Hepatic failure in adult Niemann-Pick disease

Am J Med Sci. 1986 Sep;292(3):168-72. doi: 10.1097/00000441-198609000-00010.


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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Brain / metabolism
  • Hepatitis B / complications
  • Humans
  • Lipid Metabolism
  • Liver / metabolism
  • Liver / pathology
  • Liver / ultrastructure
  • Liver Diseases / etiology*
  • Male
  • Niemann-Pick Diseases / complications*
  • Niemann-Pick Diseases / metabolism
  • Niemann-Pick Diseases / pathology