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杜氏肌营养不良症和多发性肌炎中骨骼肌改变的比较研究。

Comparative study of alterations of skeletal muscle in Duchenne muscular dystrophy and polymyositis.

作者信息

Morizumi H, Hizawa K, Nunomura S, Ii K

出版信息

Acta Pathol Jpn. 1984 Nov;34(6):1221-42. doi: 10.1111/j.1440-1827.1984.tb00550.x.

Abstract

Histological and ultrastructural alterations of skeletal muscles in Duchenne muscular dystrophy (DMD) (32 cases) and polymyositis (PM) (33 cases) were compared qualitatively and quantitatively. Regeneration is more numerous in PM than DMD throughout all stages. Stromal fibrosis and hyaline fiber with delta lesion are characteristic for DMD. In the electron microscope, we observed the usual preservation of basement membrane in degenerating muscle fibers and uncomplicated muscle regeneration in PM. On the other hand, there were partial disruption of the plasma membrane lining the delta lesion and degeneration of basement membrane and probable invasion of collagen fiber into sarcoplasm in some hypercontracted fibers in DMD. Besides, there appeared atypical form of regeneration in DMD, namely myocytes arrested at the stage of mononuclear myoblast or primitive myotube with degeneration of myofibrils and cytoplasmic organelles. At the advanced stage of DMD, there was scarce activity of regeneration. Stromal fibrosis, abnormality of membrane systems, and failure of regeneration were the characteristics of DMD.

摘要

对32例杜氏肌营养不良症(DMD)和33例多发性肌炎(PM)患者骨骼肌的组织学和超微结构改变进行了定性和定量比较。在所有阶段,PM中的再生都比DMD更为显著。间质纤维化和伴有δ病变的透明纤维是DMD的特征。在电子显微镜下,我们观察到PM中退化的肌纤维基底膜通常保持完整,且肌肉再生未出现并发症。另一方面,在DMD中,一些过度收缩的纤维中,δ病变处的质膜出现部分破坏,基底膜退化,胶原纤维可能侵入肌浆。此外,DMD中出现了非典型的再生形式,即肌细胞停滞在单核成肌细胞或原始肌管阶段,伴有肌原纤维和细胞质细胞器的退化。在DMD的晚期,再生活动稀少。间质纤维化、膜系统异常和再生失败是DMD的特征。

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