Renal abnormalities in paucity of interlobular bile ducts

Paucity of interlobular bile ducts is a common histologic characteristic in persistent conjugated hyperbilirubinemia of infancy. It occurs in two forms: the syndromatic and the nonsyndromatic types. The syndromatic form is also called arteriohepatic dysplasia. The nonsyndromatic type of paucity of interlobular bile ducts occurs less frequently and is usually associated with more severe disease and a less favorable prognosis. We present two unusual renal anomalies in association with paucity of interlobular bile ducts. In the first case, juvenile nephronophthisis was diagnosed in a 4-week-old infant with arteriohepatic dysplasia; the patient died from severe renal disease by 2 months of age. The second case presented with severe bilateral hydronephrosis and hydroureter secondary to posterior urethral valves associated with the nonsyndromatic form of paucity of interlobular bile ducts. He, however, improved after corrective surgery of the urethral valves. Other renal abnormalities previously reported in the literature in association with chronic liver disease are also reviewed.