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秋水仙碱治疗儿童家族性地中海热12例分析

李冀 孙之星 王薇 钟林庆 张岩 宋红梅

中国实用儿科杂志2017,Vol.32Issue(7):522-525,4.
中国实用儿科杂志2017,Vol.32Issue(7):522-525,4.DOI:10.19538/j.ek2017070610

秋水仙碱治疗儿童家族性地中海热12例分析

Colchicine therapy for Chinese children with familial Mediterranean fever: An analysis of 12 cases

李冀 1孙之星 1王薇 1钟林庆 1张岩 1宋红梅1

作者信息

  • 1. 中国医学科学院北京协和医院儿科,北京100730
  • 折叠

摘要

Abstract

Objective To assess the efficiency of colchicine therapy in the treatment of Chinese pediatric patients with familial Mediterranean fever(FMF).Methods In the prospective trial,pediatric patients with proven FMF,either by Tel Hashomer criteria or by MEFV gene testing,received colchicine treatment.The following indexes and results were recorded,including the clinical manifestations and signs,CRP,ESR,MEFV sequencing results of pathogenic genes,and the dosage,effective rate and follow-up of colchicine treatment.Results Twelve children(10 diagnosed by Tel Hashomer criteria vs.2 by MEFV sequencing) were enrolled in the study,with the median age at diagnosis/initiation of colchicine of 8 (1 ~ 10) years.They received colchicine treatment at the dose of 0.01 ~ 0.025 mg/(kg· d).Among them 11 completed the study,while 1 (8.3%) had anaphylaxis and quit.During a median follow-up of 11 (8~ 38) months,the response rate was 100%.Conclusion Colchicine is effective in Chinese children patients with FMF,which should be given as earlier as possible to prevent the consequent damage caused by chronic inflammation.

关键词

自身炎症性疾病/家族性地中海热/MEFV基因/秋水仙碱/儿童

Key words

autoinflammatory diseases/familial Mediterranean fever/MEFV/colchicine/child

分类

医药卫生

引用本文复制引用

李冀,孙之星,王薇,钟林庆,张岩,宋红梅..秋水仙碱治疗儿童家族性地中海热12例分析[J].中国实用儿科杂志,2017,32(7):522-525,4.

基金项目

国家卫生和计划生育委员会公益性行业科研专项基金资助项目(201402012) (201402012)

中国实用儿科杂志

OA北大核心CSCDCSTPCD

1005-2224

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