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24例假肥大型肌营养不良症的超微结构观察

[Ultrastructural observations on 24 cases of pseudo-hypertrophic muscular dystrophy].

作者信息

Hang Z B, Luo D R, Wu K M

机构信息

Department of Pathology, West China University of Medical Sciences, Chengdu, Sichuan.

出版信息

Zhonghua Bing Li Xue Za Zhi. 1994;23(3):159-61.

PMID:7954955
Abstract

Muscular biopsy specimens of 24 cases of pseudo-hypertrophic (Duchenne's) muscular dystrophy (DMD) were studied with light and electron microscopy. Ultrastructural changes include: I. Disappearance of sarcolemma in focal areas of muscle fibers with degeneration or focal necrosis, which may be the preliminary muscle fiber changes in DMD. 2. Repair of sarcolemma and regenerative sarcomere were observed in some injured muscle fibers, but the ability to repair and regeneration being so weak that injured muscle fibers can not be completely restored. 3. In the early stage of DMD when muscle fibers have not yet to become obviously atrophied, fat cell infiltration between swollen or intact muscle fibers can be observed but cannot be explained. In addition, 3 carriers (mothers of 3 DMD patients) and one asymptomatic brother of a DMD patient were examined, some serological and ultrastructural changes were found.

摘要

对24例假肥大性(杜兴氏)肌营养不良症(DMD)患者的肌肉活检标本进行了光镜和电镜研究。超微结构变化包括:I. 肌肉纤维发生变性或局灶性坏死的局部区域肌膜消失,这可能是DMD中肌肉纤维的早期变化。2. 在一些受损肌肉纤维中观察到肌膜修复和再生肌节,但修复和再生能力非常弱,受损肌肉纤维无法完全恢复。3. 在DMD早期,当肌肉纤维尚未明显萎缩时,可观察到肿胀或完整的肌肉纤维之间有脂肪细胞浸润,但原因不明。此外,对3名携带者(3名DMD患者的母亲)和1名DMD患者的无症状兄弟进行了检查,发现了一些血清学和超微结构变化。

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