Fatal liver failure in two children with Niemann-Pick disease type B.
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.
Service de Pédiatrie, Hôpital Antoine Béclère, Clamart, France.
P
P
Labrune
P
P
Bedossa
P
P
Huguet
F
F
Roset
M T
MT
Vanier
M
M
Odievre
D002675:Child, Preschool; D005260:Female; D006801:Humans; D008099:Liver / Q000473:pathology*; D008103:Liver Cirrhosis / Q000209:etiology* / Q000473:pathology; D008297:Male; D009542:Niemann-Pick Diseases / Q000150:complications* / Q000473:pathology