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表现为成人肝脏疾病且具有非典型组织学特征的III型糖原贮积症。

Type III glycogenosis presenting as liver disease in adults with atypical histological features.

作者信息

Fellows I W, Lowe J S, Ogilvie A L, Stevens A, Toghill P J, Atkinson M

出版信息

J Clin Pathol. 1983 Apr;36(4):431-4. doi: 10.1136/jcp.36.4.431.

Abstract

Two cases of type III glycogen storage disease are reported in adults; the occurrence of cirrhosis in one case illustrates the potential development of chronic liver disease in this condition. The other was the oldest patient with this condition found in a review of published reports. Electron microscopy of peripheral blood leucocytes to demonstrate excess glycogen was found to be a quick and useful aid to diagnosis. Histology of these adult cases showed a distribution of hepatocyte vacuolation which has not been previously recorded.

摘要

报告了两例成人III型糖原贮积病;其中一例出现肝硬化,说明了这种情况下慢性肝病的潜在发展。另一例是在对已发表报告的回顾中发现的患有这种疾病的最年长患者。通过外周血白细胞的电子显微镜检查来证明糖原过量,被发现是一种快速且有用的诊断辅助手段。这些成人病例的组织学显示肝细胞空泡化的分布情况此前未曾有过记录。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6a13/498238/9137b620e415/jclinpath00509-0068-a.jpg

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