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首页> 外文期刊>Clinical immunology: The official journal of the Clinical Immunology Society >Lymphocyte characteristics in children with common variable immunodeficiency.
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Lymphocyte characteristics in children with common variable immunodeficiency.

机译：常见可变免疫缺陷儿童的淋巴细胞特征。

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摘要

The diagnosis of common variable immunodeficiency (CVID) is reserved for patients who suffer from undefined B cell dysfunction. Division of the CVID population into subgroups enables research for underlying disease causes. We studied clinical features and lymphocyte characteristics in 38 children with CVID and compared them to 30 children with less severe antibody deficiencies (e.g. specific antibody deficiency combined with IgG subclass deficiency) and with 65 pediatric controls. Most pediatric immune phenotypes were comparable to adult CVID phenotypes, including a selective increase in newly formed B cells and a decrease in memory B cells and CD4(+) T cells. Eighteen percent of pediatric patients had a mutation in the TNFRSF13B gene, which requires further investigation. Finally, pediatric patients with decreased class-switched memory B cells had significantly more complications. A pediatric classification for CVID may enable prediction and early diagnosis of disease related complications and provide a framework for further etiologic research.
机译：常见的可变免疫缺陷病（CVID）诊断保留给患有不确定的B细胞功能障碍的患者。将CVID人群分为亚组可以研究潜在的疾病原因。我们研究了38例CVID患儿的临床特征和淋巴细胞特征，并将其与30例严重的抗体缺乏症（例如，特异性抗体缺乏症与IgG亚类缺乏症相结合）和65例儿科对照的儿童进行了比较。大多数儿科免疫表型与成人CVID表型相当，包括新形成的B细胞的选择性增加以及记忆B细胞和CD4（+）T细胞的减少。 18％的儿科患者的TNFRSF13B基因突变，需要进一步研究。最后，分类切换记忆B细胞减少的儿科患者并发症多得多。 CVID的儿科分类可实现疾病相关并发症的预测和早期诊断，并为进一步的病因学研究提供框架。

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