Abstract:

Abstract：Objective To investigate the general characteristics， undrelying causative factors and prognosis of bronchiectasis in chilren. Methods A total of 172 chilren diagnosed with bronchiectasis were included in the study. Demographics， clinical data， HRCT， lung function， and underlying etiology were collected and retrospectively analyzed. Part of the patients were followed up. Results Cough and expectoration were the two most common symptoms. Chest X-ray was significantly less sensitive （4.42%） than HRCT （100%） scan in diagnosis of bronchiectasis. Immunodeficiency， bronchiolitis obliterans and primary ciliary dyskinesia were the three main etiology causing widespread distribution in HRCT （P＜0.05）. Lung functions in 76.92% of the patients were abnormal. The underlying etiologies were identified in 113 patients （65.70%）， and pulmonary infecion（31.40%） was the most common. It was found that FEV1 decreased by 1.28% per year with the extension of the course in the follow-up patients. There was improvement of HRCT in one patient whose symptom and lung fuction were also improved. Conclusion Bronchiectasis usually presents nonspecific symptoms. Most patients can be found with the underlying causes， and pulmonary infection is the most common cause. HRCT is needed as early as possible in children with chronic respiratory symptoms. FEV1% declines slowly with the course extended. Part of the bronchiectasis children can be improved.

Key words: bronchiectasis, etiology, follow-up, child

摘要：

目的 探讨儿童支气管扩张症临床表现、 影像学及肺功能方面特点， 分析病因构成， 了解疾病预后。方法    回顾性总结 172 例支气管扩张症患儿的临床表现、 影像学、 肺功能特点及病因， 对部分患儿进行随访。结果    支气管扩张症患儿临床主要表现为咳嗽、咯痰、生长发育受限、杵状指等。胸部X线诊断支气管扩张症的检出阳性率（4.42%）显著低于高分辨率CT（HRCT）的100%。存在免疫缺陷、闭塞性细支气管炎及原发性纤毛运动障碍的患儿在HRCT中弥漫性支气管扩张较其他病因更显著（P＜0.05）。76.92%的患儿肺功能异常。65.70%的患儿可以发现潜在病因，以肺部感染（31.40%）为主。门诊随访患儿FEV1%随病程的延长以每年1.28%的速度下降。其中1例临床症状、HRCT、肺功能均有好转。结论    儿童支气管扩张症临床表现没有明显特异性。大部分患儿可以发现潜在病因，以肺部感染最为常见。对于临床怀疑支气管扩张症的患儿应尽早进行HRCT检查，积极寻找潜在病因。支气管扩张症患儿的肺功能可随着病程的进展缓慢下降。部分患儿在临床症状、HRCT及肺功能上可有好转。

关键词: 支气管扩张症, 病因, 随访, 儿童