Changing Epidemiology of Pediatric Pulmonary Exacerbations in Cystic Fibrosis.

IF 2.7 3区医学 Q1 PEDIATRICS

Pediatric Pulmonology Pub Date : 2025-03-01 DOI:10.1002/ppul.71019

Yaron Fireizen, Mohamoud Ahmed, Timothy Vigers, Kathryn Akong, Julie Ryu, Andrea Hahn, Hani Fanous, Anastassios Koumbourlis, Pornchai Tirakitsoontorn, Antonio Arrieta, Elizabeth B Burgener, Jonathan Koff, Jonathan D Cogen, Drake C Bouzek, Elin Hanley, Allison Keck, Dayna Stout, John Bradley, Scott D Sagel

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引用次数: 0

Abstract

Rationale: The introduction of elexacaftor/tezacaftor/ivacaftor (ETI), a highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy, to younger ages and the COVID-19 pandemic have significantly reduced pulmonary exacerbations requiring hospitalization among children with CF.

Objective: To assess demographic and clinical characteristics of children and young adults with CF hospitalized for pulmonary exacerbations before and after pediatric ETI approval.

Methods: A retrospective chart review was conducted at five United States CF Foundation-accredited care centers. Hospitalization data from children and young adults with CF in 2018 and 2022 were analyzed.

Results: Hospitalizations decreased from 471 cases (241 individuals) in 2018 to 163 cases (110 individuals) in 2022. The racial distribution shifted, with more hospitalized patients identifying as people of color in 2022 (28% vs. 14%; p = 0.018). A greater proportion of hospitalized children in 2022 had two non-F508del mutations compared with children hospitalized in 2018 (38% vs. 19%) and were less likely to be infected with methicillin-resistant Staphylococcus aureus (MRSA). Comparing 2022-2018, children on CFTR modulator therapy, including ETI (76%), showed reduced infections with Pseudomonas aeruginosa and Achromobacter xylosoxidans.

Conclusions: The decline in hospitalizations for pulmonary exacerbations likely reflects the benefits of ETI therapy, as a higher proportion of children and young adults hospitalized in 2022 had two non-F508del mutations and were not eligible for ETI. A greater percentage of those hospitalized in 2022 identified as belonging to minority racial groups, highlighting ongoing health disparities in the ETI era. Additionally, there were notable changes in the microbiological characteristics between 2018 and 2022.

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囊性纤维化儿童肺恶化的变化流行病学。

理由：elexaftor /tezacaftor/ivacaftor （ETI）是一种高效的囊性纤维化跨膜传导调节剂（CFTR）调节剂，在更年轻的年龄和COVID-19大流行中引入，显著减少了CF儿童住院的肺恶化。目的：评估儿童ETI批准前后因肺恶化住院的CF儿童和青年患者的人口学和临床特征。方法：在5个美国CF基金会认可的护理中心进行回顾性图表回顾。分析2018年和2022年CF儿童和青年的住院数据。结果：住院人数从2018年的471例（241人）下降到2022年的163例（110人）。种族分布发生了变化，2022年有更多的住院患者被认定为有色人种(28%对14%；p = 0.018)。与2018年住院儿童相比，2022年住院儿童中有两种非f508del突变的比例更高（38%对19%），并且感染耐甲氧西林金黄色葡萄球菌（MRSA）的可能性更低。与2022年至2018年相比，接受CFTR调节剂治疗的儿童，包括ETI(76%)，铜绿假单胞菌和木氧化无色杆菌的感染减少。结论：肺恶化住院率的下降可能反映了ETI治疗的益处，因为2022年住院的儿童和年轻人中有更高比例的人患有两种非f508del突变，不符合ETI的条件。2022年住院的人中有更大比例被确定属于少数种族群体，突显了ETI时代持续存在的健康差距。此外，2018年至2022年期间，微生物特征也发生了显著变化。

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.