Bradwell A R
Department of Immunology, Medical School, University of Birmingham, United Kingdom.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi. 1995 May-Jun;36(3):164-9.
Selective IgG subclass (IgGSc) deficiencies are frequently found in association with recurrent infections in childhood. IgG1 deficiency is the most severe and is associated with features typical of panhypogammaglobulinaemia. Immunoglobulin replacement therapy is usually required. IgG2 deficiency is associated with recurrent infections with encapsulated bacteria such as Haemophilus influenzae and Streptococcus pneumoniae. IgG2 deficiency may be transient in children under five years of age and patients improve with antibiotics and immunisation. IgG3 and IgG4 deficiency are commonly found in children with recurrent infections and may indicate a disordered immune system since absence of these antibodies alone appears insufficient to cause symptoms. Children may also have selective IgGSc deficiencies in the absence of recurrent infections. This is explained by compensatory factors in other parts of the immune system. Measurement of IgGSc levels should be based on highly specific polyclonal antisera which show no IgGSc cross-reactivity. Most monoclonal antibodies are unsatisfactory since allotypes are detected variably, leading to excess reporting of IgGSc deficiencies and Mabs cannot be used for nephelometric or turbidimetric methods.
选择性IgG亚类(IgGSc)缺陷在儿童复发性感染中经常被发现。IgG1缺陷最为严重,与全低丙种球蛋白血症的典型特征相关。通常需要进行免疫球蛋白替代治疗。IgG2缺陷与由包膜细菌(如流感嗜血杆菌和肺炎链球菌)引起的复发性感染有关。IgG2缺陷在五岁以下儿童中可能是暂时的,患者通过抗生素治疗和免疫接种后病情会有所改善。IgG3和IgG4缺陷常见于复发性感染的儿童中,可能表明免疫系统紊乱,因为仅缺乏这些抗体似乎不足以引起症状。儿童在没有复发性感染的情况下也可能存在选择性IgGSc缺陷。这可以用免疫系统其他部分的代偿因素来解释。IgGSc水平的测定应基于高度特异性的多克隆抗血清,这些抗血清不显示IgGSc交叉反应性。大多数单克隆抗体并不令人满意,因为同种异型的检测结果不一致,导致IgGSc缺陷报告过多,并且单克隆抗体不能用于比浊法或散射比浊法。