Pediatric Pulmonology最新文献

{"title":"Two Pediatric Cases of Mounier-Kuhn Syndrome: Diagnostic and Therapeutic Challenges.","authors":"Ruxuan He, Lanhong Ma, Huimin Li, Haiming Yang","doi":"10.1002/ppul.71553","DOIUrl":"10.1002/ppul.71553","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 3","pages":"e71553"},"PeriodicalIF":2.3,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147377372","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":"Assessing Dietary Patterns and Composition Among Adults With Cystic Fibrosis Taking Highly Effective Modulator Therapy.","authors":"Julianna Bailey, Natalie R Rose, Ashritha R Chalamalla, Justin D Anderson, Elizabeth Baker, Jennifer S Guimbellot","doi":"10.1002/ppul.71532","DOIUrl":"10.1002/ppul.71532","url":null,"abstract":"<p><strong>Background: </strong>The metabolic impact of poor diet quality in cystic fibrosis (CF), coupled with a rise in obesity and modulator-induced weight gain, is a growing concern. Our study aimed to understand knowledge and perspectives regarding dietary changes on modulators, and how measured nutrient intake changes with different dietary patterns in response to diet education.</p><p><strong>Methods: </strong>A cross-sectional survey was administered to 82 adult CF patients at the University of Alabama Birmingham. A subset of 10 participants received diet education and followed two diet patterns sequentially: a general healthful (GH) diet and a high fat (HF) diet. Three-day diet records were analyzed following each diet pattern.</p><p><strong>Results: </strong>A total of 82 adults responded to the survey and 42% of respondents reported making healthful dietary changes on modulators. Only 56% of respondents were able to correctly identify which foods contained fat. Diet record analyses showed a significant decrease in energy intake and fat intake on the GH diet compared to the HF diet. Baseline Healthy Eating Index (HEI) scores improved significantly (p = 0.0254) following education on a GH diet pattern, but were significantly lower following the HF diet (p = 0.0179).</p><p><strong>Conclusions: </strong>While nearly half of survey respondents reported making healthy dietary changes on modulators, basic nutrition knowledge deficits persist. Measured diet quality was poor at baseline and significantly improved after receiving an educational session on GH eating. Findings highlight the need for targeted, basic education on GH eating patterns in the clinical practice and larger studies of nutrition interventions for improving diet quality in CF.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 3","pages":"e71532"},"PeriodicalIF":2.3,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12967693/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147378229","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":"MLPA-Detected CFTR Exon Deletions in Children With Inconclusive CFTR Sequencing: A Single-Center Experience From Türkiye.","authors":"Sanem Eryılmaz Polat, Çelebi Yıldırım, Hande Yetişgin, Ayyüce Aktemur Ünlü, Gamze Akça Dinç, Meltem Kürtül Çakar, Işıl Bilgiç, Satı Özkan Tabakçı, Şule Selin Akyan Soydaş, Salih Uytun, Murat Yasin Gençoğlu, Dilber Ademhan Tural, Gökçen Dilşa Tuğcu, Büşranur Çavdarlı, Ahmet Cevdet Ceylan, Güzin Cinel","doi":"10.1002/ppul.71556","DOIUrl":"10.1002/ppul.71556","url":null,"abstract":"<p><strong>Introduction: </strong>Cystic fibrosis (CF) is an autosomal recessive disorder caused by variants in the CFTR gene and shows marked genetic heterogeneity in diverse populations. Although next-generation sequencing (NGS) has significantly improved CFTR variant detection, it may fail to identify large exon deletions or duplications, leaving the CFTR genotype incomplete in some patients. This study evaluated the diagnostic contribution of the multiplex ligation-dependent probe amplification (MLPA) method in pediatric CF cases with incomplete CFTR genotyping after NGS.</p><p><strong>Methods: </strong>A total of 51 pediatric patients who were evaluated for CF diagnosis or suspicion at our center between 2019 and 2024 and had incomplete CFTR genotyping after NGS were retrospectively reviewed. MLPA analysis was performed on these patients. Clinical data, sweat chloride test results, genetic analysis findings, and eligibility for modulator treatment were evaluated.</p><p><strong>Results: </strong>MLPA analysis detected deletions in the CFTR gene in 12 cases (23.5%). The most frequently observed deletions were at exons 2 and 11. In 6 cases where no pathogenic CFTR variant was detected by NGS analysis, large exon deletions were identified by MLPA. The remaining 39 cases were evaluated after NGS and MLPA. CF was excluded in 22 of these cases, whereas clinical suspicion persisted in 17 patients despite negative genetic results. This group was referred for further molecular analysis.</p><p><strong>Conclusion: </strong>MLPA is a complementary method that contributes to the detection of gene rearrangements in cases where CFTR genotyping is incomplete after NGS. Using MLPA as an additional analytical tool in populations with significant genetic heterogeneity may help characterize CFTR variants more comprehensively.