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静脉注射免疫球蛋白治疗普通变异性免疫缺陷儿童的疗效。

Efficacy of intravenous immunoglobulin treatment in children with common variable immunodeficiency.

机构信息

Marmara University, Division of Pediatric Allergy and Immunology, Istanbul, Turkey.

出版信息

J Investig Allergol Clin Immunol. 2011;21(7):514-21.

Abstract

BACKGROUND

Children with common variable immunodeficiency (CVID) have increased susceptibility to infections.

OBJECTIVE

We evaluated the role of intravenous immunoglobulin (IVIG) replacement therapy on the clinical outcome of patients with CVID.

METHODS

We studied children diagnosed with CVID and treated with IVIG (500 mg/kg every 3 weeks).

RESULTS

The study population comprised 29 children with CVID (mean [SD] age, 11.8 [6.1] years) with at least 1 year of follow-up before IVIG replacement therapy. Mean follow-up duration was 5.6 (3.5) years (range, 15 months-14 years). During therapy, median serum IgG levels increased from 410 to 900 mg/dL. The mean number of respiratory infections per patient per year decreased significantly from 10.2 to 2.5. The annual number and length of hospital stays decreased significantly from 1.36 to 0.21 and 16.35 to 6.33 days per patient, respectively. The mean annual number of antibiotics used decreased significantly from 8.27 to 2.50 per patient. Twelve patients had developed bronchiectasis before initiation of IVIG; 3 patients were cured of this condition. Age at diagnosis, diagnostic delay, number of respiratory tract infections, and number of antibiotics were found to be significantly higher in patients with bronchiectasis, as was lower B-cell percentage. However, gastrointestinal involvement due to noninfectious causes did not improve significantly after IVIG replacement therapy.

CONCLUSION

CVID patients treated with IVIG (500 mg/kg every 3 weeks) had satisfactory serum IgG levels, fewer respiratory tract infections, fewer and shorter hospital stays, and reduced antibiotic usage. However, no effect on gastrointestinal involvement was observed. Early IVIG replacement therapy is important in preventing bronchiectasis.

摘要

背景

患有普通变异性免疫缺陷(CVID)的儿童易受感染。

目的

我们评估静脉注射免疫球蛋白(IVIG)替代疗法对 CVID 患者临床结局的影响。

方法

我们研究了接受 IVIG(500mg/kg,每 3 周一次)治疗的 CVID 患儿。

结果

研究人群包括 29 例 CVID 患儿(平均[SD]年龄 11.8[6.1]岁),在接受 IVIG 替代治疗前至少有 1 年的随访。平均随访时间为 5.6(3.5)年(范围,15 个月至 14 年)。在治疗期间,中位血清 IgG 水平从 410mg/dL 增加到 900mg/dL。每位患者每年的呼吸道感染次数从 10.2 次显著减少到 2.5 次。每位患者的年住院次数和住院时间分别从 1.36 次和 16.35 天显著减少到 0.21 次和 6.33 天。每位患者每年使用的抗生素数量也从 8.27 种显著减少到 2.50 种。在开始使用 IVIG 前,有 12 例患者已发展为支气管扩张症,其中 3 例患者治愈。支气管扩张症患者的年龄、诊断延迟、呼吸道感染次数和抗生素使用次数均显著较高,B 细胞比例则显著较低。然而,非感染性原因导致的胃肠道受累在接受 IVIG 替代治疗后并未显著改善。

结论

接受 IVIG(500mg/kg,每 3 周一次)治疗的 CVID 患者血清 IgG 水平满意,呼吸道感染次数减少,住院次数和住院时间缩短,抗生素使用减少。然而,胃肠道受累未见改善。早期 IVIG 替代治疗对预防支气管扩张症很重要。

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