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The Role of Macrolides in Childhood Non-Cystic Fibrosis-Related Bronchiectasis

机译:大胶质剂在儿童时期非囊性纤维化相关支气管切除的作用

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Non-cystic fibrosis-related bronchiectasis is a chronic inflammatory lung disease, which is regarded as an "orphan" lung disease, with little research devoted to the study of this condition. Bronchiectasis results in impaired quality of life and mortality if left untreated. The tools available in the armamentarium for the management of bronchiectasis entail antibiotic therapy traditionally used to treat exacerbations, stratagems to improve mucociliary clearance, and avoidance of toxins. Macrolides have been known for the last two decades to have not only anti-bacterial effects but immunomodulatory properties as well. In cystic fibrosis, the use of macrolides is well documented in subjects colonized with Pseudomonas aeruginosa, to improve quality of life and lung function. There is currently emerging evidence to suggest the benefit of macrolides in subjects not colonized with Pseudomonas aeruginosa. This beneficial effect has been less explored in the context of bronchiectasis from other causes. The purpose of this paper is to review the current literature on the use of macrolides in non-cystic fibrosis related bronchiectasis in paediatrics. The term bronchiectasis is derived from the Greek words bronkia (bronchial tubes), ek (out), and tasis (stretching). The earliest description of bronchiectasis was by Laennac in 1819. There are two anatomical classification systems used for the diagnosis of bronchiectasis, namely, the Reid and Whitwell classifications . In the past few years, the diagnostic criteria for bronchiectasis have changed, with the diagnosis being based on the less invasive high-resolution computerized tomography (HRCT). HRCT scanning has led to easier diagnosis and follow up of bronchiectasis. The exact pathophysiological mechanisms for bronchiectasis are unknown, with the currently accepted concept being the "vicious cycle" theory proposed by Cole in the mid-eighties (Figure 1) . Cole's theory evolves around an initial "hit" or trigger that results in airway inflammation. The inflammatory process is established such that, with subsequent lung infections, persistent airway inflammation occurs.
机译:非囊性纤维化相关的支气管扩张是一种慢性炎症性肺病,被认为是“孤儿”肺病,具有较少的研究致力于这种情况的研究。如果未经处理,支气管扩张会导致生命质量和死亡率受损。用于支气管扩张的武器中可用的工具需要传统上用于治疗加剧,Stratagems以改善粘液间隙,避免毒素的抗生素治疗。在过去二十年中已知的大环内酯不仅具有抗细菌效应,而且还具有免疫调节性能。在囊性纤维化中,在用假单胞菌铜绿假单胞菌殖民地定植的受试者中,使用大溴化硼的使用,以提高生命和肺功能的质量。目前有新的证据表明,在没有铜绿假单胞菌未殖民的受试者中提出大溴化胶质的益处。在来自其他原因的支气管扩张的背景下,这种有益效果不太探讨。本文的目的是审查当前关于在非囊性纤维化中使用大溴化胶质相关支气管扩张的文献。术语支气管扩张术语来自希腊词Bronkia(支气管管),ek(out)和tasis(拉伸)。 Bronchiectasis的最早描述是Laennac于1819年。有两个解剖分类系统用于诊断支气管扩张,即Reid和Whitwell分类。在过去几年中,支气管扩张的诊断标准已经改变,诊断基于较少的侵入性高分辨率计算机断层扫描(HRCT)。 HRCT扫描导致诊断和跟踪支气管扩张。支气管扩张的确切病理生理机制是未知的,目前接受的概念是八十年代中央COLE提出的“恶性循环”理论(图1)。科尔的理论围绕着初始的“击中”或导致气道炎症导致的触发。建立炎症过程,使得随后的肺部感染,发生持续的气道炎症。

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