Tolia V, Dubois R S, Watts F B, Perrin E
Department of Pediatrics, Wayne State University, Children's Hospital of Michigan, Detroit 48201.
J Pediatr Gastroenterol Nutr. 1987 Nov-Dec;6(6):971-6. doi: 10.1097/00005176-198711000-00026.
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.
小叶间胆管缺乏是婴儿持续性结合胆红素血症常见的组织学特征。它有两种形式：综合征型和非综合征型。综合征型也称为动脉性肝发育不良。非综合征型小叶间胆管缺乏较少见，通常与更严重的疾病及预后不良相关。我们报告两例与小叶间胆管缺乏相关的不寻常肾脏异常。第一例，一名4周大患动脉性肝发育不良的婴儿被诊断为青少年肾单位肾痨；该患者在2月龄时死于严重肾病。第二例表现为严重双侧肾积水和继发于后尿道瓣膜的双侧输尿管积水，与非综合征型小叶间胆管缺乏相关。然而，他在尿道瓣膜矫正手术后病情好转。本文还回顾了文献中先前报道的与慢性肝病相关的其他肾脏异常。
