency occurred in seven. Therapy consisted of supplementation of those vitamins and administrat ion of cholestyramine, phenobarbital, prednisolone, or ursodeoxycholic acid. While one child had a successful orthotopic liver transplantati on, three died. Consanguinity rate was 80% among the parents, and five of the patients had siblings with similar symptoms. Conclusions: Prog nosis of these patients is variable. Differentiation from other forms of cholestasis is important especially to avoid surgery.