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

15 Citations (Scopus)

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
StatePublished - 1992
Externally publishedYes

Fingerprint

Niemann-Pick Diseases
Liver Failure
Oceans and Seas
Sphingomyelins
Liver
Portal Hypertension
Rare Diseases
Lipids
Brain

Keywords

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

ASJC Scopus subject areas

  • Gastroenterology

Cite this

Liver failure and the sea-blue histocyte/adult niemann-pick disease : Case report and review of the literature. / Putterman, Chaim; Zelingher, Julian; Shouval, Daniel.

In: Journal of Clinical Gastroenterology, Vol. 15, No. 2, 1992, p. 146-149.

Research output: Contribution to journalArticle

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