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管理老龄化囊性纤维化患者群体:挑战与优先事项。

Managing an ageing cystic fibrosis population: challenges and priorities.

作者信息

Frost Freddy J, Peckham Daniel G, Felton Imogen C, Snowball Joanna E, Gray Robert D, Jones Andrew M, Simmonds Nicholas J, Lord Robert W, Lip Gregory Y H, Chandler Hannah, Murphy Kevin, Downey Damian G, Sheppard David N, Davies Jane C, Bull Jane, Sommer Paula, Cupid Belinda, Allen Lucy, Duckers Jamie

机构信息

Adult Cystic Fibrosis Centre Liverpool Heart and Chest Hospital NHS Foundation Trust, Liverpool, UK

Liverpool Centre for Cardiovascular Sciences at University of Liverpool, Liverpool John Moores University and Liverpool Heart and Chest Hospital NHS Foundation Trust, Liverpool, UK.

出版信息

Eur Respir Rev. 2025 May 14;34(176). doi: 10.1183/16000617.0261-2024. Print 2025 Apr.

DOI:10.1183/16000617.0261-2024
PMID:40368426
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12076158/
Abstract

The increasing life expectancy of people with cystic fibrosis (pwCF), largely driven by advancements in early diagnosis, multidisciplinary care and the recent introduction of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies, is likely to herald a shift in the focus of care toward managing the complexities of ageing. This review highlights key challenges and research priorities for addressing the health needs of an ageing CF population. A growing body of evidence underscores the heightened risks of cancers, cardiovascular diseases and changing nutritional and metabolic profiles as pwCF age. CFTR modulators have improved clinical outcomes, but their effects on inflammation, immunity and long-term disease trajectories remain incompletely understood. Nutritional management, particularly the implications of obesity and body composition, poses new challenges, as does the potential accelerated ageing of immune and pulmonary systems in CF. Emerging issues such as menopause in females with CF, lifetime antimicrobial resistance and the interplay between chronic inflammation and ageing further complicate the care landscape. The review emphasises the urgent need for multidisciplinary research programmes that integrate clinical, patient and community perspectives. Leveraging established CF registries, clinical trial networks and collaborations with ageing research frameworks is critical to addressing these challenges. Ultimately, the goal is to ensure that pwCF not only live longer but also experience improved quality of life and holistic wellbeing as they realise the full benefits of therapeutic advances.

摘要

囊性纤维化患者(pwCF)预期寿命的增加,很大程度上得益于早期诊断、多学科护理的进步以及近期引入的囊性纤维化跨膜传导调节因子(CFTR)调节剂疗法,这可能预示着护理重点将转向应对老龄化带来的复杂性。本综述强调了应对老龄化囊性纤维化人群健康需求的关键挑战和研究重点。越来越多的证据表明,随着pwCF年龄的增长,患癌症、心血管疾病的风险增加,营养和代谢状况也在发生变化。CFTR调节剂改善了临床结果,但其对炎症、免疫和长期疾病轨迹的影响仍不完全清楚。营养管理,尤其是肥胖和身体成分的影响,带来了新的挑战,CF中免疫和肺部系统潜在的加速衰老也是如此。诸如CF女性的更年期、终身抗菌耐药性以及慢性炎症与衰老之间的相互作用等新出现的问题,进一步使护理情况复杂化。该综述强调迫切需要开展整合临床、患者和社区观点的多学科研究项目。利用现有的囊性纤维化登记系统、临床试验网络以及与老龄化研究框架的合作,对于应对这些挑战至关重要。最终目标是确保pwCF不仅寿命更长,而且在充分受益于治疗进展的同时,生活质量和整体幸福感得到改善。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9741/12076158/be0a172d0041/ERR-0261-2024.02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9741/12076158/2bb5c90b5125/ERR-0261-2024.01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9741/12076158/be0a172d0041/ERR-0261-2024.02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9741/12076158/2bb5c90b5125/ERR-0261-2024.01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9741/12076158/be0a172d0041/ERR-0261-2024.02.jpg

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本文引用的文献

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Cardiometabolic risk factors in adults with cystic fibrosis undergoing elexacaftor/tezacaftor/ivacaftor therapy.接受依列卡福妥/替扎卡福妥/依伐卡托治疗的成年囊性纤维化患者的心脏代谢危险因素。
J Cyst Fibros. 2025 Jan;24(1):53-56. doi: 10.1016/j.jcf.2024.11.009. Epub 2024 Dec 6.
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Development of metabolic syndrome in people with Cystic Fibrosis one year after exposure to elexacaftor-tezacaftor-ivacaftor.暴露于依列卡福妥-替扎卡福妥-依伐卡福妥一年后囊性纤维化患者代谢综合征的发生情况
J Cyst Fibros. 2025 Jan;24(1):47-52. doi: 10.1016/j.jcf.2024.09.022. Epub 2024 Oct 16.
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Longitudinal changes in BMD in adults with cystic fibrosis.
成年囊性纤维化患者骨密度的纵向变化。
J Bone Miner Res. 2024 Nov 29;39(12):1716-1721. doi: 10.1093/jbmr/zjae139.
4
ECFS standards of care on CFTR-related disorders: Identification and care of the disorders.欧洲囊性纤维化协作组(ECFS)关于 CFTR 相关疾病的护理标准:疾病的识别和护理。
J Cyst Fibros. 2024 Jul;23(4):590-602. doi: 10.1016/j.jcf.2024.03.008. Epub 2024 Mar 19.
5
Prioritizing sexual and reproductive health research and care for people with cystic fibrosis: A 2023 workshop report from the Cystic Fibrosis Foundation Sexual Health, Reproduction, and Gender (SHARING) Research Working Group.优先考虑囊性纤维化患者的性健康和生殖健康研究与护理:囊性纤维化基金会性健康、生殖和性别(SHARING)研究工作组 2023 年研讨会报告。
J Cyst Fibros. 2024 Jul;23(4):639-646. doi: 10.1016/j.jcf.2024.03.007. Epub 2024 Mar 13.
6
Cystic Fibrosis in Adults: A Paradigm of Frailty Syndrome? An Observational Study.成人囊性纤维化:虚弱综合征的范例?一项观察性研究。
J Clin Med. 2024 Jan 19;13(2):585. doi: 10.3390/jcm13020585.
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The Trajectory of Successful Aging: Insights from Metagenome and Cytokine Profiling.成功老龄化的轨迹:来自宏基因组和细胞因子分析的见解。
Gerontology. 2024;70(4):390-407. doi: 10.1159/000536082. Epub 2024 Jan 19.
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ESPEN-ESPGHAN-ECFS guideline on nutrition care for cystic fibrosis.ESPEN-ESPGHAN-ECFS 指南:囊性纤维化的营养护理。
Clin Nutr. 2024 Feb;43(2):413-445. doi: 10.1016/j.clnu.2023.12.017. Epub 2023 Dec 27.
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