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 3","pages":"e71556"},"PeriodicalIF":2.3,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147378284","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":"Mode of Delivery and Neonatal Characteristics as Risk Factors for Childhood Asthma in Qatar: A Case-Control Study.","authors":"Samah Elshaar, Khalid Zahraldin, Ahmed Adel, Abdelrahman Ibrahim Babiker, Mohammad Abdelwahab Abdelhamid Bayoumi, Manar Elsheikh Abdel-Rahman","doi":"10.1002/ppul.71569","DOIUrl":"10.1002/ppul.71569","url":null,"abstract":"<p><strong>Background: </strong>Asthma rates are rising globally, posing a significant public health challenge. In Qatar, childhood asthma affects 34.6% of children, making it a common chronic condition. The global scope of existing literature on the association between childhood asthma and perinatal and neonatal risk factors may not fully account for Qatar's distinctive socio-cultural, genetic, and healthcare aspects. Therefore, this research aims to emphasize the importance of considering regional variations in health outcomes.</p><p><strong>Objectives: </strong>To investigate the association between childhood asthma development and mode of delivery, gestational age, and birthweight in Qatar.</p><p><strong>Methods: </strong>A hospital-based case-control study was conducted in Qatar across Hamad General, Al Wakrah, and Al Khor hospitals from January 2020 to July 2023, including 830 asthma cases and 1,512 non-asthma controls aged 6-14 years. Secondary data from Electronic Medical Records were analyzed using logistic regression, with odds ratios (ORs) and adjusted odds ratios (aORs) calculated for each factor. Confounders were managed through Directed Acyclic Graphs (DAG), and multiple imputations addressed missing data.</p><p><strong>Results: </strong>Asthma prevalence was 35.4%. Compared to controls, asthma cases were more likely to be males (65% vs. 41%) and have a family history of asthma (31.3% vs. 1.7%). In addition, Cesarean delivery (aOR 1.15, 95% CI 0.92, 1.44), preterm birth (aOR 1.07, 95% CI 0.79), and low birth weight (aOR 1.03, 95% CI 0.61, 1.73) showed slight increases in asthma risk. However, none of these associations reached statistical significance.</p><p><strong>Conclusion: </strong>Despite non-significant associations, findings underscore the importance of considering regional variations and context-specific research.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 3","pages":"e71569"},"PeriodicalIF":2.3,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147494115","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":"Pediatric Provider Experiences With Single Maintenance and Reliever Therapy (SMART).","authors":"Michelle K Trivedi, Michelle Spano, Deicy Mejia Agudelo, Stephanie Simms, Christine Frisard, Grace W Ryan, Melissa Goulding, James G Krings, Stephenie Lemon, Nancy Byatt, Jerry A Krishnan, Sybil Crawford, Lori Pbert","doi":"10.1002/ppul.71542","DOIUrl":"10.1002/ppul.71542","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 3","pages":"e71542"},"PeriodicalIF":2.3,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147321898","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":"Normal Values for Respiratory Oscillometry in Pediatrics: An Argument for a Local Control Population.","authors":"Heather Boas, Joseph McDonough, Adam Lane, Sara B DeMauro, Clement L Ren, Maureen Josephson, Samuel B Goldfarb, Paul D Robinson, Julian L Allen","doi":"10.1002/ppul.71508","DOIUrl":"10.1002/ppul.71508","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 3","pages":"e71508"},"PeriodicalIF":2.3,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12947609/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147309001","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":"Urinary System Involvement in Children With Cystic Fibrosis: A Single-Center Retrospective Cohort.","authors":"Pelin Asfuroglu, Ahmet Asfuroglu","doi":"10.1002/ppul.71551","DOIUrl":"10.1002/ppul.71551","url":null,"abstract":"<p><strong>Background: </strong>Cystic fibrosis (CF) is a multisystem genetic disorder. While pulmonary and gastrointestinal manifestations are well recognized, urinary system involvement in children with CF remains limited.</p><p><strong>Methods: </strong>We retrospectively evaluated children with CF who had at least one urine sample available for analysis. Demographic and clinical characteristics, routine urinalysis findings, spot urine biochemical measurements (available in a subset of children), and abdominal ultrasonography results were reviewed. Urinary abnormalities were defined as the presence of calcium oxalate crystalluria and/or renal calculi detected on ultrasonography.