METHODS: In a prospective, multi-institutional study, adult PwCF completed the 22-Question SinoNasal Outcome Test (SNOT-22), Smell Identification Test (SIT), Questionnaire of Olfactory Disorder Negative Statements (QOD-NS), and Cystic Fibrosis Questionnaire-Revised (CFQ-R). Lund-Kennedy scores, sinus computed tomography, and clinical data were collected. Data were analyzed across race/ethnicity, sex, and socioeconomic factors using multivariate regression.
RESULTS: Seventy-three PwCF participated with a mean age of 34.7 ± 10.9 years and 49 (67.1%) were female. Linear regression identified that elexacaftor/tezacaftor/ivacaftor (ETI) use (β = ‒4.09, 95% confidence interval [CI] [‒6.08, ‒2.11], p < 0.001), female sex (β = ‒2.14, 95% CI [‒4.11, ‒0.17], p = 0.034), and increasing age (β = ‒0.14, 95% CI [‒0.22, ‒0.05], p = 0.003) were associated with lower/better endoscopy scores. Private health insurance (β = 17.76, 95% CI [5.20, 30.32], p = 0.006) and >16 educational years (β = 13.50, 95% CI [2.21, 24.80], p = 0.020) were associated with higher baseline percent predicted forced expiratory volume in one second (ppFEV1). Medicaid/Medicare insurance was associated with worse endoscopy scores, CFQ-R respiratory scores, and ppFEV1 (all p < 0.017), and Hispanic/Latino ethnicity was associated with worse SNOT-22 scores (p = 0.047), prior to adjustment for other cofactors. No other SDS factors were associated with SNOT-22, QOD-NS, or SIT scores.
CONCLUSIONS: Differences in objective measures of CRS severity exist among PwCF related to sex, age, and ETI use. Variant status and race did not influence patient-reported CRS severity measures or olfaction in this study. Understanding how these factors impact response to treatment may improve care disparities among PwCF.
RESULTS: NCT04469439.
方法:在前瞻性中,多机构研究,成人PwCF完成了22个问题的鼻窦结果测试(SNOT-22),气味识别测试(SIT),嗅觉障碍负面陈述问卷(QOD-NS),和囊性纤维化问卷修订(CFQ-R)。Lund-Kennedy得分,鼻窦计算机断层扫描,收集临床资料。跨种族/族裔分析数据,性别,和社会经济因素使用多元回归。
结果:参加了73例PwCF,平均年龄为34.7±10.9岁,女性为49例(67.1%)。线性回归确定elexacaftor/tezacaftor/ivacaftor(ETI)使用(β=-4.09,95%置信区间[CI][-6.08,-2.11],p<0.001),女性(β=-2.14,95%CI[-4.11,-0.17],p=0.034),年龄增加(β=-0.14,95%CI[-0.22,-0.05],p=0.003)与较低/较好的内窥镜检查评分相关。私人医疗保险(β=17.76,95%CI[5.20,30.32],p=0.006)和>16个教育年限(β=13.50,95%CI[2.21,24.80],p=0.020)与较高的基线百分比在一秒内预测用力呼气量(ppFEV1)相关。Medicaid/Medicare保险与内窥镜检查评分较差有关,CFQ-R呼吸评分,和ppFEV1(所有p<0.017),西班牙裔/拉丁裔种族与更差的SNOT-22得分相关(p=0.047),在调整其他辅因子之前。无其他SDS因子与SNOT-22、QOD-NS、或SIT得分。
结论:与性别相关的PwCF在CRS严重程度的客观测量方面存在差异,年龄,和ETI使用。在这项研究中,变异状态和种族不影响患者报告的CRS严重程度或嗅觉。了解这些因素如何影响对治疗的反应可能会改善PwCF之间的护理差异。
结果:NCT04469439。