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新生儿血色病中的原发性胆汁性肝硬化特异性抗线粒体抗体。

Primary biliary cirrhosis-specific antimitochondrial antibodies in neonatal haemochromatosis.

作者信息

Smyk Daniel S, Mytilinaiou Maria G, Grammatikopoulos Tassos, Knisely A S, Mieli-Vergani Giorgina, Bogdanos Dimitrios P, Vergani Diego

机构信息

Institute of Liver Studies, GI and Nutrition Centre, King's College London School of Medicine at King's College Hospital, Denmark Hill Campus, London SE5 9RS, UK.

出版信息

Clin Dev Immunol. 2013;2013:642643. doi: 10.1155/2013/642643. Epub 2013 Sep 19.

Abstract

BACKGROUND AND AIM

Neonatal hemochromatosis (NH) is characterised by severe liver injury and extrahepatic siderosis sparing the reticuloendothelial system. Its aetiology is obscure, although it has been proposed as an alloimmune disease, resulting from immunological reaction to self-antigens (alloantigens) which the body recognizes as foreign. We studied an infant with NH and his mother whose sera contained antimitochondrial antibody (AMA), the hallmark of primary biliary cirrhosis (PBC).

MATERIAL AND METHODS

To investigate the origin of AMA in the infant, we studied isotype distributions in serum from the mother and infant. Serum samples were obtained at diagnosis of NH, after liver transplantation (LT; age 1 month), and over the ensuing 17 months.

RESULTS

At NH diagnosis, infant and maternal serum contained AMA of the IgG isotype, predominantly of the G3 and G1 subclasses. AMA strongly reacted against the pyruvate dehydrogenase complex E2 subunit (PDC-E2), the major PBC-specific AMA autoantigen. Anti-PDC-E2 responses in both infant and mother declined over time, being present 2 months after LT (mother and child) and absent 10 months later (mother) and 17 months later (child).

CONCLUSION

The association of maternally transferred IgG1 and IgG3 subclass AMA with the appearance of liver damage in an infant with NH may suggest a causal link between antibody and liver damage.

摘要

背景与目的

新生儿血色病(NH)的特征为严重肝损伤和不累及网状内皮系统的肝外铁沉积。其病因不明,尽管有人提出它是一种自身免疫性疾病,由机体将自身抗原(同种抗原)识别为外来抗原而引发免疫反应所致。我们研究了一名患有NH的婴儿及其母亲,其血清中含有原发性胆汁性肝硬化(PBC)的标志性抗体——抗线粒体抗体(AMA)。

材料与方法

为研究婴儿体内AMA的来源,我们研究了母亲和婴儿血清中的抗体亚型分布。在NH诊断时、肝移植(LT;1月龄)后以及随后的17个月内采集血清样本。

结果

在NH诊断时,婴儿和母亲的血清中均含有IgG亚型的AMA,主要为G3和G1亚类。AMA与丙酮酸脱氢酶复合体E2亚基(PDC-E2)强烈反应,PDC-E2是PBC特异性AMA的主要自身抗原。婴儿和母亲体内的抗PDC-E2反应均随时间下降,肝移植后2个月时(母亲和孩子)存在,10个月后(母亲)和17个月后(孩子)消失。

结论

母婴传递的IgG1和IgG3亚类AMA与患有NH的婴儿肝损伤的出现之间的关联可能提示抗体与肝损伤之间存在因果关系。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6ab1/3792542/7f15e3032e64/CDI2013-642643.001.jpg

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