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依伐卡托与泰他卡托和艾克巴司他联合制剂治疗囊性纤维化慢性鼻-鼻窦炎的疗效。

Efficacy of Elexacaftor-Tezacaftor-Ivacaftor on chronic rhinosinusitis in cystic fibrosis.

机构信息

University Hospital of Verona, Section of Otolaryngology-Head and Neck Surgery, Department of Surgical Sciences, 37126 Verona, Italy.

University Hospital of Verona, Cystic Fibrosis Center, 37126 Verona, Italy.

出版信息

Am J Otolaryngol. 2024 May-Jun;45(3):104236. doi: 10.1016/j.amjoto.2024.104236. Epub 2024 Feb 24.

DOI:10.1016/j.amjoto.2024.104236
PMID:38417261
Abstract

PURPOSE

Our work aims to add evidence on the effectiveness of Elexacaftor-Tezacaftor-Ivacaftor on chronic rhinosinusitis in cystic fibrosis.

MATERIALS AND METHODS

We conducted an observational retrospective cohort study at the Cystic Fibrosis Center of a tertiary care hospital to investigate the effect of Elexacaftor-Tezacaftor-Ivacaftor on chronic rhinosinusitis in cystic fibrosis patients, aged 12 or older. The study's endpoints were the change in the occurrence of acute exacerbations of chronic rhinosinusitis, and the variation of the endoscopic and radiologic findings scored using the Lund-Kennedy endoscopic scale, Lund-Mackay, and modified Lund-Mackay radiologic scales, in patients who underwent both pre-treatment and post-treatment examinations.

RESULTS

The study population comprised 136 patients, of which 28 underwent both pre-treatment and post-treatment nasal endoscopy and 15 had pre- and post-treatment CT scans. Elexacaftor-Tezacaftor-Ivacaftor provided a significant improvement in chronic rhinosinusitis. The mean number of acute exacerbations of chronic rhinosinusitis per year in the pre-treatment time was 0.55 versus 0.35 during the treatment (p < 0.0021). The Lund-Kennedy scale had a pre-treatment average score of 4.21 points versus 1.5 points after the start of Elexacaftor-Tezacaftor-Ivacaftor (p < 0.0001). The average Lund-Mackay and modified Lund-Mackay scores in the pre-treatment time were respectively 14.6 and 16.45 points; and after the start of the therapy, they became 5.87 and 6.73 (p < 0.0001).

CONCLUSION

Elexacaftor-Tezacaftor-Ivacaftor was associated with fewer acute exacerbations of chronic rhinosinusitis, and a significant improvement of chronic rhinosinusitis evaluated endoscopically and radiologically. To our knowledge, this is the first study investigating the change in the occurrence of acute exacerbation of chronic rhinosinusitis in patients affected by cystic fibrosis in therapy with Elexacaftor-Tezacaftor-Ivacaftor.

摘要

目的

我们的工作旨在为依伐卡托特钠-泰比卡托特钠-艾氟卡托特钠治疗囊性纤维化慢性鼻-鼻窦炎提供疗效证据。

材料和方法

我们在一家三级医院的囊性纤维化中心进行了一项观察性回顾性队列研究,以调查依伐卡托特钠-泰比卡托特钠-艾氟卡托特钠对 12 岁及以上囊性纤维化患者慢性鼻-鼻窦炎的影响。研究的终点是慢性鼻-鼻窦炎急性加重的发生率变化,以及使用 Lund-Kennedy 内镜评分、Lund-Mackay 和改良 Lund-Mackay 放射评分评估的内镜和放射学发现的变化,这些患者都接受了治疗前和治疗后的检查。

结果

研究人群包括 136 名患者,其中 28 名患者进行了治疗前和治疗后的鼻内镜检查,15 名患者进行了治疗前和治疗后的 CT 扫描。依伐卡托特钠-泰比卡托特钠-艾氟卡托特钠治疗可显著改善慢性鼻-鼻窦炎。治疗前每年慢性鼻-鼻窦炎急性加重的平均次数为 0.55 次,治疗期间为 0.35 次(p<0.0021)。Lund-Kennedy 评分治疗前平均得分为 4.21 分,依伐卡托特钠-泰比卡托特钠-艾氟卡托特钠治疗开始后为 1.5 分(p<0.0001)。治疗前 Lund-Mackay 和改良 Lund-Mackay 评分分别为 14.6 和 16.45 分;治疗开始后,它们分别变为 5.87 和 6.73(p<0.0001)。

结论

依伐卡托特钠-泰比卡托特钠-艾氟卡托特钠与慢性鼻-鼻窦炎急性加重次数减少有关,并在影像学和内镜下显著改善慢性鼻-鼻窦炎。据我们所知,这是第一项研究囊性纤维化患者接受依伐卡托特钠-泰比卡托特钠-艾氟卡托特钠治疗后慢性鼻-鼻窦炎急性加重发生率变化的研究。

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