Airway Disease Progression on Chest Computed Tomography in Children With Primary Ciliary Dyskinesia.

IF 2.3 3区医学 Q1 PEDIATRICS

Pediatric Pulmonology Pub Date : 2025-09-01 DOI:10.1002/ppul.71287

BreAnna Kinghorn, Federico Mollica, Daan Caudri, Stephanie D Davis, Sharon Dell, Thomas W Ferkol, Robert Andrew Gardner, Michael R Knowles, Carlos Milla, Jessica E Pittman, Margaret Rosenfeld, Scott D Sagel, Adam J Shapiro, Erin Sullivan, Kelli M Sullivan, Harm A W M Tiddens, Maimoona A Zariwala, Margaret W Leigh

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引用次数: 0

Abstract

Rationale: The longitudinal trajectory of airway disease on chest computed tomography (CT) and risk factors for progression in primary ciliary dyskinesia (PCD) is largely unknown.

Methods: Longitudinal analysis of structural airway disease among children with PCD enrolled in a prospective, multicenter observational study, stratified by ultrastructural defect group. CTs were scored utilizing the Melbourne-Rotterdam Annotated Grid Morphometric Analysis for PCD, evaluating airway abnormalities in a hierarchical order: atelectasis, bronchiectasis (%BE), airway wall thickening, and mucus plugging/tree in bud opacities (%MP). Volume fraction of each component was expressed as % of total lung volume. %Disease (%DIS) is the sum of all components. Mixed effects regression analyses were utilized to describe the association between clinical predictors and CT scores adjusted for relevant covariates.

Results: Chest CTs (N = 424) were obtained from 142 children over a mean (SD) of 4.9 (3.7) years. Mean (SD) age at first CT was 8.5 (4.6) years. Mean %DIS increased with age for the cohort (0.09% per year, 95% CI: 0.02-0.15, p < 0.01), driven by increased %BE (0.06 higher per year, 95% CI: 0.04-0.08, p = 0.0001) and increased odds of MP (1.14 higher odds per year, 95% CI: 1.08-1.21, p < 0.0001). Children with inner dynein arm and microtubular defects had increased mean %DIS and increased odds of MP compared to other ciliary abnormalities (1.73% higher, 95% CI: 0.71-2.74, p = 0.001; 3.21 higher odds per year, 95% CI: 1.73-5.95, p < 0.001). Annual increase in CT scores did not differ between defect groups.

Conclusions: Children with PCD experience progressive bronchiectasis and worsening mucus plugging with age.

查看原文本刊更多论文

原发性纤毛运动障碍儿童胸部计算机断层扫描显示气道疾病进展。

理由：气道疾病在胸部计算机断层扫描（CT）上的纵向轨迹和原发性纤毛运动障碍（PCD）进展的危险因素在很大程度上是未知的。方法：在一项前瞻性、多中心观察性研究中，对PCD儿童的结构性气道疾病进行纵向分析，按超微结构缺陷组分层。使用墨尔本-鹿特丹PCD注释网格形态计量分析对ct进行评分，按等级顺序评估气道异常：肺不张、支气管扩张（%BE）、气道壁增厚和粘液堵塞/芽状混浊（%MP）。各组分的体积分数以占肺总体积的%表示。%Disease （%DIS）是所有组成部分的总和。混合效应回归分析用于描述临床预测因子与经相关协变量调整后的CT评分之间的关联。结果：142名儿童进行胸部ct检查（N = 424），平均（SD） 4.9（3.7）岁。首次CT的平均（SD）年龄为8.5（4.6）岁。在队列中，DIS的平均百分比随着年龄的增长而增加（每年0.09%,95% CI: 0.02-0.15, p）。结论：PCD患儿随着年龄的增长会出现进行性支气管扩张和粘液堵塞的恶化。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pediatric Pulmonology 医学-呼吸系统

CiteScore

6.00

自引率

12.90%

发文量

468

审稿时长

3-8 weeks

期刊介绍： Pediatric Pulmonology (PPUL) is the foremost global journal studying the respiratory system in disease and in health as it develops from intrauterine life though adolescence to adulthood. Combining explicit and informative analysis of clinical as well as basic scientific research, PPUL provides a look at the many facets of respiratory system disorders in infants and children, ranging from pathological anatomy, developmental issues, and pathophysiology to infectious disease, asthma, cystic fibrosis, and airborne toxins. Focused attention is given to the reporting of diagnostic and therapeutic methods for neonates, preschool children, and adolescents, the enduring effects of childhood respiratory diseases, and newly described infectious diseases. PPUL concentrates on subject matters of crucial interest to specialists preparing for the Pediatric Subspecialty Examinations in the United States and other countries. With its attentive coverage and extensive clinical data, this journal is a principle source for pediatricians in practice and in training and a must have for all pediatric pulmonologists.