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Hypogammaglobulinaemia.

机译:低丙种球蛋白血症。

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摘要

This article reviews the primary immunodeficiencies that result in hypogammaglobulinemia or predominantly antibody deficiency disorders. This group makes up the largest proportion of patients with primary immunodeficiency. Significant advances have been made in understanding the molecular basis and clinical characteristics of patients with the more severe forms of antibody deficiency in the last 6 years. Recognition of these disorders remains poor with significant diagnostic delay. The milder forms of antibody deficiency disorders, especially those with normal total serum immunoglobulin G levels, remain poorly characterized and understood. Further work remains to be done in understanding and recognizing these syndromes to benefit patient care and foster further knowledge of the immune system.
机译:本文回顾了导致低球蛋白血症或主要是抗体缺乏症的主要免疫缺陷。该组占原发性免疫缺陷患者的最大比例。在最近的6年中,在了解抗体缺乏更为严重形式的患者的分子基础和临床特征方面取得了重大进展。这些疾病的认识仍然很差,而且诊断延迟很长。抗体缺乏症的轻度形式,尤其是血清总免疫球蛋白G水平正常的形式,仍然缺乏特征和了解。在理解和认识这些综合征以利于患者护理并增进对免疫系统的进一步了解方面,还有待做进一步的工作。

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