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2047 例患者队列中支气管扩张症的病因。西班牙历史支气管扩张症登记处的分析。

Etiology of Bronchiectasis in a Cohort of 2047 Patients. An Analysis of the Spanish Historical Bronchiectasis Registry.

机构信息

Servicio de Neumología, Hospital Regional Universitario de Málaga, Instituto de Biomedicina de Málaga (IBIMA), Facultad de Medicina, Universidad de Málaga, Málaga, España.

Unidad de Neumología, Agencia Sanitaria Costa del Sol, Marbella, Málaga, España.

出版信息

Arch Bronconeumol. 2017 Jul;53(7):366-374. doi: 10.1016/j.arbres.2016.12.003. Epub 2017 Jan 21.

Abstract

INTRODUCTION

Bronchiectasis is caused by many diseases. Establishing its etiology is important for clinical and prognostic reasons. The aim of this study was to evaluate the etiology of bronchiectasis in a large patient sample and its possible relationship with demographic, clinical or severity factors, and to analyze differences between idiopathic disease, post-infectious disease, and disease caused by other factors.

METHODS

Multicenter, cross-sectional study of the SEPAR Spanish Historical Registry (RHEBQ-SEPAR). Adult patients with bronchiectasis followed by pulmonologists were included prospectively. Etiological studies were based on guidelines and standardized diagnostic tests included in the register, which were later included in the SEPAR guidelines on bronchiectasis.

RESULTS

A total of 2,047 patients from 36 Spanish hospitals were analyzed. Mean age was 64.9years and 54.9% were women. Etiology was identified in 75.8% of cases (post-Infection: 30%; cystic fibrosis: 12.5%; immunodeficiencies: 9.4%; COPD: 7.8%; asthma: 5.4%; ciliary dyskinesia: 2.9%, and systemic diseases: 1.4%). The different etiologies presented different demographic, clinical, and microbiological factors. Post-infectious bronchiectasis and bronchiectasis caused by COPD and asthma were associated with an increased risk of poorer lung function. Patients with post-infectious bronchiectasis were older and were diagnosed later. Idiopathic bronchiectasis was more common in female non-smokers and was associated with better lung function, a higher body mass index, and a lower rate of Pseudomonas aeruginosa than bronchiectasis of known etiology.

CONCLUSIONS

The etiology of bronchiectasis was identified in a large proportion of patients included in the RHEBQ-SEPAR registry. Different phenotypes associated with different causes could be identified.

摘要

简介

支气管扩张症由多种疾病引起。确定其病因对于临床和预后均具有重要意义。本研究旨在评估大样本支气管扩张症患者的病因,并分析其与人口统计学、临床或严重程度因素的可能关系,同时分析特发性疾病、感染后疾病和其他因素引起的疾病之间的差异。

方法

这是一项多中心、回顾性的西班牙 SEPAR 历史注册研究(RHEBQ-SEPAR)。前瞻性纳入由肺病专家诊治的成年支气管扩张症患者。病因研究基于指南和纳入该注册的标准化诊断性检查,这些检查后来被纳入 SEPAR 支气管扩张症指南。

结果

共纳入来自西班牙 36 家医院的 2047 例患者。患者平均年龄为 64.9 岁,54.9%为女性。75.8%的患者确定了病因(感染后:30%;囊性纤维化:12.5%;免疫缺陷:9.4%;COPD:7.8%;哮喘:5.4%;纤毛运动障碍:2.9%,以及全身性疾病:1.4%)。不同病因具有不同的人口统计学、临床和微生物学特征。感染后支气管扩张症以及由 COPD 和哮喘引起的支气管扩张症与肺功能更差相关。感染后支气管扩张症患者年龄更大且诊断时间更晚。特发性支气管扩张症在女性非吸烟者中更为常见,与已知病因的支气管扩张症相比,其肺功能更好、体质量指数更高且铜绿假单胞菌定植率更低。

结论

在 RHEBQ-SEPAR 注册研究中纳入的患者中,有很大一部分患者确定了病因。可以识别出与不同病因相关的不同表型。

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