Hepatic failure in adult Niemann-Pick disease.
The case of a 29-year-old man with Niemann-Pick disease and hepatic failure is presented. Massive hepatomegaly with hepatic calcification were noted in association with a course of persistent hepatitis B serum antigenemia with rapid hepatic decompensation, ascites, encephalopathy and renal failure. The possible relationship of the clinical course to the underlying disease process is discussed, and a review of Niemann-Pick disease is presented.
D000328:Adult; D001921:Brain / Q000378:metabolism; D006509:Hepatitis B / Q000150:complications; D006801:Humans; D050356:Lipid Metabolism; D008099:Liver / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D008107:Liver Diseases / Q000209:etiology*; D008297:Male; D009542:Niemann-Pick Diseases / Q000150:complications* / Q000378:metabolism / Q000473:pathology