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非囊性纤维化支气管扩张症成人表型分析:一项前瞻性观察队列研究。

Phenotyping adults with non-cystic fibrosis bronchiectasis: a prospective observational cohort study.

机构信息

Department of Respiratory Medicine, Northumbria Healthcare Trust, Newcastle, UK.

出版信息

Respir Med. 2013 Jul;107(7):1001-7. doi: 10.1016/j.rmed.2013.04.013. Epub 2013 May 11.

DOI:10.1016/j.rmed.2013.04.013
PMID:23672995
Abstract

BACKGROUND

Bronchiectasis is the outcome of a number of different airway insults. Very few studies have characterised the aetiology and utility of a dedicated screening proforma in adult patients attending a general bronchiectasis clinic.

METHODS

A prospective observational study of 189 bronchiectasis patients attending two centres in the North East of England over a two-year period was performed.

RESULTS

The aetiology of bronchiectasis was identified in 107/189(57%) patients. Idiopathic bronchiectasis (IB) represented the largest subgroup (43%). Post-infection bronchiectasis (PIB) constituted the largest proportion (24%) of known causes. Mean age (SD) at diagnosis was 54(20) years with a mean age at symptom onset of 37(24) years, accounting for a diagnostic delay of 17 years. Age of symptom onset was significantly younger in patients with PIB compared to IB (p < 0.0001) and in Pseudomonas sputum positive patients (p = 0.007). Screening for APBA and total immunoglobulin deficiency identified 9 (5%) patients who then had tailored treatment. Routine screening for other aetiologies was deemed unnecessary.

CONCLUSION

IB and PIB accounted for two thirds of cases of bronchiectasis in a general population. We recommend routine screening for ABPA and total immunoglobulin deficiency but not for other rarer aetiologies.

摘要

背景

支气管扩张是多种不同气道损伤的结果。很少有研究描述了专门的筛查方案在成人支气管扩张症门诊患者中的病因和应用价值。

方法

对在英格兰东北部的两个中心就诊的 189 例支气管扩张症患者进行了为期两年的前瞻性观察研究。

结果

在 189 例患者中,107 例(57%)确定了支气管扩张症的病因。特发性支气管扩张症(IB)是最大的亚组(43%)。感染后支气管扩张症(PIB)构成了已知病因的最大比例(24%)。诊断时的平均年龄(标准差)为 54(20)岁,症状起始时的平均年龄为 37(24)岁,说明存在 17 年的诊断延迟。与 IB 相比,PIB 患者的症状起始年龄明显更年轻(p<0.0001),与铜绿假单胞菌痰阳性患者相比(p=0.007)也是如此。针对 ABPA 和总免疫球蛋白缺乏症的筛查发现了 9 例(5%)患者,然后为其提供了针对性的治疗。常规筛查其他病因被认为是不必要的。

结论

在普通人群中,IB 和 PIB 占支气管扩张症的三分之二。我们建议常规筛查 ABPA 和总免疫球蛋白缺乏症,但不筛查其他罕见病因。

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