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患有IgA缺乏症且反复出现或严重呼吸道感染的儿童中的IgG亚类缺陷。

IgG subclass deficiency in children with IgA deficiency presenting with recurrent or severe respiratory infections.

作者信息

Beard L J, Ferrante A, Oxelius V A, Maxwell G M

出版信息

Pediatr Res. 1986 Oct;20(10):937-42. doi: 10.1203/00006450-198610000-00006.

Abstract

A group of 22 children presenting with recurrent or severe respiratory tract infections who had low IgA levels (more than 2 SD below the mean for age) were examined for IgG subclass deficiency. Patients were screened for possible defects in neutrophil chemotaxis, bactericidal, fungicidal, and quantitative iodination activity, as well as for complement function. The majority of the patients showed IgG subclass levels below the mean for age. Nine of the children showed definite IgG subclass deficiency and at least two showed definite deficiency of more than one IgG subclass. The predominant subclass deficiency was found to be IgG1. While nine children showed IgG4 levels below the level detectable by the technique used, it is not possible to assess whether these patients are deficient in this isotype since some healthy subjects also give values below the level of detection. Most of the patients who had very low (1-6 mg/dl) or undetectable (less than 1 mg/dl) levels of serum IgA did not show IgG subclass deficiencies, while IgG subclass deficiencies were common among those with borderline low IgA levels (slightly more than 2 SD below the mean for age). Nine children showed total IgG levels close to 2 SD below mean for age, and at least six of these showed IgG subclass deficiency. The result suggests that patients with recurrent and/or severe respiratory infections who have borderline IgA and IgG levels may have IgG subclass deficiencies and if they do could benefit from immunoglobulin therapy.

摘要

对一组22名患有复发性或严重呼吸道感染且IgA水平较低(比年龄均值低2个标准差以上)的儿童进行了IgG亚类缺陷检查。对患者进行了中性粒细胞趋化性、杀菌、杀真菌和定量碘化活性以及补体功能方面可能存在的缺陷筛查。大多数患者的IgG亚类水平低于年龄均值。9名儿童表现出明确的IgG亚类缺陷,至少2名儿童表现出不止一种IgG亚类的明确缺陷。发现主要的亚类缺陷是IgG1。虽然9名儿童的IgG4水平低于所用技术可检测的水平,但由于一些健康受试者的检测值也低于检测水平,因此无法评估这些患者在该同种型上是否存在缺陷。大多数血清IgA水平极低(1 - 6mg/dl)或无法检测到(低于1mg/dl)的患者未表现出IgG亚类缺陷,而IgG亚类缺陷在IgA水平临界低(比年龄均值低略超过2个标准差)的患者中很常见。9名儿童的总IgG水平接近比年龄均值低2个标准差,其中至少6名表现出IgG亚类缺陷。结果表明,IgA和IgG水平临界的复发性和/或严重呼吸道感染患者可能存在IgG亚类缺陷,若存在则可能从免疫球蛋白治疗中获益。

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