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新生儿重症肌无力中的抗乙酰胆碱受体抗体:异质性及致病意义

Anti-acetylcholine receptor antibodies in neonatal myasthenia gravis: heterogeneity and pathogenic significance.

作者信息

Eymard B, Vernet-der Garabedian B, Berrih-Aknin S, Pannier C, Bach J F, Morel E

机构信息

Centre Chirurgical Marie-Lannelongue CNRS URA 1153, Laboratoire d'Immunologie, Le Plessis Robinson, France.

出版信息

J Autoimmun. 1991 Apr;4(2):185-95. doi: 10.1016/0896-8411(91)90017-7.

Abstract

Precipitating, blocking and modulating anti-AChR antibodies and their capacity to recognize embryo or adult muscle were investigated in parallel in maternal and neonatal sera of fifty-two newborns and their myasthenic mothers. Twenty-four babies presented neonatal myasthenia gravis (NMG) with a common expression in twenty cases and foetal involvement in four cases. Occurrence of NMG was clearly related to levels of maternal precipitating, decamethonium blocking (DC blocking) and modulating antibodies (respectively P less than 0.001, P less than 0.02, P less than 0.01, Mann-Whitney test), these three parameters being interrelated. The only significant difference between mothers with severely affected babies and others with mild NMG newborns concerned DC blocking antibodies. Transfer fraction of DC blocking antibodies was significantly higher in NMG than in asymptomatic babies. Our data suggest that (1) the amount of anti-AChR antibodies, whatever the tested category, is involved in the pathogeny of NMG in a more direct manner than in adult MG where the correlation with their levels is poor, and (2) among antibodies, those with blocking effects could be preponderant for triggering NMG and be involved in the severity of the disease in the child.

摘要

在52例新生儿及其患重症肌无力的母亲的母血和新生儿血清中,同时研究了沉淀性、阻断性和调节性抗乙酰胆碱受体(AChR)抗体及其识别胚胎或成年肌肉的能力。24例婴儿出现新生儿重症肌无力(NMG),其中20例表现为常见症状,4例有胎儿受累情况。NMG的发生与母亲的沉淀性抗体、十烃季铵阻断(DC阻断)抗体和调节性抗体水平明显相关(分别为P<0.001、P<0.02、P<0.01,曼-惠特尼检验),这三个参数相互关联。重症患儿的母亲与轻症NMG新生儿的母亲之间唯一显著的差异在于DC阻断抗体。NMG患儿中DC阻断抗体的转移分数显著高于无症状婴儿。我们的数据表明:(1)无论检测的是哪一类抗AChR抗体,其数量在NMG发病机制中的作用比在成年型重症肌无力中更直接,成年型重症肌无力中抗体水平与病情的相关性较差;(2)在这些抗体中,具有阻断作用的抗体可能在引发NMG中起主要作用,并与患儿病情的严重程度有关。

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