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对定期监测的患有IgG亚类缺陷的土耳其儿童进行临床和实验室评估。

Clinical and laboratory evaluation of periodically monitored Turkish children with IgG subclass deficiencies.

作者信息

Karaca Neslihan Edeer, Karadeniz Cem, Aksu Guzide, Kutukculer Necil

机构信息

Ege University Faculty of Medicine, Department of Pediatrics, Division of Pediatric Immunology, Izmir, Turkey.

出版信息

Asian Pac J Allergy Immunol. 2009 Mar;27(1):43-8.

PMID:19548629
Abstract

IgG subclass deficiencies are common immune system disorders during childhood. The aim of this retrospective study was to review clinical findings and laboratory results of patients with IgG subclass deficiencies in order to determine the changes in serum IgG subclass levels during follow-up, the percentage and time span until normalization of the IgG subclass levels to age-corresponding normal levels, the type of infections incurred and the benefits of prophylaxis. Among the 59 pediatric patients reviewed, the most frequent defect was an IgG3 subclass deficiency (77%). Nine percent of the patients had an isolated IgG2 deficiency and 14% had an IgG2+G3 deficiency. The most common clinical presentations were recurrent upper respiratory tract infections, followed by pneumonia, acute gastroenteritis and urinary tract infections. Atopy was present in 15% of the patients. Ninety percent of the patients were given a prophylactic treatment (benzathine penicillin, oral antibiotics, oral bacterial lysate or intravenous immunoglobulin). The frequency of recurrent infections decreased from 13.4 +/- 7.4 per year to 5.7 +/- 3.9 in patients receiving a prophylactic regimen. Serum IgG subclass levels reached normal ranges in 30% of the patients in the IgG3 deficiency group and in 35.7% of the patients in the IgG2+G3 deficiency group. Patients with an isolated IgG2 deficiency did not reach age-related normal levels during the study period. Our study shows that IgG subclass levels may normalize in 30 to 40% of patients at about 6 years of age. We emphasize the need of monitoring IgG levels together with the clinical symptomatology in affected individuals and initiate preventive measures when appropriate.

摘要

IgG亚类缺陷是儿童期常见的免疫系统疾病。这项回顾性研究的目的是回顾IgG亚类缺陷患者的临床发现和实验室结果,以确定随访期间血清IgG亚类水平的变化、IgG亚类水平恢复至与年龄相应正常水平的百分比和时间跨度、所发生感染的类型以及预防的益处。在所回顾的59例儿科患者中,最常见的缺陷是IgG3亚类缺陷(77%)。9%的患者存在孤立性IgG2缺陷,14%的患者存在IgG2 + G3缺陷。最常见的临床表现是反复上呼吸道感染,其次是肺炎、急性胃肠炎和尿路感染。15%的患者有特应性。90%的患者接受了预防性治疗(苄星青霉素、口服抗生素、口服细菌裂解物或静脉注射免疫球蛋白)。接受预防性治疗方案的患者,反复感染的频率从每年13.4±7.4次降至5.7±3.9次。IgG3缺陷组30%的患者和IgG2 + G3缺陷组35.7%的患者血清IgG亚类水平达到正常范围。在研究期间,孤立性IgG2缺陷的患者未达到与年龄相关的正常水平。我们的研究表明,约6岁时,30%至40%的患者IgG亚类水平可能恢复正常。我们强调需要对受影响个体的IgG水平和临床症状进行监测,并在适当的时候采取预防措施。

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