Verola O, de Roquancourt A, Chanu B, Rouffy J, Brocheriou C
Sem Hop. 1983 Jun 9;59(23):1753-9.
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.
胆固醇酯贮积病是沃尔曼病的一种轻度形式，由胆固醇酯溶酶体酸性水解酶缺乏引起，导致机体许多细胞中脂质蓄积。作者研究了两名患者的肝实质。脂质沉积见于肝细胞、巨噬细胞和库普弗细胞以及胆小管壁。组织学研究显示它们富含甘油三酯和胆固醇酯。超微结构研究证实了它们的溶酶体内性质。最后，对其中一名患者的腱性黄色瘤进行检查，发现巨噬细胞中有相同性质的沉积物。
