Suppr超能文献

静脉注射秋水仙碱治疗儿童家族性地中海热的安全性和有效性。

Safety and efficacy of intravenous Colchicine in children with Familial Mediterranean Fever.

机构信息

Pediatric Day Care Center, Schneider Children's Medical Center of Israel, 4920235, Petach Tikva, Israel.

Pediatric Rheumatology Unit, Schneider Children's Medical Center of Israel, 4920235, Petach Tikva, Israel.

出版信息

Rheumatol Int. 2020 Jan;40(1):121-128. doi: 10.1007/s00296-019-04348-y. Epub 2019 Jun 22.

Abstract

Familial Mediterranean Fever (FMF), the most common monogenic inflammatory disease, is mainly treated by oral Colchicine. However, 5% of patients are considered non-responders and, therefore, candidates for biologic therapy. Intravenous (IV) Colchicine treatment has been shown to be effective and safe in adult patients. The objective of this study was to evaluate the safety of IV Colchicine for pediatric FMF patients in our hospital, refractory to oral Colchicine, by reviewing their medical records. Inclusion criteria were all patients with FMF who commenced treatment with IV Colchicine before the age of 18 years, and received at least 6 months of IV therapy. The patients completed questionnaires to assess the efficacy of the treatment. Between 2004 and 2017, 7 pediatric FMF patients receiving maximal oral Colchicine doses and deemed non-responders were treated with weekly IV Colchicine, including 38 cumulative patient years of follow-up data (a full blood count, renal and liver function tests). All patients were homozygous for the M694V genotype. Long-term follow-up showed normal laboratory results with no Colchicine-related hospital admissions or toxicity. Global health assessment and the number of disease-free days have significantly improved (P < 0.05). Prolonged IV Colchicine use is described in pediatric FMF patients for the first time, with an excellent safety profile in our population, and decrease in intensity and frequency of attacks. In the biological era, IV Colchicine, although not leading to complete remission, may be considered a second-line option in countries where anti-interleukin 1 blockers are not available, or as a third-line option in case of failure to respond to biologics.

摘要

家族性地中海热(FMF)是最常见的单基因炎症性疾病,主要通过口服秋水仙碱治疗。然而,有 5%的患者被认为是无反应者,因此是生物治疗的候选者。静脉注射(IV)秋水仙碱治疗已被证明在成年患者中是有效和安全的。本研究的目的是通过回顾我院口服秋水仙碱治疗无效的儿科 FMF 患者的病历,评估 IV 秋水仙碱治疗儿科 FMF 患者的安全性。纳入标准为所有在 18 岁之前开始接受 IV 秋水仙碱治疗且至少接受 6 个月 IV 治疗的 FMF 患者。患者完成了评估治疗效果的问卷。2004 年至 2017 年,7 名接受最大剂量口服秋水仙碱且被认为无反应的儿科 FMF 患者接受了每周 IV 秋水仙碱治疗,包括 38 个患者年的随访数据(全血细胞计数、肾功能和肝功能检查)。所有患者均为 M694V 基因型纯合子。长期随访显示实验室结果正常,无秋水仙碱相关住院或毒性。总体健康评估和无疾病天数显著改善(P < 0.05)。首次在儿科 FMF 患者中描述了长期使用 IV 秋水仙碱,在我们的人群中具有良好的安全性,且发作的强度和频率降低。在生物治疗时代,IV 秋水仙碱虽然不能完全缓解疾病,但在没有抗白细胞介素 1 阻滞剂的国家,可作为二线治疗选择,或在生物治疗失败时作为三线治疗选择。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验