Division of Oncology, Center for Childhood Cancer Research, Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
Eur J Pediatr. 2011 Jun;170(6):693-702. doi: 10.1007/s00431-011-1474-x. Epub 2011 May 5.
Primary antibody deficiencies (PADs) are the most common primary immunodeficiencies and are characterized by a defect in the production of normal amounts of antigen-specific antibodies. PADs represent a heterogeneous spectrum of conditions, ranging from often asymptomatic selective IgA and IgG subclass deficiencies to the severe congenital agammaglobulinemias, in which the antibody production of all immunoglobulin isotypes is severely decreased. Apart from recurrent respiratory tract infections, PADs are associated with a wide range of other clinical complications. This review will describe the pathophysiology, diagnosis, and treatment of the different PADs.
原发性抗体缺陷病（PAD）是最常见的原发性免疫缺陷病，其特征是产生正常量抗原特异性抗体的能力缺陷。PAD 代表了一组异质性疾病，从常见的无症状选择性 IgA 和 IgG 亚类缺陷到严重的先天性无丙种球蛋白血症，其中所有免疫球蛋白同种型的抗体产生都严重减少。除了反复呼吸道感染外，PAD 还与广泛的其他临床并发症相关。本文将描述不同 PAD 的发病机制、诊断和治疗。
