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[球形细胞脑白质营养不良(克拉伯病)]

[Globoid cell leukodystrophy (Krabbe's disease)].

作者信息

P'ianov R P, Mikhaĭlova S A

出版信息

Arkh Patol. 1985;47(2):72-5.

PMID:3994543
Abstract

One case of Krabbe's disease was found for 25 years among 40500 newborns; this concerned a girl who died from the aspiration pneumonia at the age of 8.5 months. A number of typical one- and multinuclear globoid cells were observed in white matter of the large hemispheres and brain stem. Brain was atrophied by 27%. Thrombosis of some brain vessels was noted microscopically in the zone affected, this being attributed to the cytomegalovirus angiotoxic effect. The presence of polynuclear leukocytes in the nervus trigeminus branches is considered to be an equivalent of the brain globoid cells that phagocytize the products of the myelin destruction in the peripheral nerves.

摘要

在40500例新生儿中,25年间发现1例克拉伯病;患儿为一名女孩,8.5个月时死于吸入性肺炎。在大脑半球白质和脑干中观察到一些典型的单核和多核球样细胞。脑萎缩27%。在病变区域显微镜下可见一些脑血管血栓形成,这归因于巨细胞病毒血管毒性作用。三叉神经分支中多核白细胞的存在被认为等同于吞噬周围神经髓鞘破坏产物的脑球样细胞。

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