Pediatric Pulmonology最新文献

{"title":"CFTR Modulator Therapy and Aerobic Fitness Changes in Aerobic Fitness Following Initiation of CFTR Modulator Therapy: A Systematic Review and Meta-Analysis.","authors":"Ingrid Silveira de Almeida, Fernanda Maria Vendrusculo, Mariana Severo da Costa, Márcio Vinícius Fagundes Donadio","doi":"10.1002/ppul.71826","DOIUrl":"10.1002/ppul.71826","url":null,"abstract":"<p><strong>Introduction: </strong>Cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy has led to relevant clinical advances in people with cystic fibrosis (pwCF). However, its effects on aerobic fitness are unclear.</p><p><strong>Objective: </strong>To evaluate the effects of CFTR modulator therapy on aerobic fitness in pwCF through a systematic review and meta-analysis.</p><p><strong>Methods: </strong>This systematic review (CRD420251066602) was conducted by searching major databases without date or language restrictions. Analytical studies evaluating cardiopulmonary exercise testing outcomes before and after CFTR modulator therapy were included. The primary outcome was peak oxygen uptake (peak VO₂). Secondary outcomes included peak VO₂ (% predicted), VO₂ at the first ventilatory threshold (VT₁), breathing reserve, workload, forced expiratory volume in one second (FEV₁), and body mass index. Meta-analyses were performed using a random-effects model with heterogeneity assessed by the I² statistic, and sensitivity analyses conducted.</p><p><strong>Results: </strong>Thirteen studies (n = 578 participants) were included, of which 11 were eligible for meta-analysis. The meta-analysis did not identify a significant improvement in peak VO₂ (mL·kg^-1·min^-1) after CFTR modulator use (MD = -0.22; 95% CI: -2.10 to 1.65; p = 0.82). For submaximal outcomes, after sensitivity analysis, VO₂ at VT₁ was significantly lower in the post-intervention period (MD = -1.85; 95% CI: -2.61 to -1.10; p < 0.00001). Breathing reserve increased significantly (MD = 7.94; 95% CI: 2.15 to 13.73; p = 0.007), and workload also increased significantly (MD = 15.39; 95% CI: 2.20 to 28.58; p = 0.02).</p><p><strong>Conclusion: </strong>CFTR modulator therapy is not consistently associated with improvements in aerobic fitness, as assessed by peak VO₂, although it appears to variably influence outcomes related to ventilatory response and exercise performance.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 9","pages":"e71826"},"PeriodicalIF":2.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13539545/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148888215","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Geographic Inequalities in Pediatric Asthma and Workforce Distribution.","authors":"Anand Gourishankar, Eugene Suwandhi","doi":"10.1002/ppul.71840","DOIUrl":"10.1002/ppul.71840","url":null,"abstract":"<p><strong>Background: </strong>Asthma is one of the most common chronic diseases affecting children in the United States, with millions impacted annually. There is ongoing concern and disparity in the pediatric workforce. However, there is limited research evaluating how the geographic distribution of these providers aligns with pediatric asthma burden across states.</p><p><strong>Objective: </strong>To assess the degree of geographic alignment between pediatric asthma prevalence and the distribution of general pediatricians and pediatric hospitalists in the United States using inequality metrics and spatial analysis.</p><p><strong>Methods: </strong>This was a cross-sectional, state-level ecological study using publicly available data. Pediatric asthma prevalence was obtained from the CDC Behavioral Risk Factor Surveillance System. Pediatric provider data were sourced from the American Board of Pediatrics, and child population estimates were retrieved from the U.S. Census Bureau. Workforce density was calculated as the number of general pediatricians and pediatric hospitalists per 100,000 children in each state. Lorenz curves and Gini coefficients were used to quantify geographic inequality in asthma burden and workforce distribution. Bivariate choropleth maps and Pearson correlation coefficients were employed to assess spatial mismatch and association between asthma prevalence and provider availability.</p><p><strong>Results: </strong>General pediatricians had a mean state-level density of 70.6 per 100,000 children, while pediatric hospitalists had a mean density of 5.3 per 100,000 children (p < 0.0001). Gini coefficients revealed moderate inequality in the distribution of general pediatricians (0.20) and higher inequality among pediatric hospitalists (0.33). In contrast, the Gini index for pediatric asthma prevalence was 0.11. Correlation analysis showed a weak, marginally significant association between asthma prevalence and general pediatrician density (r = 0.30, p = 0.032), and a non-significant association with pediatric hospitalist density (r = 0.24, p = 0.094). Bivariate mapping identified several states with high asthma burden and low provider density, revealing substantial spatial mismatches.