Satow K, Arenz B, Kusenbach G, Reinhardt D
Monatsschr Kinderheilkd. 1986 Jul;134(7):459-62.
IgG Immunoglobulins can be differentiated into four subclasses with different structures and functions. Partial or complete defects of one or two subclasses can be related to an impaired immune defence. We describe four children with severe recurrent bacterial airway infections. Two children had developed bronchiectasia following recurrent bronchopulmonary inflammation. Prior to diagnosis of IgG subclass deficiency other common causes of recurrent airway infections were excluded. Defects of IgG 2 or IgG 4 antibodies as well as of both classes were found with compensatory elevation of IgG 1 and IgG 3. In repeated sputum cultures haemophilus influenzae and staphylococcus aureus were isolated. This might be due to an impaired antibody production against special antigens as alpha-toxin of staphylococcus or capsular polysaccharide of haemophilus influenzae. The four cases demonstrate that in children with severe recurrent airway infections including bronchiectasia and otitis media defects of IgG subclasses have to be considered. Diagnosis should be proved by repeated determinations of blood levels after exclusion of other common causes for infections. Diminution of IgG subclasses without clinical symptoms of airway infections is also possible. If diagnosis seems to be certain intravenous substitution with 7 s gammaglobulin beside symptomatic antibiotic therapy is recommended.
IgG免疫球蛋白可分为四个具有不同结构和功能的亚类。一个或两个亚类的部分或完全缺陷可能与免疫防御受损有关。我们描述了四名患有严重复发性细菌性气道感染的儿童。两名儿童在反复发生支气管肺炎后发展为支气管扩张。在诊断IgG亚类缺陷之前,排除了复发性气道感染的其他常见原因。发现IgG 2或IgG 4抗体以及这两类抗体均有缺陷,同时IgG 1和IgG 3出现代偿性升高。在反复的痰培养中分离出流感嗜血杆菌和金黄色葡萄球菌。这可能是由于针对特殊抗原(如金黄色葡萄球菌的α毒素或流感嗜血杆菌的荚膜多糖)的抗体产生受损。这四个病例表明,对于患有严重复发性气道感染(包括支气管扩张和中耳炎)的儿童,必须考虑IgG亚类缺陷。在排除感染的其他常见原因后,应通过反复测定血液水平来证实诊断。IgG亚类减少但无气道感染临床症状的情况也有可能发生。如果诊断似乎确定,除了对症抗生素治疗外,建议静脉注射7 sγ球蛋白进行替代治疗。