[Peripheral neuropathy and chronic cholestasis due to paucity of interlobular bile ducts (author's transl)].
Peripheral neuropathy was observed in three patients in the course of a chronic cholestasis due to paucity of interlobular bile ducts associated with several malformations (Alagille's syndrome). In two patients, investigations showed a chronic axonal neuropathy. In a third, the low density of myelinated fibers was associated with onion-bulb proliferation on the Schwann-cells and myelin abnormalities related to an associated dominant inherited primary hypertrophic neuropathy. The responsibility of a chronic vitamin E deficiency in the development of axonal lesions is discussed.
D000293:Adolescent; D001369:Axons / Q000473:pathology; D001653:Bile Ducts, Intrahepatic / Q000002:abnormalities*; D002648:Child; D002780:Cholestasis, Intrahepatic / Q000150:complications*; D002908:Chronic Disease; D005260:Female; D006801:Humans; D008297:Male; D010523:Peripheral Nervous System Diseases / Q000209:etiology* / Q000473:pathology; D014811:Vitamin E Deficiency / Q000150:complications
Neuropathie périphérique et cholestase chronique par hypoplasie ductulaire.