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杜兴氏肌病中尿儿茶酚胺及其代谢产物的模式与疾病进展的关系。

The pattern of urinary catecholamines and their metabolites in Duchenne myopathy, in relation to disease evolution.

作者信息

Dalmaz Y, Peyrin L, Mamelle J C, Tuil D, Gilly R, Cier J F

出版信息

J Neural Transm. 1979;46(1):17-34. doi: 10.1007/BF01243426.

Abstract

In this report we have tried to determine whether or not catecholamines are involved in the progressive muscular dystrophy. Catecholamines and their metabolites were studied in urines of children with Duchenne disease or other forms of myopathy (limb-girdle and facio-scapulo humeral myopathies). Catecholamine deaminated metabolites were normal in either form of myopathy; in contrast, Duchenne patients, contrarily to other children, eliminated excessive amounts of most amines (catecholamines and methoxylated amines) in relation to age and degree of disease evolution. Our results indicate that catecholamines are not the primary factors involved in the pathogenesis of Duchenne myopathy, but are rather secondary to some disease effects. It is suggested that the high excretion of catecholamines and their methoxylated amine metabolites observed in severely affected Duchenne boys might be related to thermoregulatory process or/and to alterations in some enzymatic systems.

摘要

在本报告中,我们试图确定儿茶酚胺是否与进行性肌营养不良有关。对患有杜氏病或其他形式肌病(肢带型和面肩肱型肌病)的儿童尿液中的儿茶酚胺及其代谢产物进行了研究。在任何一种肌病形式中,儿茶酚胺脱氨基代谢产物均正常;相比之下,与其他儿童不同,杜氏病患者相对于年龄和疾病进展程度而言,排出了过量的大多数胺类(儿茶酚胺和甲氧基化胺类)。我们的结果表明,儿茶酚胺不是杜氏肌病发病机制中的主要因素,而是某些疾病效应的继发结果。有人提出,在严重受影响的杜氏病男孩中观察到的儿茶酚胺及其甲氧基化胺代谢产物的高排泄量可能与体温调节过程或/和某些酶系统的改变有关。

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