</p><p><strong>Conclusion: </strong>There is a misalignment between the burden of pediatric asthma and the distribution of pediatric healthcare providers in the U.S., particularly in pediatric hospital medicine. States with higher asthma prevalence are not consistently supported by proportionally larger pediatric workforces. Policies that support provider redistribution, rural practice incentives, and data-driven workforce modeling are essential to improving health equity and optimizing care for children with asthma.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 9","pages":"e71840"},"PeriodicalIF":2.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149008868","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association Between Diaphragm Ultrasound Measures and Extubation Outcomes in Neonates: A Systematic Review and Meta-Analysis.","authors":"Mayson L A Sousa, Eleonora Balzani, Sebastian Dubo, Amanda A C Coelho, Taylor Davidson, Glasiela Alcala, Me-Linh Le, Shyamala Dakshinamurti, Yasser Elsayed, Annia F Schreiber","doi":"10.1002/ppul.71807","DOIUrl":"10.1002/ppul.71807","url":null,"abstract":"<p><strong>Objective: </strong>We conducted a systematic review and prognostic meta-analysis to evaluate the association between ultrasound-derived measures of diaphragm structure and contractile activity with the risk of extubation failure in mechanically ventilated neonates.</p><p><strong>Data sources: </strong>MEDLINE, Embase, the Cochrane Library, CINAH, Scopus, and ClinicalTrials.gov were systematically searched from inception to January 12, 2026, without using language restrictions.</p><p><strong>Study selection: </strong>Observational studies including mechanically ventilated neonates (≤ 28 days old) undergoing planned extubation in whom diaphragmatic thickness, thickening fraction, or excursion were measured by ultrasound and extubation outcome were reported. Studies involving congenital diaphragmatic or neuromuscular disorders were excluded. Risk of bias was assessed using the Quality in Prognosis Studies (QUIPS) tool. Random-effects meta-analyses were conducted using mean differences (MD) with 95% confidence intervals (CI).</p><p><strong>Results: </strong>Of 1,522 records identified, 110 full-text articles were assessed, and seven prospective cohort studies (n = 255 neonates) met the inclusion criteria. Extubation failure occurred in 90 neonates (35%). Neonates who failed extubation had significantly lower diaphragm thickening fraction compared with those successfully extubated (MD = -9.51%; 95% CI: -14.73 to -4.29; p = 0.0004; I² = 0%). Differences in diaphragm thickness and excursion were not statistically significant. Overall risk of bias was moderate to high, primarily due to inadequate adjustment for confounding and variability in outcome definitions.</p><p><strong>Conclusions: </strong>Lower diaphragm thickening fraction is associated with extubation failure in mechanically ventilated neonates. Standardization of measurement protocols and prospective validation studies are needed before diaphragm ultrasound can be incorporated into routine extubation decision-making.</p><p><strong>Registration: </strong>PROSPERO CRD420251045024.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 9","pages":"e71807"},"PeriodicalIF":2.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13504643/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148813537","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diagnosis of Post-Infectious Bronchiolitis Obliterans: Guiding Treatment With Infant Pulmonary Function Testing.","authors":"Jessica Reyes Angel, Jeremy Landeo Guitterez, Daniel J Weiner, Geoffrey Kurland","doi":"10.1002/ppul.71792","DOIUrl":"10.1002/ppul.71792","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 9","pages":"e71792"},"PeriodicalIF":2.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13505396/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148813752","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Case Series of Sustained Fluid-Associated Weight Gain Following Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulator Therapy.","authors":"Emma M Tillman, Arina Lazutina, Ross C Robinson, Abi Colwell, Cynthia D Brown","doi":"10.1002/ppul.71813","DOIUrl":"10.1002/ppul.71813","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 9","pages":"e71813"},"PeriodicalIF":2.