Bucciolini Di Sagni M G, Vannelli Gori G, Oriolo R A
Boll Soc Ital Biol Sper. 1982 May 30;58(10):632-8.
In order to continue the morpho-functional investigations on the muscle of subjects with Duchenne muscular dystrophy, we have studied, by means of light and electron microscope, bioptic fragments of the gastrocnemius muscle, taken from apparently healthy carriers, who had been diagnosed through laboratory tests. The first results obtained stress not only some initial alterations of the muscle fibres (variation in their caliber, with focal hyaline degeneration, necrosis, increase in the number of their nuclei etc.), but also modifications of the connectival structure of the muscle. In fact, both endomysium and perimysium appear noticeably packed, rich in collagen fibrils within a fundamental more electrondense than usual, and frequently showing a granulofilamentous aspect. Many polymorph fibroblasts stretch out thin digitations towards the capillary wall and the sarcolemmal membrane. These alterations are similar to those observed in the skeletal muscles of subjects with DMD in early age or in the preclinical stage of the disease.
为了继续对杜兴氏肌营养不良症患者的肌肉进行形态功能研究,我们通过光学显微镜和电子显微镜,对取自经实验室检查确诊的明显健康携带者的腓肠肌活检片段进行了研究。所获得的初步结果不仅强调了肌纤维的一些初始改变(其直径变化,伴有局灶性透明变性、坏死、核数量增加等),还强调了肌肉结缔组织结构的改变。事实上,肌内膜和肌束膜都明显紧密,在比平常更电子致密的基质中富含胶原纤维,并且经常呈现出颗粒丝状外观。许多多形性成纤维细胞向毛细血管壁和肌膜伸出细指状突起。这些改变与在早年或疾病临床前期的杜兴氏肌营养不良症患者的骨骼肌中观察到的改变相似。