Liver failure and the sea-blue histocyte/adult niemann-pick disease: Case report and review of the literature

Chaim Putterman, Julian Zelingher, Daniel Shouval

Research output: Contribution to journalArticle

16 Scopus citations

Abstract

Niemann–Pick disease is a metabolic disorder resulting in accumulation of sphingomyelin in visceral organs. The adult form (type B) is characterized by the sparing of brain involvement, allowing those affected to have a relatively benign course. Although the abnormal lipid accumulation in the liver is commonly recognized, hepatocellular compromise is extremely rare. We describe a patient with adult Niemann–Pick disease who over the course of over 35 years developed hepatic failure and portal hypertension, and we review the literature regarding hepatic involvement in this rare disease.

Original languageEnglish (US)
Pages (from-to)146-149
Number of pages4
JournalJournal of clinical gastroenterology
Volume15
Issue number2
DOIs
StatePublished - Sep 1992
Externally publishedYes

Keywords

  • Liver failure
  • Niemann–Pick
  • Portal hypertension
  • Sea-blue histiocyte

ASJC Scopus subject areas

  • Gastroenterology

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