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支气管扩张症。

Bronchiectasis.

机构信息

Section of Pulmonary and Critical Care Medicine, University of Chicago, Chicago, IL.

Section of Pulmonary and Critical Care Medicine, University of Pennsylvania, Philadelphia, PA.

出版信息

Chest. 2019 Apr;155(4):825-833. doi: 10.1016/j.chest.2018.10.027. Epub 2018 Nov 4.

Abstract

Bronchiectasis is an important clinical syndrome because of its increasing prevalence, substantial economic burden on health care, and associated morbidity. Until recently, the disease was considered an orphan and essentially neglected from a therapeutic standpoint, but many recent advances have been made in the field. Several national registries have formed to provide databases from which to study patients with bronchiectasis. Experts published a consensus definition of a bronchiectasis-specific exacerbation that will serve as a unified definition for future clinical trials. Several inhaled antibiotic trials aimed at reducing exacerbation frequency have been completed. Researchers have investigated nonculture techniques, such as 16S ribosomal RNA (rRNA) and whole genome sequencing, to characterize the microbiological characteristics. Studies of anti-Pseudomonas antibodies are providing interesting insight into varying host responses to chronic Pseudomonas infection. After three successful trials demonstrating that macrolides reduce exacerbations in bronchiectasis, other antiinflammatory agents have been investigated, and a trial of a novel antiinflammatory drug is ongoing. A relatively robust study has been published in airway clearance, a therapy that is accepted universally as beneficial but that has never been accompanied by strong evidence. To build on the successes with bronchiectasis thus far, investigators must develop better definitions of phenotypes of bronchiectasis. In this regard, clinical tools have been developed to quantify disease severity and predict prognosis. Studies of different clinical phenotypes of bronchiectasis in patients with bronchiectasis have been published. With continued advances in the field of bronchiectasis, there is hope that evidenced-based therapies will become available.

摘要

支气管扩张症是一种重要的临床综合征,因为它的发病率不断增加,给医疗保健带来了巨大的经济负担,并与发病率相关。直到最近,该疾病才被认为是一种罕见病,从治疗的角度来看基本上被忽视了,但在该领域已经取得了许多新的进展。已经形成了几个国家登记处,提供数据库,以便对支气管扩张症患者进行研究。专家发表了支气管扩张症特异性加重的共识定义,该定义将作为未来临床试验的统一定义。已经完成了几项旨在减少加重频率的吸入抗生素试验。研究人员已经研究了非培养技术,例如 16S 核糖体 RNA(rRNA)和全基因组测序,以表征微生物特征。针对抗铜绿假单胞菌抗体的研究为宿主对慢性铜绿假单胞菌感染的不同反应提供了有趣的见解。在三项成功的试验表明大环内酯类药物可减少支气管扩张症的加重后,已经研究了其他抗炎药,并且正在进行一种新型抗炎药的试验。一项关于气道清除的研究发表在支气管扩张症中,这是一种被普遍认为有益但从未有过有力证据支持的治疗方法。为了在迄今为止的支气管扩张症方面取得成功,研究人员必须开发出更好的支气管扩张症表型定义。在这方面,已经开发出了量化疾病严重程度和预测预后的临床工具。已经发表了关于支气管扩张症患者不同临床表型的研究。随着支气管扩张症领域的不断进步,有望获得基于证据的治疗方法。

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