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依列卡福妥/替扎卡福妥/依伐卡托治疗的囊性纤维化患者中非结核分枝杆菌的清除:一项多中心队列研究。

Eradication of Nontuberculous Mycobacteria in People with Cystic Fibrosis Treated with Elexacaftor/Tezacaftor/Ivacaftor: A Multicenter Cohort Study.

作者信息

Wiesel Vered, Aviram Micha, Mei-Zahav Meir, Dotan Miri, Prais Dario, Cohen-Cymberknoh Malena, Gur Michal, Bar-Yoseph Ronen, Livnat Galit, Goldbart Aviv, Hazan Guy, Hazan Itai, Golan-Tripto Inbal

机构信息

Medical School for International Health, Ben Gurion University, Beer Sheva, Israel; Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel.

Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel; Pediatric Pulmonary Unit, Soroka University Medical Center, Beer Sheva, Israel.

出版信息

J Cyst Fibros. 2024 Jan;23(1):41-49. doi: 10.1016/j.jcf.2023.05.003. Epub 2023 May 10.

Abstract

BACKGROUND

The prevalence of nontuberculous mycobacteria (NTM) infections is rising in people with cystic fibrosis (pwCF). NTM infection, especially infection with Mycobacterium abscessus complex (MABC), is commonly associated with severe lung deterioration. The current treatment modalities, including multiple intravenous antibiotics, frequently fail to achieve airway eradication. Although treatment with elexacaftor/tezacaftor/ivacaftor (ETI) has been shown to modulate the lung microbiome, data regarding its role in eradicating NTM in pwCF is lacking. Our aim was to evaluate the impact of ETI on the rate of NTM eradication in pwCF.

METHODS

This retrospective multicenter cohort study included pwCF from five CF centers in Israel. PwCF aged older than 6 who had at least one positive NTM airway culture in the past two years and were treated with ETI for at least one year were included. The annual NTM and bacterial isolations, pulmonary function tests, and body mass index were analyzed before and after ETI treatment.

RESULTS

Fifteen pwCF were included (median age 20.9 years, 73.3% females, 80% pancreatic insufficient). In nine patients (66%) NTM isolations were eradicated following treatment with ETI. Seven of them had MABC. The median time between the first NTM isolation and treatment with ETI was 2.71 years (0.27-10.35 years). Eradication of NTM was associated with improved pulmonary function tests (p<0.05).

CONCLUSIONS

For the first time, we report successful eradication of NTM, including MABC, following treatment with ETI in pwCF. Additional studies are needed to assess whether treatment with ETI can result in the long-term eradication of NTM.

摘要

背景

非结核分枝杆菌(NTM)感染在囊性纤维化患者(pwCF)中的患病率正在上升。NTM感染,尤其是脓肿分枝杆菌复合体(MABC)感染,通常与严重的肺部恶化相关。目前的治疗方式,包括多种静脉用抗生素,常常无法实现气道清除。尽管已证明依列卡福/替扎卡福/艾伐卡福(ETI)治疗可调节肺部微生物群,但关于其在根除pwCF患者NTM方面作用的数据尚缺。我们的目的是评估ETI对pwCF患者NTM根除率的影响。

方法

这项回顾性多中心队列研究纳入了来自以色列五个囊性纤维化中心的pwCF。纳入年龄大于6岁、在过去两年中至少有一次NTM气道培养阳性且接受ETI治疗至少一年的pwCF。分析了ETI治疗前后的年度NTM和细菌分离情况、肺功能测试以及体重指数。

结果

纳入了15例pwCF(中位年龄20.9岁,73.3%为女性,80%胰腺功能不全)。9例患者(66%)在接受ETI治疗后NTM分离株被根除。其中7例为MABC感染。首次NTM分离与ETI治疗之间的中位时间为2.71年(0.27 - 10.35年)。NTM的根除与肺功能测试改善相关(p<0.05)。

结论

我们首次报告了在pwCF患者中使用ETI治疗后成功根除NTM,包括MABC。需要进一步研究以评估ETI治疗是否能导致NTM的长期根除。

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