Umetsu D T, Ambrosino D M, Quinti I, Siber G R, Geha R S
N Engl J Med. 1985 Nov 14;313(20):1247-51. doi: 10.1056/NEJM198511143132002.
We studied 20 children with recurrent sinopulmonary infections and serum IgG levels within the normal range, who had selective IgG-subclass deficiency. Twelve of the children were IgG2 deficient, five were IgG3 deficient, and three were deficient in both IgG2 and IgG3. IgA deficiency was present in 3 of the 20 patients. In the children with IgG2 deficiency, serum antibody concentrations to the capsular polysaccharide of Hemophilus influenzae type B (Hib) were significantly lower than those in age-matched controls, both before and after immunization with the Hib capsular polysaccharide antigen, which elicits antibody predominantly of the IgG2 subclass. In contrast, their serum antibody titers to the tetanus and diphtheria toxoid protein antigens, which elicit antibody predominantly of the IgG1 subclass, were normal in comparison with those of age-matched controls. These results suggest that impairment of the antibody response to specific microbial antigens predisposes patients with selective IgG-subclass deficiencies to recurrent infections. Thus, as an aid in determining therapy, children with recurrent infections and normal total serum IgG should be evaluated for this condition.
我们研究了20名反复发生鼻窦肺部感染且血清IgG水平在正常范围内、存在选择性IgG亚类缺陷的儿童。其中12名儿童存在IgG2缺陷,5名存在IgG3缺陷,3名同时存在IgG2和IgG3缺陷。20名患者中有3名存在IgA缺陷。在IgG2缺陷的儿童中,无论是在用B型流感嗜血杆菌(Hib)荚膜多糖抗原免疫之前还是之后,针对Hib荚膜多糖的血清抗体浓度均显著低于年龄匹配的对照组,该抗原主要诱导产生IgG2亚类抗体。相比之下,与年龄匹配的对照组相比,他们针对破伤风和白喉类毒素蛋白抗原的血清抗体滴度正常,这些抗原主要诱导产生IgG1亚类抗体。这些结果表明,对特定微生物抗原的抗体反应受损使选择性IgG亚类缺陷患者易发生反复感染。因此,作为确定治疗方法的辅助手段,对于反复感染且血清总IgG正常的儿童,应评估是否存在这种情况。