Assessment and monitoring of lung disease in patients with severe alpha 1 antitrypsin deficiency: a european delphi consensus of the EARCO group.

IF 5.8 2区 医学 Q1 Medicine Respiratory Research Pub Date : 2024-08-19 DOI:10.1186/s12931-024-02929-5
Marc Miravitlles, Alice M Turner, Maria Sucena, Jean-François Mornex, Timm Greulich, Marion Wencker, N Gerard McElvaney
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Abstract

Background: Currently, there is conflicting information and guidance on the effective management of Alpha 1 Antitrypsin Deficiency (AATD). Establishing a consensus of assessment and disease management specific to AATD is important for achieving a standardized treatment pathway and for improving patient outcomes. Here, we aim to utilize the Delphi method to establish a European consensus for the assessment and management of patients with severe AATD.

Methods: Two rounds of a Delphi survey were completed online by members of the European Alpha-1 Research Collaboration (EARCO). Respondents were asked to indicate their agreement with proposed statements for patients with no respiratory symptoms, stable respiratory disease, and worsening respiratory disease using a Likert scale of 1-7. Levels of agreement between respondents were calculated using a weighted average.

Results: Round 1 of the Delphi survey was sent to 103 members of EARCO and 38/103 (36.9%) pulmonologists from across 15 countries completed all 109 questions. Round 2 was sent to all who completed Round 1 and 36/38 (94.7%) completed all 79 questions. Responses regarding spirometry, body plethysmography, high-resolution computed tomography, and the initiation of augmentation therapy showed little variability among physicians, but there was discordance among other aspects, such as the use of low-dose computed tomography in both a research setting and routine clinical care.

Conclusions: These results provide expert opinions for the assessment and monitoring of patients with severe AATD, which could be used to provide updated recommendations and standardized treatment pathways for patients across Europe.

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严重α1 抗胰蛋白酶缺乏症患者肺部疾病的评估和监测:EARCO 小组达成的欧洲德尔菲共识。
背景:目前,关于如何有效管理α1 抗胰蛋白酶缺乏症(AATD)的信息和指导存在冲突。就针对 AATD 的评估和疾病管理达成共识对于实现标准化治疗路径和改善患者预后非常重要。在此,我们希望利用德尔菲法就严重 AATD 患者的评估和管理达成欧洲共识:方法:欧洲阿尔法-1 研究合作组织 (EARCO) 的成员在线完成了两轮德尔菲调查。调查要求受访者用 1-7 分的李克特量表来表示他们是否同意针对无呼吸道症状、呼吸道疾病稳定和呼吸道疾病恶化患者提出的声明。受访者之间的同意程度采用加权平均法计算:第一轮德尔菲调查发送给了 103 位 EARCO 成员,来自 15 个国家的 38/103 位(36.9%)肺病学专家完成了全部 109 个问题。第二轮调查发给了所有完成第一轮调查的人员,36/38(94.7%)人完成了全部 79 个问题。医生们对肺活量测定、体褶造影术、高分辨率计算机断层扫描和启动增强疗法的回答几乎没有差异,但在其他方面,如在研究环境和常规临床护理中使用低剂量计算机断层扫描方面,则存在分歧:这些结果为评估和监测严重急性肺动脉高压症患者提供了专家意见,可用于为欧洲各地的患者提供最新建议和标准化治疗路径。
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来源期刊
Respiratory Research
Respiratory Research RESPIRATORY SYSTEM-
CiteScore
9.70
自引率
1.70%
发文量
314
审稿时长
4-8 weeks
期刊介绍: Respiratory Research publishes high-quality clinical and basic research, review and commentary articles on all aspects of respiratory medicine and related diseases. As the leading fully open access journal in the field, Respiratory Research provides an essential resource for pulmonologists, allergists, immunologists and other physicians, researchers, healthcare workers and medical students with worldwide dissemination of articles resulting in high visibility and generating international discussion. Topics of specific interest include asthma, chronic obstructive pulmonary disease, cystic fibrosis, genetics, infectious diseases, interstitial lung diseases, lung development, lung tumors, occupational and environmental factors, pulmonary circulation, pulmonary pharmacology and therapeutics, respiratory immunology, respiratory physiology, and sleep-related respiratory problems.
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