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histopathological findings in cholesteryl ester storage disease: a report of three cases
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نویسنده
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shahramian iraj ,azimi-khatibi esmaeil ,mahjoob fatemeh ,sargazi aliyeh ,delaramnasab mojtaba ,bazi ali
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منبع
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gene cell tissue - 2017 - دوره : 4 - شماره : 3 - صفحه:1 -5
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چکیده
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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.
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کلیدواژه
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cholesteryl ester storage disease ,lysosomal storage diseases ,lipidoses ,hepatology ,histopathology ,triglyceride
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آدرس
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zabol university of medical sciences, pediatric digestive and hepatic research center, ایران, zabol university of medical sciences, school of medicine, department of pathology, ایران, zabol university of medical sciences, children’s medical center, center of excellence, department of pathology, ایران, zabol university of medical sciences, student research committee, ایران, zabol university of medical sciences, amir-almomenin hospital, clinical research development unit, ایران, zabol university of medical sciences, pediatric digestive and hepatic research center, amir-almomenin hospital, clinical research development unit, ایران
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پست الکترونیکی
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m.baziali@gmail.com
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Authors
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