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.
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