Edelstein R A, Filling-Katz M R, Pentchev P, Gal A, Chandra R, Shawker T, Guzzetta P, Comly M, Kaneski C, Brady R O
Developmental and Metabolic Neurology Branch, National Institutes of Health, Bethesda, Maryland.
Am J Gastroenterol. 1988 Jun;83(6):687-92.
Cholesteryl ester storage disease (CESD), a rare lysosomal storage disorder characterized by functional deficiency of acid lipase activity, classically features hepatomegaly in conjunction with lipid-laden macrophages containing excessive quantities of cholesteryl esters. We present a patient whose clinical course was complicated by massive, symptomatic splenomegaly, and an unsuspected splenic abscess. Computed tomographic and magnetic resonance imaging are correlated. Histologic, electron microscopic, and biochemical features are presented. To our knowledge, this is the first report of splenic abscess in CESD.
胆固醇酯贮积病（CESD）是一种罕见的溶酶体贮积症，其特征为酸性脂肪酶活性功能缺陷，典型表现为肝肿大并伴有含有过量胆固醇酯的脂质巨噬细胞。我们报告一例临床过程中并发大量有症状脾肿大及未被怀疑的脾脓肿的患者。对计算机断层扫描和磁共振成像结果进行了对比。展示了组织学、电子显微镜和生化特征。据我们所知，这是关于CESD中脾脓肿的首例报告。
