Lung ultrasound in children with primary ciliary dyskinesia or cystic fibrosis.

IF 2.7 3区医学 Q1 PEDIATRICS Pediatric Pulmonology Pub Date : 2024-12-01 Epub Date: 2024-09-02 DOI:10.1002/ppul.27215

Noah Marzook, Alexander S Dubrovsky, Karl Muchantef, David Zielinski, Larry C Lands, Adam J Shapiro

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Abstract

Introduction: Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are respiratory conditions requiring regular chest radiography (CXR) surveillance to monitor pulmonary disease. However, CXR is insensitive for lung disease in CF and PCD. Lung ultrasound (LU) is a radiation-free alternative showing good correlation with severity of lung disease in CF but has not been studied in PCD.

Method: Standardized, six-zone LU studies and CXR were performed on a convenience sample of children with PCD or CF during a single visit when well. LU studies were graded using the LU scoring system, while CXR studies received a modified Chrispin-Norman score. Scores were correlated with clinical outcomes.

Result: Data from 30 patients with PCD and 30 with CF (median age PCD 11.5 years, CF 9.1 years) with overall mild pulmonary disease (PCD median FEV₁ 90% predicted, CF FEV₁ 100%) were analyzed. LU abnormalities appear in 11/30 (36%) patients with PCD and 9/30 (30%) with CF. Sensitivity, specificity, positive predictive, and negative predictive values for abnormal LU compared to the gold standard of CXR are 42%, 61%, 42%, and 61% in PCD, and 44%, 81%, 50%, and 77% in CF, respectively. Correlation between LU and CXR scores are poor for both diseases (PCD r = -0.1288, p = 0.4977; CF r = 0.0343, p = 0.8571), and LU score does not correlate with clinical outcomes in PCD.

Conclusion: The correlation of LU findings with CXR surveillance studies is poor in patients with mild disease burdens from PCD or CF, and LU scores do not correlate with clinical outcomes in PCD.

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原发性睫状肌运动障碍或囊性纤维化患儿的肺部超声波检查。

简介：原发性睫状肌运动障碍（PCD）和囊性纤维化（CF）是呼吸系统疾病，需要定期进行胸部放射摄影（CXR）监测，以监测肺部疾病。然而，CXR 对 CF 和 PCD 的肺部疾病不敏感。肺部超声（LU）是一种无辐射的替代方法，与 CF 肺病的严重程度有很好的相关性，但尚未在 PCD 中进行研究：方法：对患有 PCD 或 CF 的儿童进行标准化的六区 LU 检查和 CXR，并在病情良好时进行单次就诊。LU检查采用LU评分系统进行评分，CXR检查则采用改良的Chrispin-Norman评分。评分与临床结果相关：对 30 名 PCD 患者和 30 名 CF 患者（中位年龄 PCD 11.5 岁，CF 9.1 岁）的数据进行了分析，这些患者总体上肺部疾病较轻（PCD 中位 FEV1 预测值为 90%，CF FEV1 预测值为 100%）。11/30（36%）名 PCD 患者和 9/30（30%）名 CF 患者出现 LU 异常。与金标准 CXR 相比，LU 异常的敏感性、特异性、阳性预测值和阴性预测值在 PCD 患者中分别为 42%、61%、42% 和 61%，在 CF 患者中分别为 44%、81%、50% 和 77%。两种疾病的LU和CXR评分之间的相关性都很差（PCD r = -0.1288，p = 0.4977；CF r = 0.0343，p = 0.8571），LU评分与PCD的临床结果没有相关性：结论：在疾病负担较轻的 PCD 或 CF 患者中，LU 结果与 CXR 监测研究的相关性较差，且 LU 评分与 PCD 的临床预后无关。

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.