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肌营养不良症中的血清肌红蛋白。

Serum myoglobin in muscular dystrophy.

作者信息

Kagen L J, Moussavi S, Miller S L, Tsairis P

出版信息

Muscle Nerve. 1980 May-Jun;3(3):221-6. doi: 10.1002/mus.880030306.

Abstract

Elevated levels of serum myoglobin (MGB) were found in patients with several types of myopathic disorders, namely, Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), facioscapulohumeral dystrophy, and limb-girdle dystrophy. The frequency of hypermyoglobinemia was greatest in patients with DMD and BMD (91.7% and 100%, respectively). There was no precise direct relationship between serum creatine kinase (CK) and MGB levels, although the disorders with the highest CK levels tended to be the ones with the highest MGB levels. Among the patients with DMD, hypermyoglobinemia was slightly less marked in older children with restricted ability to walk than in the younger ones. Hypermyoglobinemia las noted among 43.8% of the mothers and 41.7% of the sisters of patients with DMD, slightly greater frequencies than for abnormal CK determinations in this group. Multiple serum samples were analyzed from 10 of these women, and the overall frequency of abnormal MGB levels was the same as that of abnormal CK levels; however, levels of CK and MGB were discrepant in some individual serum samples. For this reason, serum MGB determination may be a useful adjunct in the study of possible DMD carriers.

摘要

在几种类型的肌病患者中发现血清肌红蛋白(MGB)水平升高,即杜氏肌营养不良症(DMD)、贝克肌营养不良症(BMD)、面肩肱型肌营养不良症和肢带型肌营养不良症。高肌红蛋白血症的发生率在DMD和BMD患者中最高(分别为91.7%和100%)。血清肌酸激酶(CK)与MGB水平之间没有精确的直接关系,尽管CK水平最高的疾病往往也是MGB水平最高的疾病。在DMD患者中,行走能力受限的大龄儿童高肌红蛋白血症的表现略低于年幼患者。在DMD患者的母亲中有43.8%、姐妹中有41.7%存在高肌红蛋白血症,这一频率略高于该组中CK测定异常的频率。对其中10名女性的多个血清样本进行了分析,MGB水平异常的总体频率与CK水平异常的频率相同;然而,在一些个体血清样本中,CK和MGB水平存在差异。因此,血清MGB测定可能是研究潜在DMD携带者的有用辅助手段。

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