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13507763/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148819271","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Reproductive Futures in Focus: Contraceptive Choices Among Individuals With Cystic Fibrosis-Patterns, Preferences, and Influencing Factors.","authors":"Kevin P Lonabaugh, HollyAnn Jacobs, Jordan Orofino, Leanne Saville, Brittany A Wright","doi":"10.1002/ppul.71809","DOIUrl":"10.1002/ppul.71809","url":null,"abstract":"<p><strong>Introduction: </strong>Advancements in the care of people with cystic fibrosis (PwCF) have made discussions about reproductive health increasingly important. Rising rates of both intended and unintended pregnancies highlight the need for effective contraception counseling. Understanding current contraception use patterns and identifying priorities for contraception education amongst PwCF are therefore essential.</p><p><strong>Methods: </strong>A web-based survey was developed in partnership with Community Voice, a virtual community of PwCF and their family members supported by the Cystic Fibrosis Foundation. The survey was distributed over a 5-week period (June-July 2025) to participants from Community Voice and Elevate. Females with cystic fibrosis aged 18 years and older were eligible to participate.</p><p><strong>Results: </strong>Ninety-seven PwCF completed the survey, representing 27 U.S. states and four Canadian provinces. Among individuals currently using contraception, the most common methods were intrauterine devices (34%), combined oral contraceptives (28.3%), and condoms/barrier methods (24.5%). Although 90% of PwCF were not actively trying to conceive, only 55% reported using a form of birth control. Knowledge-based questions revealed persistent misconceptions about the impact of common antibiotics on contraceptive effectiveness. While most PwCF (68.1%) wanted contraception information from their CF care team, only 28.9% reported receiving such education. Side effects were a major factor in decision-making: 78.9% selected contraceptive methods based on side-effect profiles (valued more than efficacy), 68.6% reported experiencing side effects, and side effects were the leading reason for contraception discontinuation.</p><p><strong>Conclusion: </strong>CF care teams can play a critical role in initiating and supporting conversations about contraception. Team members, including pharmacists, can provide medication counseling and help PwCF select contraceptive methods that align with their individual goals and needs.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 9","pages":"e71809"},"PeriodicalIF":2.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13527759/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148866061","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical Outcomes of Chronic Airway Infection in Primary Ciliary Dyskinesia.","authors":"Thomas G Saba, Madsen Zimbric, Harlan McCaffery, Lindsay J Caverly","doi":"10.1002/ppul.71828","DOIUrl":"10.1002/ppul.71828","url":null,"abstract":"<p><strong>Introduction: </strong>Primary ciliary dyskinesia (PCD) is a rare disorder of impaired respiratory mucociliary clearance and chronic pulmonary infections. The clinical impact of chronic infection is poorly understood. The objective of this single-center, retrospective cohort study was to assess clinical outcomes of people with PCD and chronic airway infection. We hypothesized that chronic airway infection would be associated with greater declines in lung function and higher pulmonary exacerbation rates.</p><p><strong>Methods: </strong>Linear mixed effects models were used to analyze the association of chronic infection with percent predicted forced expiratory volume in 1 s (ppFEV1). Poisson mixed models were used to analyze the association of chronic infection with exacerbation rates.</p><p><strong>Results: </strong>Thirty-two people with PCD were included: 19 (59%) with chronic infection including 5 (16%) with chronic Pseudomonas aeruginosa (PA), 10 (31%) with chronic Haemophilus influenzae (HI), 2 (6%) with chronic methicillin-sensitive Staphylococcus aureus (MSSA) and 2 (6%) with chronic Aspergillus fumigatus (AF). A lower ppFEV1 was associated with chronic AF, but not with other chronic infections. The exacerbation rate ratio was 1.29 [95% CI 0.72, 2.30] (p = 0.388), 5.03 [95% CI 1.41, 18.0] (p = 0.013), 0.41 [95% CI 0.16, 1.08] (p = 0.072) and 2.19 [95% CI 0.39, 12.30] (p = 0.373) after development of chronic HI infection, chronic PA infection, chronic MSSA infection and chronic AF infection, respectively.</p><p><strong>Conclusion: </strong>In this single-center, retrospective study of chronic airway infections in PCD, chronic infection with PA was associated with a greater rate of pulmonary exacerbations but ppFEV1 decline was only associated with chronic AF infection.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 9","pages":"e71828"},"PeriodicalIF":2.