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[麦卡德尔病:一例报告]

[McArdle's disease: a case report].

作者信息

Levy J A, Gagioti S M, Cavalieri M J, Pereira J R

出版信息

Arq Neuropsiquiatr. 1980 Dec;38(4):411-4. doi: 10.1590/s0004-282x1980000400012.

Abstract

This disease was described by McArdle as an inherited autosomal recessive affection characterized by glycogen storage with normal chain in the skeletal muscles, due to absence of myophosphorylase activity. Under a clinical aspect, excessive fatigability, cramps and myoglobinuria appear following physical exercise. A case of this disease in a 36-year-old male patient is reported. Failure of elevation of venous blood lactate after physical effort under anaerobic conditions, as well as muscle histochemistry, made diagnosis confirmation possible. The authors comment on the differential diagnosis between McArdle's disease and the other causes of myoglobinuria, specially phosphofructokinase and carnitine-palmityl-transferase deficiency.

摘要

这种疾病由麦卡德尔描述为一种常染色体隐性遗传病,其特征是由于缺乏肌磷酸化酶活性,骨骼肌中糖原链正常但存在糖原储存。从临床角度来看,体育锻炼后会出现过度疲劳、痉挛和肌红蛋白尿。本文报告了一名36岁男性患者患此病的病例。无氧条件下体力活动后静脉血乳酸水平未升高,以及肌肉组织化学检查,使得确诊成为可能。作者对麦卡德尔病与其他导致肌红蛋白尿的病因,特别是磷酸果糖激酶和肉碱-棕榈酰转移酶缺乏症之间的鉴别诊断进行了评论。

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