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欧洲α-抗胰蛋白酶缺乏症的诊断与管理:一项专家调查。

Diagnosis and management of α-antitrypsin deficiency in Europe: an expert survey.

作者信息

Horváth Ildikó, Canotilho Maria, Chlumský Jan, Chorostowska-Wynimko Joanna, Corda Luciano, Derom Eric, Ficker Joachim H, Kneussl Meinhard, Miravitlles Marc, Sucena Maria, Thabut Gabriel, Turner Alice M, van 't Wout Emily, McElvaney N Gerard

机构信息

Dept of Pulmonology, National Koranyi Institute for Pulmonology in Budapest, Budapest, Hungary.

Dept of Pneumology, Hospital of Santo Andre - Centro Hospitalar de Leiria, Leiria, Portugal.

出版信息

ERJ Open Res. 2019 Mar 11;5(1). doi: 10.1183/23120541.00171-2018. eCollection 2019 Feb.

Abstract

Despite recent improvements, α-antitrypsin deficiency (AATD) remains a rarely diagnosed and treated condition. To assess the variability of AATD diagnosis/treatment in Europe, and to evaluate clinicians' views on methods to optimise management, specialist AATD clinicians were invited to complete a web-based survey. Surveys were completed by 15 physicians from 14 centres in 13 European countries. All respondents perceived the AATD diagnosis rate to be low in their country; 77% of physicians believed that ∼15% of cases were diagnosed. Low awareness was perceived as the greatest barrier to diagnosis. Spirometry was considered more practical than quantitative computed tomography (QCT) for monitoring AATD patients in clinical practice; QCT was considered more useful in trials. AAT therapy provision was reported to be highly variable: France and Germany were reported to treat the highest proportion (∼60%) of diagnosed patients, in contrast to the UK and Hungary, where virtually no patients receive AAT therapy. Most clinicians supported self-administration and extended dosing intervals to improve convenience of AAT therapy. This survey indicates that AATD diagnosis and management are highly heterogeneous in Europe; European cooperation is essential to generate data to support access to AAT therapy. Improving convenience of AAT therapy is an ongoing objective.

摘要

尽管最近有所改善,但α-抗胰蛋白酶缺乏症(AATD)仍然是一种很少被诊断和治疗的疾病。为了评估欧洲AATD诊断/治疗的可变性,并评估临床医生对优化管理方法的看法,邀请了AATD专科临床医生完成一项基于网络的调查。来自13个欧洲国家14个中心的15名医生完成了调查。所有受访者都认为本国的AATD诊断率很低;77%的医生认为约15%的病例得到了诊断。低认知度被认为是诊断的最大障碍。在临床实践中,肺功能测定被认为比定量计算机断层扫描(QCT)更实用,用于监测AATD患者;QCT在试验中被认为更有用。据报道,AAT治疗的提供差异很大:据报道,法国和德国治疗的确诊患者比例最高(约60%),而英国和匈牙利几乎没有患者接受AAT治疗。大多数临床医生支持自我给药和延长给药间隔以提高AAT治疗的便利性。这项调查表明,欧洲的AATD诊断和管理存在很大差异;欧洲合作对于生成支持获得AAT治疗的数据至关重要。提高AAT治疗的便利性是一个持续的目标。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4636/6409083/d84c2548b2b5/00171-2018.01.jpg

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