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贝克尔型肌营养不良的组织病理学发现。

Histopathological findings in Becker-type muscular dystrophy.

作者信息

ten Houten R, De Visser M

出版信息

Arch Neurol. 1984 Jul;41(7):729-33. doi: 10.1001/archneur.1984.04050180051017.

Abstract

Muscle biopsy specimens from 14 patients with Becker-type muscular dystrophy were analyzed to investigate possible neurogenic factors underlying the histopathological changes. Group atrophy, pyknotic nuclear clumps, and angular small fibers were seen respectively in 71%, 85%, and 100% of the cases. In one biopsy specimen, notable type grouping was observed. A prominent finding was the appearance of groups of regenerating fibers in biopsy specimens from younger patients. Fiber degeneration was present in only 57% of the cases. While myopathic features predominated in some biopsy specimens, others were compatible with denervation. It is not possible to give an answer to the question whether the changes are basically myopathic or neurogenic (or both), but evidence is growing that a neurogenic component may play a part in the pathogenesis of the disease.

摘要

对14例贝克尔型肌营养不良患者的肌肉活检标本进行分析,以研究组织病理学改变背后可能的神经源性因素。分别在71%、85%和100%的病例中观察到群组性萎缩、固缩核团和角形小纤维。在一份活检标本中,观察到显著的肌纤维类型群组化。一个突出的发现是在较年轻患者的活检标本中出现了再生纤维群。仅57%的病例存在纤维变性。虽然在一些活检标本中肌病特征占主导,但其他标本与去神经支配相符。无法回答这些改变本质上是肌病性的还是神经源性的(或两者皆有)这一问题,但越来越多的证据表明神经源性成分可能在该疾病的发病机制中起作用。

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