J Pediatr Gastroenterol Nutr 1987 Nov-Dec;6(6):971-6

Renal abnormalities in paucity of interlobular bile ducts

Tolia V, Dubois RS, Watts FB Jr, Perrin E
Department of Pediatrics, Wayne State University, Children's Hospital of
Michigan, Detroit 48201

Abstract
Paucity of interlobular bile ducts is a common histologic characteristic inpersistent conjugated hyperbilirubinemia of infancy. It occurs in two forms: thesyndromatic and the nonsyndromatic types. The syndromatic form is also calledarteriohepatic 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