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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.

One new case of arteriohepatic dysplasia (Alagille's syndrome) is reported. The case showed signs of progressive portal hypertension with the liver biopsy revealing evidence of hypoplasia of the intrahepatic portal venous branches without evident cirrhosis, next to the characteristic paucity of intrahepatic bile ducts. This venous abnormality might be responsible for the unfavourable outcome observed in some cases with Alagille's syndrome and corresponds to a severe form of the disease.