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线粒体松散耦合性肌病的随访研究

Follow-up study of a myopathy with loosely coupled mitochondria.

作者信息

Meijer A E, van Wijngaarden G K

出版信息

Pathol Res Pract. 1983 Aug;178(1):73-7. doi: 10.1016/S0344-0338(83)80088-0.

Abstract

This communication deals with a clinical, histological, histochemical and biochemical follow-up study of a boy with an ameliorating myopathy with loosely coupled mitochondria who was studied earlier. In the course of about 15 years the clinical condition of the patient improved greatly. In a biopsy of skeletal muscle the same pathological features were found as before, but the pathological changes were much less severe. In contrast to earlier findings, the majority of the fibres with a loosely coupled state of the mitochondria belonged to the anaerobic type II fibres.

摘要

本通讯报道了对一名患有线粒体解偶联性改善型肌病男孩的临床、组织学、组织化学和生物化学随访研究,该男孩此前已接受过研究。在大约15年的时间里,患者的临床状况有了很大改善。在骨骼肌活检中发现了与之前相同的病理特征,但病理变化的严重程度要轻得多。与早期发现相反,线粒体处于解偶联状态的大多数纤维属于无氧II型纤维。

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