Laurence-Moon-Biedl syndrome accompanied by congenital hepatic fibrosis.
A 33 year old woman with mental deficiency, retinitis pigmentosa, obesity, and parental consanguinity (heredity) was diagnosed as having Laurence-Moon-Biedl syndrome. She also had diabetes mellitus, slight hepatic dysfunction, and hepatomegaly. Liver biopsy showed perilobular fibrosis and bile duct proliferation with cystic dilatation; these findings are consistent with those of congenital hepatic fibrosis.
Nakamura
F
F
Department of Internal Medicine, Hiroshima Memorial Hospital, Japan.
Sasaki
H
H
Kajihara
H
H
Yamanoue
M
M
D000328:Adult; D048909:Diabetes Complications; D005260:Female; D006801:Humans; D007849:Laurence-Moon Syndrome / Q000150:complications* / Q000235:genetics; D008103:Liver Cirrhosis / Q000151:congenital*; D010375:Pedigree