背景:目前,关于α1抗胰蛋白酶缺乏症(AATD)的有效管理存在相互矛盾的信息和指导。建立针对AATD的评估和疾病管理共识对于实现标准化治疗途径和改善患者预后非常重要。这里,我们的目标是利用Delphi方法为严重AATD患者的评估和管理建立欧洲共识.
方法:由欧洲Alpha-1研究合作组织(EARCO)的成员在线完成了两轮Delphi调查。受访者被要求表明他们同意为没有呼吸道症状的患者提出的陈述,稳定的呼吸系统疾病,使用Likert量表1-7,呼吸系统疾病恶化。受访者之间的协议水平是使用加权平均值计算的。
结果:Delphi调查的第1轮已发送给EARCO的103名成员,来自15个国家的38/103(36.9%)肺科医师完成了所有109个问题。第2轮发送给所有完成第1轮的人,第36/38轮(94.7%)完成了所有79个问题。关于肺活量测定的反应,身体体积描记术,高分辨率计算机断层扫描,增强疗法的开始在医生之间显示出很小的差异,但是其他方面存在不一致,例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。
结论:这些结果为评估和监测严重AATD患者提供了专家意见,可用于为欧洲各地的患者提供更新的建议和标准化的治疗途径。
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.