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[尼曼-匹克病(克罗克C型):一例的超微结构研究(作者译)]

[Niemann-Pick disease (Crocker's type C): ultrastructural study of a case (author's transl)].

作者信息

Pellissier J F, Hassoun J, Gambarelli D, Bryon P A, Casanova P, Toga M

出版信息

Acta Neuropathol. 1976 Jan 31;34(1):65-76. doi: 10.1007/BF00684945.

Abstract

The authors report electron microscopic findings in brain, bone marrow and liver biopsies in a case of juvenile Niemann-Pick disease (Crocker's type C). The diagnosis was supported by clinical data increase of blood sphingomyelin and vacuolated histiocytes in bone marrow and liver. Neurons and glial cells were filled with two types of cytosomes: classical multilamellar bodies and unusual pleiomorphic bodies. The latter type probably showed some lipofuscinic component. The relationship between type C and classical Niemann-Pick disease is discussed.

摘要

作者报告了一例青少年尼曼-匹克病(克罗克C型)患者脑、骨髓和肝脏活检的电子显微镜检查结果。临床数据显示血液中鞘磷脂增加以及骨髓和肝脏中出现空泡化组织细胞,支持了该诊断。神经元和神经胶质细胞内充满了两种类型的胞质小体:典型的多层小体和不寻常的多形性小体。后一种类型可能显示出一些脂褐素成分。文中讨论了C型与经典尼曼-匹克病之间的关系。

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