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13547882/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148900981","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Bronchial Hyperresponsiveness in Pediatric Non-Cystic Fibrosis Bronchiectasis.","authors":"Lisa Gerbeau, Armelle Finet, Margaux Bertrand, Emmanuelle Bosdure, Laura Papazian, Kenza Tahiri, Melisande Baravalle, Julie Mazenq, Jean-Christophe Dubus","doi":"10.1002/ppul.71839","DOIUrl":"10.1002/ppul.71839","url":null,"abstract":"<p><strong>Objective: </strong>The prevalence and significance of bronchial hyperresponsiveness (BHR) in patients with non-cystic fibrosis (CF) bronchiectasis remain unclear. The objective is to determine the prevalence of BHR, defined as a post-bronchodilator forced expiratory volume in 1 s (FEV1) improvement > 10%, in children with non-CF bronchiectasis and to assess its association with clinical, functional, and radiological characteristics.</p><p><strong>Design: </strong>This retrospective, observational monocentric study included children with non-CF bronchiectasis followed-up at an university center for rare pediatric respiratory diseases.</p><p><strong>Patients: </strong>Patients were eligible if they had performed spirometry with bronchodilator reversibility testing within 3 years of a diagnostic chest computed tomography (CT).</p><p><strong>Main outcomes measures: </strong>Asthma symptoms, asthma exacerbations, asthma treatments, allergic markers, infectious exacerbations, causes of bronchiectasis, FEV1, FEV1/forced vital capacity (FVC) ratio, reversibility, and mosaic attenuation on CT were compared between patients with and without BHR.</p><p><strong>Results: </strong>Among 157 patients, 65 (male/female sex ratio 0.71, mean age 10 years old, asthma symptoms in over 56% of patients, 67% treated with inhaled corticosteroids) met the inclusion criteria. BHR was present in 32.3% of cases. Patients with BHR had a significantly altered lung function (baseline Z-score FEV1 -2.12 vs -0.87; FEV1/FVC -2.28 vs -0.85). No significant association was found between BHR and clinical and radiological characteristics.</p><p><strong>Conclusion: </strong>BHR is not associated with clinical, radiological or therapeutic characteristics in pediatric non-CF bronchiectasis, except a more severe airflow limitation. These findings highlight the importance of lung function assessment for non-CF bronchiectasis patients.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 9","pages":"e71839"},"PeriodicalIF":2.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149017537","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Development of the Novel FLOX Score to Assess Risk of Intubation in Children With Immunocompromising Conditions Receiving High Flow Nasal Cannula.","authors":"Michelle A Mai, Colin M Rogerson, Courtney M Rowan","doi":"10.1002/ppul.71814","DOIUrl":"10.1002/ppul.71814","url":null,"abstract":"<p><strong>Background: </strong>Heated, humidified, high-flow nasal cannula (HFNC) before intubation has been associated with increased mortality in children with immunocompromising conditions (ICC).</p><p><strong>Objectives: </strong>We sought to describe HFNC failure (intubation) rate and aimed to develop a novel score associated with HFNC failure.</p><p><strong>Methods: </strong>This is a single center, retrospective cohort. Patients (aged 0-26 years) with ICCs who used HFNC between 2021 and 2023 were included.</p><p><strong>Results: </strong>Forty-four (47.3%) of 93 included subjects failed HFNC. PICU mortality was 14% (n = 13). The median duration of HFNC was 18.7 h (IQR: 6.0, 40.4). We developed the novel FLOX ((flow in Liters/minute)/weight (kg)*FiO₂) score. Median FLOX score was higher with HFNC failure at initiation (p = 0.04), 2 h (p = 0.03), 4 h (p = 0.05), and 6 h (p = 0.05). Adjusting for acuity, the odds of HFNC failure with a FLOX score (≥0.20) were as follows: Initiation OR = 2.5 (95%CI: 1.03, 5.9), p = 0.042); 2 h of HFNC, OR = 4.3 ((95%CI: 1.7, 10.9), p = 0.002); 4 h of HFNC, OR = 6.5 (95%CI: 2.5, 16.8), (p < 0.001); and 6 h of HFNC, OR = 4.9 (95%CI: 1.9, 12.7), (p = 0.001).</p><p><strong>Conclusion: </strong>In children with ICCs, almost half fail HFNC. Mortality was high in those who fail HFNC. The FLOX score, specifically a FLOX ≥ 0.20 is associated with increased odds of failing HFNC in our ICC cohort.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 9","pages":"e71814"},"PeriodicalIF":2.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13527891/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148866006","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Comment on \"Asthma in Children With Congenital Lung Malformations: A Population-Based Case-Control Study\".","authors":"Sachin Kumar, Nirupma Gupta, Sujeet Kumar Singh, Hariharan Srinivasan, Varshini Vadhithala","doi":"10.1002/ppul.71805","DOIUrl":"10.1002/ppul.71805","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 9","pages":"e71805"},"PeriodicalIF":2.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13505276/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148813707","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}