Roussel B, Leroux B, Gaillard D, Fandre M
Helv Paediatr Acta. 1985 Dec;40(5):405-13.
The authors report the case of a 6-year-old girl with Laurence-Moon-Bardet-Biedl syndrome and chronic tubulo-interstitial nephritis with glomerulosclerosis requiring chronic peritoneal dialysis, then haemodialysis. One year later, minimal hepatic involvement occurs, leading to portal hypertension. The liver biopsy reveals periportal fibrosis with many small bile ducts.
作者报告了一例6岁患有劳伦斯-穆恩-巴德-比德尔综合征的女孩,其患有慢性肾小管间质性肾炎伴肾小球硬化,需要进行慢性腹膜透析,随后进行血液透析。一年后,出现轻微肝脏受累,导致门静脉高压。肝脏活检显示门周纤维化伴许多小胆管。