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对接受皮质类固醇脉冲疗法治疗的闭塞性细支气管炎儿科患者的随访。

Follow-up on pediatric patients with bronchiolitis obliterans treated with corticosteroid pulse therapy.

作者信息

Tomikawa Silvia Onoda, Adde Fabíola Villac, da Silva Filho Luiz Vicente Ribeiro Ferreira, Leone Claudio, Rodrigues Joaquim Carlos

机构信息

Pediatric Pulmonology Division, Instituto da Criança, Hospital das Clínicas, University of São Paulo, Avenida Dr Enéas de Carvalho Aguiar, 647, São Paulo, CEP 05403-000, SP, Brazil.

出版信息

Orphanet J Rare Dis. 2014 Aug 15;9:128. doi: 10.1186/s13023-014-0128-2.

Abstract

BACKGROUND

Bronchiolitis obliterans (BO) is a rare but severe disease in children. Currently, there is no consensus on the treatment for BO with respect to the systemic use of corticosteroids. Here we report on the follow-up of children with a diagnosis of BO who were treated with corticosteroid pulse therapy.

METHODS

Forty patients fulfilling the BO diagnosis criteria were treated with methylprednisolone pulse therapy in monthly cycles until clinical improvement. After the pulse therapy began, we analyzed the clinical and laboratory data at intervals. Statistical analyses were performed using non-parametric tests to compare repeated measures (Friedman, Wilcoxon) or paired nominal data (McNemar) (α = 5%).

RESULTS

The frequency of wheezing exacerbations and hospitalizations was reduced (p = 0.0042 and p < 0.0001, respectively) and oxygen saturation improved (p = 0.0002) in the pulse therapy-treated patients. Prolonged oral corticosteroid therapy was discontinued in 83% of these patients. The mean Z-score length for age improved from -1.08 to -0.63, and the mean Z-score weight for age improved from -0.91 to -0.59. The adverse effects during the infusion were temporary and none were serious.

CONCLUSIONS

Our data suggest that pulse corticotherapy could be a safe alternative to prolonged systemic oral corticotherapy in children with BO, thus minimizing the adverse effects of the oral therapy. New prospective controlled studies are required to confirm this proposition.

摘要

背景

闭塞性细支气管炎(BO)在儿童中是一种罕见但严重的疾病。目前,关于全身使用糖皮质激素治疗BO尚无共识。在此我们报告接受糖皮质激素冲击治疗的BO诊断患儿的随访情况。

方法

40例符合BO诊断标准的患者接受甲泼尼龙冲击治疗,每月1个周期,直至临床改善。冲击治疗开始后,我们定期分析临床和实验室数据。采用非参数检验进行统计分析,以比较重复测量数据(Friedman检验、Wilcoxon检验)或配对名义数据(McNemar检验)(α = 5%)。

结果

接受冲击治疗的患者喘息加重频率和住院次数降低(分别为p = 0.0042和p < 0.0001),血氧饱和度改善(p = 0.0002)。这些患者中有83%停用了长期口服糖皮质激素治疗。年龄的平均Z评分身长从-1.08改善至-0.63,年龄的平均Z评分体重从-0.91改善至-0.59。输液期间的不良反应是暂时的,均不严重。

结论

我们的数据表明,对于BO患儿,冲击性皮质激素治疗可能是长期全身口服皮质激素治疗的一种安全替代方法,从而将口服治疗的不良反应降至最低。需要新的前瞻性对照研究来证实这一观点。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2715/4243923/8ee5a5551ce8/13023_2014_128_Fig1_HTML.jpg

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