Division of Pulmonology, Cardiothoracic and Vascular Department, AOU Città della Salute e della Scienza, Turin, Italy -
General and Specialistic Medical Department, Division of Gastroenterology, AOU Città della Salute e della Scienza, Turin, Italy.
Panminerva Med. 2018 Sep;60(3):101-108. doi: 10.23736/S0031-0808.18.03450-X. Epub 2018 Apr 24.
Alpha-1 antitrypsin deficiency (AATD) is a clinically under-recognized inherited disorder affecting the lungs and the liver. The most common manifestations are pulmonary emphysema, bronchiectasis and liver disease. The recent publication of the European Respiratory Society statement on diagnosis and treatment of pulmonary diseases has replaced the 2003 American Thoracic Society and European Respiratory Society one. New outcome parameters have been introduced and validated by observational and randomized clinical trials, and new information about efficacy and safety of augmentation therapy have been published. In this narrative review we have commented the crucial points of the new European Respiratory Society statement on AATD, including a review of the literature on liver involvement and treatments.
α1-抗胰蛋白酶缺乏症（AATD）是一种临床认识不足的遗传性疾病，影响肺部和肝脏。最常见的表现为肺气肿、支气管扩张和肝病。最近发表的欧洲呼吸学会关于肺部疾病的诊断和治疗的声明取代了 2003 年美国胸科学会和欧洲呼吸学会的声明。新的观察性和随机临床试验引入并验证了新的结局参数，并发表了关于增敏治疗的疗效和安全性的新信息。在这篇叙述性综述中，我们对新的欧洲呼吸学会关于 AATD 的声明的关键点进行了评论，包括对肝脏受累和治疗的文献回顾。
