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每一次呼吸中的希望:探索囊性纤维化的治疗前景

Hope in Every Breath: Navigating the Therapeutic Landscape of Cystic Fibrosis.

作者信息

Sreenivasulu Himabindu, Muppalla Sudheer Kumar, Vuppalapati Sravya, Shokrolahi Mohammad, Reddy Pulliahgaru Apeksha

机构信息

General Medicine, People's Education Society (PES) Institute of Medical Sciences and Research, Kuppam, IND.

Pediatrics, People's Education Society (PES) Institute of Medical Sciences and Research, Kuppam, IND.

出版信息

Cureus. 2023 Aug 16;15(8):e43603. doi: 10.7759/cureus.43603. eCollection 2023 Aug.

Abstract

Cystic fibrosis (CF) has long posed a complex challenge to medical science. Still, the tides are turning with remarkable progress in prognosis and demographics, thanks to cutting-edge medical management and treatment breakthroughs. It affects multiple systems, necessitating a comprehensive approach to its management. This article thoroughly reviews the latest advancements in CF treatment across three key areas: respiratory care, infection prevention, and pharmacological management. In respiratory care, emphasis is placed on airway clearance therapies and nebulized saline, while infection prevention strategies encompass hand hygiene, respiratory etiquette, and environmental cleaning and disinfection. Pharmacological management explores pancreatic enzyme replacement therapy (PERT), antimicrobial treatments, cystic fibrosis transmembrane regulator (CFTR) modulators, and promising gene therapies. Patient education and support are highlighted as crucial components of effective CF management, while mental health assessments are emphasized due to CF patients' susceptibility to anxiety and depression. This review highlights the tremendous progress made in the management of CF. Integrating early detection, infection prevention, pharmacological interventions, gene therapy, and patient support is revolutionizing the care and quality of life for individuals with CF.

摘要

囊性纤维化(CF)长期以来一直是医学面临的复杂挑战。不过,由于前沿的医疗管理和治疗突破,预后和人口统计学方面取得了显著进展,形势正在发生转变。它影响多个系统,因此需要采取综合管理方法。本文全面回顾了CF治疗在三个关键领域的最新进展:呼吸护理、感染预防和药物管理。在呼吸护理方面,重点是气道清除疗法和雾化生理盐水,而感染预防策略包括手部卫生、呼吸礼仪以及环境清洁和消毒。药物管理探讨了胰酶替代疗法(PERT)、抗菌治疗、囊性纤维化跨膜传导调节因子(CFTR)调节剂以及有前景的基因疗法。患者教育和支持被视为有效管理CF的关键组成部分,同时由于CF患者易患焦虑和抑郁,心理健康评估也受到重视。本综述突出了CF管理方面取得的巨大进展。整合早期检测、感染预防、药物干预、基因治疗和患者支持正在彻底改变CF患者的护理和生活质量。

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