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J Cyst Fibros. 2022 Sep;21(5):784-791. doi: 10.1016/j.jcf.2022.01.011. Epub 2022 Jan 24.
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Triple Therapy for Cystic Fibrosis -Gating and -Residual Function Genotypes.囊性纤维化-门控和-残留功能基因型的三联疗法。
N Engl J Med. 2021 Aug 26;385(9):815-825. doi: 10.1056/NEJMoa2100665.
3
Telemedicine and cystic fibrosis: Do we still need face-to-face clinics?远程医疗和囊性纤维化:我们还需要面对面的诊所吗?
Paediatr Respir Rev. 2022 Jun;42:23-28. doi: 10.1016/j.prrv.2021.05.002. Epub 2021 May 19.
4
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Can Fam Physician. 2021 Jun;67(6):439-448. doi: 10.46747/cfp.6706439.
5
Cystic Fibrosis.囊性纤维化。
Pediatr Rev. 2021 Feb;42(2):55-67. doi: 10.1542/pir.2019-0212.
6
Elexacafator/tezacaftor/ivacaftor resolves subfertility in females with CF: A two center case series.Elexacaftor/tezacaftor/ivacaftor 可解决 CF 女性的生育能力低下问题:两项中心病例系列研究。
J Cyst Fibros. 2021 May;20(3):399-401. doi: 10.1016/j.jcf.2020.12.011. Epub 2021 Jan 19.
7
The Effectiveness of Patient-Centred Medical Home-Based Models of Care versus Standard Primary Care in Chronic Disease Management: A Systematic Review and Meta-Analysis of Randomised and Non-Randomised Controlled Trials.以患者为中心的医疗之家模式与标准初级保健在慢性病管理中的效果比较:随机和非随机对照试验的系统评价和荟萃分析。
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Paediatr Respir Rev. 2022 Mar;41:23-29. doi: 10.1016/j.prrv.2020.07.009. Epub 2020 Aug 2.
9
The future of cystic fibrosis care: a global perspective.囊性纤维化护理的未来:全球视角。
Lancet Respir Med. 2020 Jan;8(1):65-124. doi: 10.1016/S2213-2600(19)30337-6. Epub 2019 Sep 27.
10
Specialty-care access for community health clinic patients: processes and barriers.社区健康诊所患者获得专科护理的情况:流程与障碍
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将初级保健纳入囊性纤维化管理。

Integrating Primary Care into the Management of Cystic Fibrosis.

机构信息

Alpert Medical School of Brown University, Providence, RI, USA.

出版信息

J Prim Care Community Health. 2023 Jan-Dec;14:21501319231173811. doi: 10.1177/21501319231173811.

DOI:10.1177/21501319231173811
PMID:37158604
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10176593/
Abstract

Over the last 50 years, cystic fibrosis has radically transformed from a fatal disease of infancy to a chronic disease of adulthood. By 2025 it is estimated that 70% of individuals with cystic fibrosis (iwCF) will be cared for in adult clinics. We believe the role of a dedicated primary care provider (PCP) for preventative care will be crucial for the longevity of iwCF. There are various models for incorporating primary care medicine into CF management, but no universally accepted standard exists. Ideally, the PCP and pulmonologist practice in a patientcentered medical home, given the growing evidence that these care models are associated with improved quality-of-life measures, mental health, and disease-specific outcomes. To improve engagement with primary care in CF, there needs to be a shift in education at the undergraduate medical education and provider training levels. Increasing the knowledge of CF-related illness is vital in fostering a close relationship between the PCP and their patient. To meet this need, primary care doctors will need tools and practical experiences in managing this rare condition. This can start being addressed by providing ample opportunities for the inclusion of PCPs into subspecialty clinics and through engagement with community providers through readily available didactics, seminars, and open lines of communication. As PCPs and CF clinicians, we feel that shifting the domain of preventative care to the expertise of a primary care physician will allow for a more CF-specific focus in subspecialty clinics and help prevent these vital health maintenance tasks from being overlooked, altogether advancing the health and well-being of iwCF.

摘要

在过去的 50 年里,囊性纤维化已经从婴儿期的致命疾病转变为成年期的慢性疾病。据估计,到 2025 年,70%的囊性纤维化(iwCF)患者将在成人诊所接受治疗。我们认为,专门的初级保健提供者(PCP)提供预防保健对于 iwCF 的长寿至关重要。有各种将初级保健医学纳入 CF 管理的模式,但没有普遍接受的标准。理想情况下,PCP 和肺病学家在以患者为中心的医疗之家中实践,因为越来越多的证据表明,这些护理模式与改善生活质量措施、心理健康和特定疾病结果相关。为了改善 CF 中对初级保健的参与,需要在本科医学教育和提供者培训层面上转变教育。增加对 CF 相关疾病的了解对于培养 PCP 与其患者之间的密切关系至关重要。为了满足这一需求,初级保健医生需要在管理这种罕见疾病方面的工具和实践经验。通过为 PCP 提供充分的机会参与专业诊所,并通过社区提供者的现成教学、研讨会和开放的沟通渠道与之互动,可以开始解决这个问题。作为 PCP 和 CF 临床医生,我们认为将预防保健领域转移到初级保健医生的专业领域,将允许在专业诊所更专注于 CF,并有助于防止这些重要的健康维护任务被忽视,从而整体上提高 iwCF 的健康和福祉。