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[杜兴氏肌营养不良症早期患者及“可能的”携带者骨骼肌的结构和超微结构变化]

[Structural and ultrastructural changes in the skeletal muscles of patients in the early stages of Duchenne muscular dystrophy and "possible" carriers].

作者信息

Bucciolini Di Sagni M G, Vannelli Gori G, Oriolo R A

出版信息

Boll Soc Ital Biol Sper. 1982 May 30;58(10):632-8.

PMID:7115577
Abstract

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.

摘要

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。

相似文献

1
[Structural and ultrastructural changes in the skeletal muscles of patients in the early stages of Duchenne muscular dystrophy and "possible" carriers].[杜兴氏肌营养不良症早期患者及“可能的”携带者骨骼肌的结构和超微结构变化]
Boll Soc Ital Biol Sper. 1982 May 30;58(10):632-8.
2
[Further observations by electron microscope of striated muscle in progressive Duchenne muscular dystrophy].
Boll Soc Ital Biol Sper. 1979 Feb 15;55(3):241-5.
3
Identification of Duchenne muscular dystrophy carriers. Electron microscopical investigation of skeletal muscle.杜兴氏肌营养不良症携带者的鉴定。骨骼肌的电子显微镜研究。
Arch Pathol. 1975 Aug;99(8):436-41.
4
Alterations in creatine kinase in fresh muscle and cell cultures in Duchenne dystrophy.
Ann Neurol. 1981 Apr;9(4):394-9. doi: 10.1002/ana.410090413.
5
An electron microscopical study of the T-system in biopsied muscles from Fukuyama type congenital muscular dystrophy.对福山型先天性肌营养不良活检肌肉中T系统的电子显微镜研究。
Muscle Nerve. 1984 Oct;7(8):629-35. doi: 10.1002/mus.880070806.
6
[Electron microscopic studies on the skeletal muscles in progressive muscular dystrophy. 2. (with special reference to preclinical stages and carriers)].
Nihon Seikeigeka Gakkai Zasshi. 1970 Jul;44(7):493-502.
7
[Ultrasound imaging of muscles in Duchenne muscular dystrophy].[杜兴氏肌营养不良症肌肉的超声成像]
Rinsho Shinkeigaku. 1989 Jan;29(1):49-53.
8
[Ultrastructural investigations of muscular system in a case of duchenne muscular dystrophy in order to identify carriers. The myofibrillar degeneration].[对一例杜兴氏肌营养不良症患者肌肉系统进行超微结构研究以确定携带者。肌原纤维变性]
Beitr Pathol. 1970;141(2):123-41.
9
Muscle and serum enzymes and isoenzymes in muscular dystrophies.肌营养不良症中的肌肉和血清酶及同工酶。
Arch Phys Med Rehabil. 1981 Jun;62(6):265-9.
10
[Ultrastructural observations on 24 cases of pseudo-hypertrophic muscular dystrophy].24例假肥大型肌营养不良症的超微结构观察
Zhonghua Bing Li Xue Za Zhi. 1994;23(3):159-61.

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