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Noncystic Fibrosis Bronchiectasis: Evaluation of an Extensive Diagnostic Protocol in Determining Pediatric Lung Disease Etiology.
Pediatric Allergy, Immunology, and Pulmonology ( IF 1.1 ) Pub Date : 2019-12-11 , DOI: 10.1089/ped.2019.1030
Nike I Beckeringh 1 , Niels W Rutjes 2 , Joost van Schuppen 3 , Taco W Kuijpers 1
Affiliation  

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:

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。
更新日期:2019-12-11
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