Stafler Patrick, Carr Siobhán B
Barts and The London Children's Hospital, UK.
Arch Dis Child Educ Pract Ed. 2010 Jun;95(3):73-82. doi: 10.1136/adc.2007.130054.
An increase in the frequency of diagnosing non-cystic fibrosis bronchiectasis in children is due to heightened awareness of the disease and the wider availability of high-resolution computed tomography. The most common underlying conditions leading to bronchiectasis include infections, immunodeficiency, aspiration and primary ciliary dyskinesia. Treatment centres on airway clearance with aggressive antibiotic regimens and physiotherapy; more specific approaches are available for some of the underlying conditions. A high index of suspicion that a child may have underlying bronchiectasis must be maintained in the presence of prolonged or recurrent "wet/productive" cough. The classic definition of bronchiectasis is of irreversible bronchial dilatation; however, at the milder end of the spectrum it appears that radiographic changes may be reversible. Untreated, in its severest form bronchiectasis can progress to end stage pulmonary failure in adult life. In this article, we review its pathogenesis and diagnosis and the evidence base for available treatments.
儿童非囊性纤维化支气管扩张症诊断频率的增加归因于对该疾病认识的提高以及高分辨率计算机断层扫描的更广泛应用。导致支气管扩张的最常见潜在病因包括感染、免疫缺陷、误吸和原发性纤毛运动障碍。治疗以积极的抗生素方案和物理治疗进行气道清理为主；针对某些潜在病因还有更具针对性的治疗方法。当儿童出现长期或反复的“湿性/咳痰性”咳嗽时，必须高度怀疑其可能存在潜在的支气管扩张症。支气管扩张的经典定义是不可逆的支气管扩张；然而，在病情较轻的阶段，影像学改变似乎可能是可逆的。未经治疗的支气管扩张症在最严重的情况下可在成年期发展为终末期肺衰竭。在本文中，我们将综述其发病机制、诊断以及现有治疗方法的循证依据。
