[Hepatic cholesterolosis. Histological, histochemical and ultrastructural study of 2 cases].
Cholesterol ester storage disease, a mild form of Wolman's disease, is caused by a cholesterol ester lysosomial acid hydrolase deficiency leading to an accumulation of lipids in numerous cells of the organism. The authors have studied the liver parenchyma of two patients. The lipid deposits are found in the hepatocytes, macrophages and Küpffer cells, and in the walls of the biliary canaliculi. Histological study shows them to be rich in triglycerides and cholesterol esters. Ultrastructural study confirms their intralysosomal nature. Finally, examination of a tendinous xanthoma in one of the patients revealed deposits of the same nature in the macrophages.
D002788:Cholesterol Esters / Q000378:metabolism*; D005260:Female; D006651:Histocytochemistry; D006801:Humans; D008049:Lipase / Q000172:deficiency; D008052:Lipid Metabolism, Inborn Errors / Q000473:pathology*; D008099:Liver / Q000201:enzymology / Q000473:pathology / Q000648:ultrastructure*; D008107:Liver Diseases / Q000473:pathology*; D008247:Lysosomes / Q000201:enzymology; D008297:Male; D008875:Middle Aged
D002788:Cholesterol Esters; D008049:Lipase
Cholestérolose hépatique. Etude histologique, histochimique et ultrastructurale de deux observations.