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超低剂量胸部CT联合共同生产在囊性纤维化护理中的初步研究。

A pilot study of ultra-low-dose chest CT combined with co-production in cystic fibrosis care.

作者信息

Zhang Richard, Phelps Andrew, MacDonald Kelvin, Stone Anne

机构信息

Larner College of Medicine, University of Vermont, Burlington, VT, USA.

Division of Pediatric Radiology, Department of Diagnostic Radiology, Oregon Health and Science University, Portland, OR, USA.

出版信息

Pediatr Res. 2025 Sep 18. doi: 10.1038/s41390-025-04379-1.

Abstract

BACKGROUND

This pilot study determined whether ultra-low-dose chest tomography (ULDCT) is a feasible tool to assess structural airway abnormalities in adolescents and young adults with cystic fibrosis (CF) taking elexacaftor/tezacaftor/ivacaftor (ETI). We explored if reviewing ULDCT findings with people with cystic fibrosis (PwCF) would impact adherence and satisfaction with airway clearance therapy (ACT).

METHODS

PwCF aged 12-25 years taking ETI underwent ULDCT and completed surveys on ACT and medication adherence and satisfaction. Participants reviewed ULDCT findings with a physician and completed follow-up surveys 8-16 weeks later.

RESULTS

In all, 20 subjects (45% male, median age 18 years, median body mass index 22.7 kg/m, and 45% F508del homozygous) completed baseline questionnaires and 17 completed ULDCT (median dose length product 6.6 milligray.cm) and post-ULDCT surveys. Findings revealed 13 subjects had bronchiectasis. Baseline surveys revealed 50% of participants reported not completing ACT the week prior to enrollment. Post-ULDCT, 82% reported completing ACT in the prior week. Reported unintentional and purposeful nonadherence to ACT decreased post-ULDCT.

CONCLUSION

ULDCT is feasible for assessing structural lung abnormalities in adolescents and young adults with CF taking ETI. Incorporating ULDCT with co-production techniques may improve patient satisfaction and align the treatment goals between PwCF and their care team.

IMPACT

This study demonstrates the utility of ultra-low-dose chest CT (ULDCT) to assess structural airway abnormalities in people with CF (PwCF) taking elexacaftor/tezacaftor/ivacaftor (ETI) at risk for airway disease. Findings add to the literature of alternative imaging methods in CF. ULDCT identified a high rate of bronchiectasis among PwCF taking ETI in this study. Data suggest a role for ULDCT in the co-production of ACT recommendations in this population. Incorporating ULDCT with co-production techniques may help align the treatment goals of PwCF and their care team.

摘要

背景

这项初步研究确定了超低剂量胸部计算机断层扫描(ULDCT)是否是评估接受依列卡福/替扎卡福/依伐卡福(ETI)治疗的青少年和年轻囊性纤维化(CF)患者气道结构异常的可行工具。我们探讨了与囊性纤维化患者(PwCF)一起查看ULDCT结果是否会影响其对气道清除治疗(ACT)的依从性和满意度。

方法

年龄在12 - 25岁、接受ETI治疗的PwCF接受ULDCT检查,并完成关于ACT、药物依从性和满意度的调查。参与者与医生一起查看ULDCT结果,并在8 - 16周后完成随访调查。

结果

共有20名受试者(45%为男性,中位年龄18岁,中位体重指数22.7kg/m²,45%为F508del纯合子)完成了基线问卷,17名完成了ULDCT检查(中位剂量长度乘积为6.6毫戈瑞·厘米)和ULDCT检查后的调查。结果显示13名受试者有支气管扩张。基线调查显示,50%的参与者报告在入组前一周未完成ACT。ULDCT检查后,82%的人报告在前一周完成了ACT。报告的无意和故意不依从ACT的情况在ULDCT检查后有所减少。

结论

ULDCT对于评估接受ETI治疗的青少年和年轻CF患者的肺部结构异常是可行的。将ULDCT与共同生产技术相结合可能会提高患者满意度,并使PwCF与其护理团队之间的治疗目标保持一致。

影响

本研究证明了超低剂量胸部CT(ULDCT)在评估有气道疾病风险的接受依列卡福/替扎卡福/依伐卡福(ETI)治疗的CF患者(PwCF)气道结构异常方面的实用性。研究结果丰富了CF替代成像方法的文献。在本研究中,ULDCT在接受ETI治疗的PwCF中发现了较高的支气管扩张率。数据表明ULDCT在为该人群共同制定ACT建议中可发挥作用。将ULDCT与共同生产技术相结合可能有助于使PwCF与其护理团队的治疗目标保持一致。

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