Olfactory dysfunction in cystic fibrosis: Impact of CFTR modulator therapy
Olfactory dysfunction in cystic fibrosis: Impact of CFTR modulator therapy
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DOI：
10.1016/j.jcf.2021.09.014
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发表时间：
2022-03-22
影响因子：
5.2
通讯作者：
Taylor-Cousar, Jennifer L.
中科院分区：
文献类型：
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作者：
Beswick, Daniel M.;Humphries, Stephen M.;Taylor-Cousar, Jennifer L.
背景：Elexacaftor-tezacaftor-ivacaftor (ETI) 可改善囊性纤维化 (PwCF) 患者的肺部健康和慢性鼻窦炎 (CRS)，但其对嗅觉的影响尚未得到研究。嗅觉功能障碍会影响生活质量（QOL）。本研究评估了 ETI 对多种嗅觉指标的影响。方法：具有 CF 跨膜电导调节基因型 F508de1/F508de1 或 F508de1/最小功能、临床启动 ETI 的成人 PwCF/CRS 参与了一项前瞻性观察性研究。终点包括 6 个月 ETI 后定量嗅觉功能（气味识别测试，SIT）、嗅觉 QOL（嗅觉障碍问卷，QOD）和嗅裂混浊百分比 (%OCO) 的变化，代表传入嗅觉神经元集中的上鼻腔炎症。结果：30 名 PwCF/CRS 符合纳入标准； 25 人完成了研究。平均 ETI 依从率为 93%。在基线时，参与者嗅觉减退（平均 SIT 31.3），具有显着的 %OCO（平均 65.6%），但报告嗅觉 QOL 未受损（平均 QOD 6.1）。随访时，平均 SIT 略有恶化 (p=0.009)，平均 %OCO 保持稳定 (p=0.46)，平均 QOD 略有改善 (p=0.008)。既往调节剂使用、基因型、鼻息肉或 CF 相关糖尿病不会影响结果。既往鼻窦手术与 QOD 改善相关 (p=0.04)。基线 QOD 分数和 %OCO 的增加（更差）与更大的改善相关（p
Background: Elexacaftor-tezacaftor-ivacaftor (ETI) improves pulmonary health and chronic rhinosinusitis (CRS) for people with cystic fibrosis (PwCF), however its impact on olfaction has not been investigated. Olfactory dysfunction impairs quality-of-life (QOL). This study evaluated the impact of ETI on multiple olfactory metrics.Methods: Adult PwCF/CRS with CF transmembrane conductance regulator genotype F508de1/F508de1 or F508de1/minimal function who clinically initiated ETI participated in a prospective, observational study. Endpoints included changes after 6 months of ETI in quantitative olfactory function (Smell Identification Test, SIT), olfactory QOL (Questionnaire of Olfactory Disorders, QOD) and percent olfactory cleft opacification (%OCO), representing superior nasal cavity inflammation where afferent olfactory neurons are concentrated.Results: 30 PwCF/CRS met inclusion criteria; 25 completed the study. Mean ETI adherence was 93%. At baseline, participants were hyposmic (mean SIT 31.3), had significant %OCO (mean 65.6%), yet reported non-impaired olfactory QOL (mean QOD 6.1). At follow-up, mean SIT worsened mildly (p=0.009), mean %OCO remained stable (p=0.46), and mean QOD improved modestly (p=0.008). No outcomes were impacted by prior modulator use, genotype, nasal polyps, or CF-related diabetes. Prior sinus surgery was associated with QOD improvement (p=0.04). Increased (worse) baseline QOD scores and %OCO were associated with greater improvements (p