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[代谢性肌病的形态学]

[Morphology of metabolic myopathies].

作者信息

Pongratz D, Hübner G

出版信息

Monatsschr Kinderheilkd. 1984 Aug;132(8):574-80.

PMID:6384764
Abstract

While muscular lesions in endocrine or metabolic diseases are fairly common primary metabolic myopathies are rare. In these, the metabolic fault is in the muscle fibre itself, which takes on a vacuolated appearance. In the majority of these myopathies there is an abnormality of the glycogen or fat metabolism due to the absence of a specific enzyme in most cases. By contrast, the pathogenesis of the so called mitochondrial myopathies is not yet well understood.

摘要

虽然内分泌或代谢性疾病中的肌肉病变相当常见,但原发性代谢性肌病却很罕见。在这些疾病中,代谢缺陷存在于肌纤维本身,肌纤维会呈现出空泡状外观。在大多数这类肌病中,由于大多数情况下缺乏特定酶,糖原或脂肪代谢存在异常。相比之下,所谓线粒体肌病的发病机制尚未完全明确。

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