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Case Reports
. 1991 Jul;13(1):104-9.
doi: 10.1097/00005176-199107000-00020.

Fatal liver failure in two children with Niemann-Pick disease type B

P Labrune  1 , P Bedossa, P Huguet, F Roset, M T Vanier, M Odievre
Affiliations
Case Reports

Fatal liver failure in two children with Niemann-Pick disease type B

P Labrune et al. J Pediatr Gastroenterol Nutr. 1991 Jul.

Abstract

We report on two young patients with Niemann-Pick disease type B presenting with severe hepatic disease. Both children developed cirrhosis and died of intrahepatic block and mechanical hemolysis. Autopsy findings revealed complete obstruction of the sinusoids by lipid-laden Kupffer's cells. These two cases illustrate a new hepatic lesion of Niemann-Pick disease.

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