管理老龄化囊性纤维化人群:挑战和优先事项。

IF 9 1区 医学 Q1 RESPIRATORY SYSTEM
European Respiratory Review Pub Date : 2025-05-14 Print Date: 2025-04-01 DOI:10.1183/16000617.0261-2024
Freddy J Frost, Daniel G Peckham, Imogen C Felton, Joanna E Snowball, Robert D Gray, Andrew M Jones, Nicholas J Simmonds, Robert W Lord, Gregory Y H Lip, Hannah Chandler, Kevin Murphy, Damian G Downey, David N Sheppard, Jane C Davies, Jane Bull, Paula Sommer, Belinda Cupid, Lucy Allen, Jamie Duckers
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引用次数: 0

摘要

早期诊断、多学科治疗和最近引入的囊性纤维化跨膜传导调节剂(CFTR)疗法的进步,很大程度上推动了囊性纤维化(pwCF)患者预期寿命的增加,这可能预示着护理重点向管理衰老复杂性的转变。本综述强调了解决老龄CF人群健康需求的主要挑战和研究重点。越来越多的证据强调,随着pwCF年龄的增长,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,尤其是肥胖和身体组成的影响,带来了新的挑战,CF患者免疫和肺系统的潜在加速衰老也带来了新的挑战。CF女性患者的更年期、终生抗微生物药物耐药性以及慢性炎症和衰老之间的相互作用等新出现的问题进一步使护理前景复杂化。这篇综述强调迫切需要多学科研究规划,将临床、患者和社区的观点结合起来。利用已建立的CF注册、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。最终的目标是确保pwCF不仅活得更长,而且在他们意识到治疗进步的全部好处时,体验到更高的生活质量和整体健康。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Managing an ageing cystic fibrosis population: challenges and priorities.

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.

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来源期刊
European Respiratory Review
European Respiratory Review Medicine-Pulmonary and Respiratory Medicine
CiteScore
14.40
自引率
1.30%
发文量
91
审稿时长
24 weeks
期刊介绍: The European Respiratory Review (ERR) is an open-access journal published by the European Respiratory Society (ERS), serving as a vital resource for respiratory professionals by delivering updates on medicine, science, and surgery in the field. ERR features state-of-the-art review articles, editorials, correspondence, and summaries of recent research findings and studies covering a wide range of topics including COPD, asthma, pulmonary hypertension, interstitial lung disease, lung cancer, tuberculosis, and pulmonary infections. Articles are published continuously and compiled into quarterly issues within a single annual volume.
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