</p><p><strong>Results: </strong>A total of 41 children with CF (23 males, 56.1%) were included, with a median age of 6.0 years (range 0.5-17). Calcium oxalate crystalluria was detected in 13 children (31.7%), and renal calculi were identified in 8 of those who underwent ultrasonography (19.5%). Most renal calculi were small (median size 3 mm) and located in the lower poles of the kidneys, and none of the children required surgical intervention. Estimated glomerular filtration rate was preserved in most patients, and no demographic or routinely assessed biochemical parameters were independently associated with urinary abnormalities.</p><p><strong>Conclusions: </strong>Urinary abnormalities, particularly crystalluria and nephrolithiasis, appear to be relatively common in children with CF, often in the absence of overt renal dysfunction. Periodic assessment of routine urinalysis and urine microscopy may facilitate early identification of urinary abnormalities and guide further evaluation when clinically indicated. Prospective studies incorporating comprehensive metabolic stone assessments are warranted to clarify the clinical significance of these findings.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 3","pages":"e71551"},"PeriodicalIF":2.3,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147366145","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":"Survival From Severe Carbon Monoxide Intoxication Complicated By Aspiration ARDS: A Case for Sequential Management Approach.","authors":"Marco Piastra, Ivonne Portaccio, Riccardo Maviglia, Silvia Maria Pulitanò, Giorgio Conti, Paolo Maurizio Soave","doi":"10.1002/ppul.71552","DOIUrl":"10.1002/ppul.71552","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 3","pages":"e71552"},"PeriodicalIF":2.3,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147377334","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":"The Role of Vitamin D in Severity and Control of Asthma in Children and Adolescents: A Systematic Review and Meta-Analysis.","authors":"Joelia M Ladeira, Olívia Zacas, Amanda Miranda Ferreira, Patrícia Chaib Gomes Stegun, Milena Baptistella Grotta, Adyleia A D Contrera Toro","doi":"10.1002/ppul.71541","DOIUrl":"10.1002/ppul.71541","url":null,"abstract":"<p><strong>Context: </strong>Vitamin D may modulate the inflammatory processes involved in asthma.</p><p><strong>Objective: </strong>To synthesize evidence on the association between serum 25-hydroxyvitamin D levels and asthma control, severity, pulmonary function, and inflammatory markers in children and adolescents.</p><p><strong>Data sources: </strong>MEDLINE PubMed, BIREME, EBSCOhost, Scopus, Web of Science, EMBASE, Cochrane Library, and ProQuest until May 2025.</p><p><strong>Study selection: </strong>Eligible studies enrolled participants aged 2-18 years with clinically diagnosed asthma and evaluated serum vitamin D levels in relation to asthma severity and/or control and pulmonary function parameters or type 2 inflammatory biomarkers. We included observational and randomized studies.</p><p><strong>Data extraction: </strong>The risk of bias for studies was assessed. Random-effects models estimated pooled outcomes. The heterogeneity was assessed using Cochran's Q and the I² statistic.</p><p><strong>Results: </strong>Forty-one studies (7780 participants) were included; 25 contributed to meta-analyses. Children with asthma had significantly lower serum 25(OH)D levels than healthy controls (mean difference -4.89 ng/mL; 95% CI -7.38 to -2.40; p < 0.001). Severe asthma was associated with lower vitamin D compared with mild disease (-4.21 ng/mL; 95% CI -6.43 to -1.98; p = 0.0002). No significant difference was observed between controlled and uncontrolled asthma. Correlations with pulmonary function were weak and non-significant (FEV₁ r = 0.18; p = 0.08). Vitamin D showed a moderate inverse association with total IgE (r = -0.37; p = 0.02), but not with eosinophil counts or IL-10. Heterogeneity was high across analyses.</p><p><strong>Conclusions: </strong>Children with asthma exhibit lower serum vitamin D levels compared with healthy peers, and these levels are inversely associated with asthma severity and total IgE. No consistent associations were observed with pulmonary function or asthma control. Further research is needed to determine whether correcting vitamin D deficiency can improve clinical and immunologic outcomes in pediatric asthma.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 3","pages":"e71541"},"PeriodicalIF":2.3,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12947299/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147309032","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":"MiniBAL as a Diagnostic Alternative in Alveolar Hemorrhage in a Critically Ill Pediatric Patient.","authors":"Diana Carolina De la Hoz, Miguel Ricardo Suarez, Sarah Pulido-Fentanes, Jacqueline Piñeros-Haiek, Angela Maria Ortega-Rodriguez","doi":"10.1002/ppul.71558","DOIUrl":"10.1002/ppul.71558","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 3","pages":"e71558"},"PeriodicalIF":2.3,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147378291","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}