α -1抗胰蛋白酶缺乏症患者的肺康复和生活质量：一项回顾性队列研究的结果

IF 7.7 1区医学 Q1 RESPIRATORY SYSTEM

Thorax Pub Date : 2025-09-18 DOI:10.1136/thorax-2025-223273

Fawaz Alwadani,Paul R Ellis,Michael Newnham,Joshua de Soyza,Aisha Butt,Anita Pye,Alice M Turner

{"title":"α -1抗胰蛋白酶缺乏症患者的肺康复和生活质量：一项回顾性队列研究的结果","authors":"Fawaz Alwadani,Paul R Ellis,Michael Newnham,Joshua de Soyza,Aisha Butt,Anita Pye,Alice M Turner","doi":"10.1136/thorax-2025-223273","DOIUrl":null,"url":null,"abstract":"Alpha-1 antitrypsin deficiency (AATD) is a rare genetic disorder associated with early-onset chronic obstructive pulmonary disease and impaired quality of life (QoL). This retrospective study evaluated the impact of pulmonary rehabilitation (PR) on QoL using repeated St George's Respiratory Questionnaire (SGRQ) scores. Among 274 patients, PR participants had more severe disease but showed no greater QoL improvement over time. Dyspnoea and exacerbation frequency were the strongest predictors of poorer outcomes. PR was not associated with significantly improved SGRQ trajectories. These findings highlight the need for personalised, disease-specific rehabilitation strategies in AATD to address limitations of conventional PR programmes in this population.","PeriodicalId":23284,"journal":{"name":"Thorax","volume":"78 1","pages":""},"PeriodicalIF":7.7000,"publicationDate":"2025-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Pulmonary rehabilitation and quality of life in alpha-1 antitrypsin deficiency: findings from a retrospective cohort study.\",\"authors\":\"Fawaz Alwadani,Paul R Ellis,Michael Newnham,Joshua de Soyza,Aisha Butt,Anita Pye,Alice M Turner\",\"doi\":\"10.1136/thorax-2025-223273\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Alpha-1 antitrypsin deficiency (AATD) is a rare genetic disorder associated with early-onset chronic obstructive pulmonary disease and impaired quality of life (QoL). This retrospective study evaluated the impact of pulmonary rehabilitation (PR) on QoL using repeated St George's Respiratory Questionnaire (SGRQ) scores. Among 274 patients, PR participants had more severe disease but showed no greater QoL improvement over time. Dyspnoea and exacerbation frequency were the strongest predictors of poorer outcomes. PR was not associated with significantly improved SGRQ trajectories. These findings highlight the need for personalised, disease-specific rehabilitation strategies in AATD to address limitations of conventional PR programmes in this population.\",\"PeriodicalId\":23284,\"journal\":{\"name\":\"Thorax\",\"volume\":\"78 1\",\"pages\":\"\"},\"PeriodicalIF\":7.7000,\"publicationDate\":\"2025-09-18\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Thorax\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1136/thorax-2025-223273\",\"RegionNum\":1,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"RESPIRATORY SYSTEM\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Thorax","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1136/thorax-2025-223273","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"RESPIRATORY SYSTEM","Score":null,"Total":0}

引用次数: 0

摘要

α -1抗胰蛋白酶缺乏症（AATD）是一种罕见的遗传性疾病，与早发性慢性阻塞性肺疾病和生活质量受损（QoL）相关。本回顾性研究使用重复的圣乔治呼吸问卷（SGRQ）评分评估肺康复（PR）对生活质量的影响。在274名患者中，PR参与者有更严重的疾病，但随着时间的推移，生活质量没有更大的改善。呼吸困难和加重频率是预后较差的最强预测因子。PR与SGRQ轨迹的显著改善无关。这些发现强调了在AATD中需要个性化的、针对疾病的康复策略，以解决传统PR计划在这一人群中的局限性。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

查看原文本刊更多论文

Pulmonary rehabilitation and quality of life in alpha-1 antitrypsin deficiency: findings from a retrospective cohort study.

Alpha-1 antitrypsin deficiency (AATD) is a rare genetic disorder associated with early-onset chronic obstructive pulmonary disease and impaired quality of life (QoL). This retrospective study evaluated the impact of pulmonary rehabilitation (PR) on QoL using repeated St George's Respiratory Questionnaire (SGRQ) scores. Among 274 patients, PR participants had more severe disease but showed no greater QoL improvement over time. Dyspnoea and exacerbation frequency were the strongest predictors of poorer outcomes. PR was not associated with significantly improved SGRQ trajectories. These findings highlight the need for personalised, disease-specific rehabilitation strategies in AATD to address limitations of conventional PR programmes in this population.

求助全文

通过发布文献求助，成功后即可免费获取论文全文。去求助

来源期刊

Thorax 医学-呼吸系统

CiteScore

16.10

自引率

2.00%

发文量

197

审稿时长

1 months

期刊介绍： Thorax stands as one of the premier respiratory medicine journals globally, featuring clinical and experimental research articles spanning respiratory medicine, pediatrics, immunology, pharmacology, pathology, and surgery. The journal's mission is to publish noteworthy advancements in scientific understanding that are poised to influence clinical practice significantly. This encompasses articles delving into basic and translational mechanisms applicable to clinical material, covering areas such as cell and molecular biology, genetics, epidemiology, and immunology.