文章基本信息

标题：Histopathological Findings in Cholesteryl Ester Storage Disease: A Report of Three Cases
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作者：Iraj Shahramian ; Esmaeil Azimi-khatibi ; Fatemeh Mahjoob 等
期刊名称：Gene, Cell and Tissue
印刷版ISSN：2345-6841
出版年度：2017
卷号：4
期号：3
DOI：10.5812/gct.58187
出版社：Zahedan University of Medical Sciences
摘要：Cholesteryl ester storage disease (CESD) is a disorder in which cholesteryl esters (CE) and triglycerides (TG) are preserved particularly in hepatocytes. The exact clinical manifestation is not known due to a lack of sufficient data. The current study presents 3 new cases of CESD with 2 of them being siblings. All had perturbed serum low-density lipoprotein (LDL), lactate dehydrogenase (LDH), triglyceride, and cholesterol. Hepatosplenomegaly was seen in all the patients. Liver biopsy showed fat storage in hepatocytes. Adrenomegaly or adrenal calcification, portal hypertension, esophageal varices, and hepatic scarring were not observed in any of the patients. Histopathologic features of CESD, including fat storage in hepatocytes, could characterize these patients.
关键词：Cholesteryl Ester Storage Disease; Lysosomal Storage Diseases; Lipidoses; Hepatology; Histopathology; Triglyceride