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一年静脉注射免疫球蛋白替代疗法:减少先天性免疫缺陷患儿住院的疗效。

One-year intravenous immunoglobulin replacement therapy: efficacy in reducing hospital admissions in pediatric patients with Inborn Errors of Immunity.

机构信息

Hospital da Criança de Brasília José Alencar, Clínica de Alergia e Imunologia, Brasília, DF, Brazil.

Universidade de Brasília (UNB), Faculdade de Medicina, Brasília, DF, Brazil.

出版信息

J Pediatr (Rio J). 2022 Mar-Apr;98(2):190-195. doi: 10.1016/j.jped.2021.05.011. Epub 2021 Jul 14.

Abstract

OBJECTIVES

To compare the frequency of hospitalization in children with Inborn Errors of Immunity with antibody deficiency previous to intravenous immunoglobulin (pre- IVIG) with a one-year period after initial IVIG (post-IVIG).

METHODS

Medical reports of 45 patients during an eight-year period were reviewed from 2018 to 2019. Wilcoxon-test was used for related samples.

RESULTS

Forty-five children were included in the study, aged 29-249 months of age, and most of them (64.4%) were males. Median ages at onset symptoms and at diagnosis were 6 and 73 months old, respectively. Specific antibody deficiency and unclassified hypogammaglobulinemia were the predominant diagnoses (31.1% and 17.8%, respectively). X-linked agammaglobulinemia, Hyper IgE syndrome, Hyper IgM, transient hypogammaglobulinemia of infancy, and Common Variable Immunodeficiency (CVID) were also reported, in a low frequency. Forty-four (97.8%) patients were hospitalized before IVIG, and 10 patients (22.2%) after. Annual mean hospital admission reduced from 2.5 to 0.5, pre and post-IVIG, respectively (p < 0.0001). Mean length of stay (LOS) reduced from 71 to 4.7 days/year (p < 0.0001) in general ward and in the PICU from 17.2 days/year to zero (p < 0.0002). Pneumonia was the main cause of hospital admission with a reduction in the number of episodes per patient from an average of 2.2-0.1 per year (p < 0.001). Concomitant use of antibiotic prophylaxis did not influence the number of hospital admission.

CONCLUSION

One-year intravenous IVIG significantly decreased the number of hospitalizations and length of stay in children with impaired antibody production. Social and economic impacts would be required.

摘要

目的

比较患有先天性免疫缺陷伴抗体缺乏症的儿童在接受静脉注射免疫球蛋白(IVIG)前(预 IVIG)和首次 IVIG 后一年内(后 IVIG)的住院频率。

方法

回顾了 2018 年至 2019 年 8 年间的 45 名患者的医疗报告。采用相关样本的 Wilcoxon 检验。

结果

45 名儿童纳入研究,年龄 29-249 个月,其中大多数(64.4%)为男性。发病症状和诊断时的中位年龄分别为 6 和 73 个月。主要诊断为特异性抗体缺陷和未分类低丙种球蛋白血症(分别为 31.1%和 17.8%)。X 连锁无丙种球蛋白血症、高 IgE 综合征、高 IgM、婴儿期一过性低丙种球蛋白血症和常见可变免疫缺陷(CVID)也有报道,但发病率较低。44 名(97.8%)患者在接受 IVIG 前住院,10 名(22.2%)患者在接受 IVIG 后住院。与 IVIG 前相比,IVIG 后每年平均住院次数从 2.5 次降至 0.5 次(p<0.0001)。普通病房和儿科重症监护病房(PICU)的平均住院时间(LOS)从 71 天/年降至 4.7 天/年(p<0.0001)和从 17.2 天/年降至 0(p<0.0002)。肺炎是住院的主要原因,每位患者的发作次数从平均 2.2 次/年降至 0.1 次/年(p<0.001)。同时使用抗生素预防并不会影响住院次数。

结论

为期一年的静脉 IVIG 显著减少了抗体生成受损儿童的住院次数和住院时间。需要进行社会和经济影响评估。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/54d9/9432171/aeefff20fee6/gr1.jpg

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