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非囊性纤维化支气管扩张症:临床表现、诊断与治疗,以一家荷兰教学医院的数据为例

Non-cystic fibrosis bronchiectasis: clinical presentation, diagnosis and treatment, illustrated by data from a Dutch Teaching Hospital.

作者信息

Altenburg J, Wortel K, van der Werf T S, Boersma W G

机构信息

Department of Pulmonary Diseases, Medical Centre Alkmaar, Alkmaar, the Netherlands.

出版信息

Neth J Med. 2015 May;73(4):147-54.

PMID:25968285
Abstract

This review article describes the epidemiology, clinical presentation, diagnostic workup and treatment options in adult non-cystic fibrosis (non-CF) bronchiectasis (widening of mainly small and medium-sized bronchi as seen on chest computed tomography (CT) scan). We illustrate evidence from the literature with our own data retrieved from chart review, involving 236 adult patients with recurrent lower respiratory tract infections and high-resolution CT-proven non-CF bronchiectasis, who visited the outpatient clinic for respiratory diseases of a large Dutch teaching hospital between 2000 and 2010. Non-CF bronchiectasis can be described as a final common pathway of a vicious cycle of excessive bronchial inflammation, bacterial colonisation and infection. Non-CF bronchiectasis may arise from several causes, headed by infection and immunodeficiency, and is clinically characterised by a chronic, productive cough and infectious exacerbations. Once non-CF bronchiectasis is diagnosed using high-resolution CT scanning, a protocol-driven work-up to identify the underlying cause is recommended. Non-medicinal treatment options are primarily directed at clearance of bronchial secretions, which can further be improved by inhalation of hyperosmolar agents. Antibiotic treatment of exacerbations is a cornerstone medicinal treatment in bronchiectasis management. Patients with frequent exacerbations can be considered for long-term low-dose macrolide treatment, supported by robust evidence. Inhaled antibiotics might be beneficial in selected patients colonised with Pseudomonas aeruginosa. Important developments in the last decade include the introduction of international guidelines and the proposal for a validated scoring system for disease severity. Bronchiectasis patients are encountered by physicians in diverse medical professions and the disease itself is still underdiagnosed. The authors aim to increase awareness of the condition and provide practical tools for diagnosis and treatment.

摘要

这篇综述文章描述了成人非囊性纤维化(非CF)支气管扩张(胸部计算机断层扫描(CT)显示主要为中小支气管扩张)的流行病学、临床表现、诊断检查和治疗选择。我们用从图表回顾中获取的自身数据阐述文献中的证据,这些数据涉及236例成年患者,他们患有复发性下呼吸道感染且经高分辨率CT证实为非CF支气管扩张,于2000年至2010年间前往荷兰一家大型教学医院的呼吸疾病门诊就诊。非CF支气管扩张可被描述为支气管过度炎症、细菌定植和感染恶性循环的最终共同途径。非CF支气管扩张可能由多种原因引起,以感染和免疫缺陷为首,临床特征为慢性、咳痰性咳嗽和感染性加重。一旦通过高分辨率CT扫描诊断为非CF支气管扩张,建议进行基于方案的检查以确定潜在病因。非药物治疗选择主要针对清除支气管分泌物,吸入高渗剂可进一步改善这一情况。加重期的抗生素治疗是支气管扩张管理中的一项关键药物治疗。频繁加重的患者可考虑长期低剂量大环内酯类药物治疗,有充分证据支持。吸入性抗生素可能对定植有铜绿假单胞菌的特定患者有益。过去十年的重要进展包括国际指南的出台以及疾病严重程度验证评分系统的提议。支气管扩张患者在不同医学专业的医生处都有遇到,且该疾病本身仍未得到充分诊断。作者旨在提高对该疾病的认识,并提供诊断和治疗的实用工具。

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