Introduction: Pediatric noncystic fibrosis (CF) bronchiectasis has a variety of causes. An early and accurate diagnosis may prevent disease progression and complications. Current diagnostics and yield regarding etiology are evaluated in a pediatric cohort at a tertiary referral center. Methods: Available data, including high-resolution computed tomography (HRCT) characteristics, microbiological testing, and immunological screening of all children diagnosed with non-CF bronchiectasis between 2003 and 2017, were evaluated. Results: In 91% of patients [n = 69; median age 9 (3-18 years)] etiology was established in the diagnostic process. Postinfection (29%) and immunodeficiency (29%) were most common, followed by congenital anomalies (10%), aspiration (7%), asthma (6%), and primary ciliary dyskinesia (1%). HRCT predominantly showed bilateral involvement in immunodeficient patients (85%) and those with idiopathic bronchiectasis (83%). Congenital malformations (71%) were associated with unilateral disease. Completion of the diagnostic process often led to a change of treatment as started after initial diagnosis. Conclusion: Using a comprehensive diagnostic protocol, the etiology of pediatric non-CF bronchiectasis was established in more than 90% of patients. HRCT provides additional diagnostic information as it points to either a more systemic or a more localized etiology. Adequate diagnostics and data analysis allow treatment to be specifically adapted to prevent disease progression.

中文翻译：

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非囊性纤维化支气管扩张：确定小儿肺病病因的广泛诊断方案的评估。

简介：小儿非囊性纤维化（CF）支气管扩张有多种原因。早期准确的诊断可能会预防疾病进展和并发症。在三级转诊中心的儿科队列中评估有关病因的当前诊断和收益。方法：评估了2003年至2017年间所有被诊断为非CF支气管扩张的儿童的高分辨率数据包括计算机断层扫描（HRCT）特征，微生物学检测和免疫筛查数据。结果：在91％的患者中[n = 69; 在诊断过程中确定了中位年龄9岁（3-18岁）的病因。感染后（29％）和免疫缺陷（29％）最常见，其次是先天性异常（10％），误吸（7％），哮喘（6％）和原发性睫状运动障碍（1％）。HRCT主要显示免疫缺陷患者（85％）和特发性支气管扩张患者（83％）的双侧受累。先天性畸形（71％）与单侧疾病有关。诊断过程的完成通常导致最初诊断后开始治疗的改变。结论：使用全面的诊断方案，在90％以上的患者中确定了小儿非CF支气管扩张的病因。HRCT提供了更多的诊断信息，因为它指出了更系统的病因或更局部的病因。适当的诊断和数据分析可对治疗进行特定调整，以防止疾病进展。诊断过程的完成通常导致最初诊断后开始治疗的改变。结论：使用全面的诊断方案，在90％以上的患者中确定了小儿非CF支气管扩张的病因。HRCT提供了更多的诊断信息，因为它指出了更系统的病因或更局部的病因。适当的诊断和数据分析可对治疗进行特定调整，以防止疾病进展。诊断过程的完成通常导致最初诊断后开始的治疗改变。结论：使用全面的诊断方案，在90％以上的患者中确定了小儿非CF支气管扩张的病因。HRCT提供更多的诊断信息，因为它指向更系统的病因或更局部的病因。适当的诊断和数据分析可对治疗进行特定调整，以防止疾病进展。