Pediatric Pulmonology最新文献

{"title":"Utility of Fecal Elastase-1 in Estimating Exocrine Pancreatic Function in Cystic Fibrosis: A Scoping Review.","authors":"Senthilkumar Sankararaman, Sara Hendrix, Mandy Neudecker, Drucy Borowitz","doi":"10.1002/ppul.71649","DOIUrl":"https://doi.org/10.1002/ppul.71649","url":null,"abstract":"<p><strong>Background: </strong>In people with cystic fibrosis (pwCF), identification of exocrine pancreatic insufficiency (EPI) is essential to prevent steatorrhea and, if not managed actively, can lead to catastrophic consequences. Fecal elastase-1 (FE-1) is a widely used test to screen for EPI in cystic fibrosis (CF). Once thought permanent, some patients with EPI on CF transmembrane conductance regulator (CFTR)-directed therapies (modulators) have been noted to show improvement in EPI. Here, we evaluated the utility of FE-1 in pwCF.</p><p><strong>Methodology: </strong>We performed a scoping review and searched several databases for the terms/concepts of \"cystic fibrosis\" and \"fecal elastase-1\" and their synonyms. The search period was from 1-1-2003 to 7-31-2025. We included all age groups and used a web-based platform for compiling and sorting out the articles.</p><p><strong>Results: </strong>We combined the eligible studies to synthesize information on four questions: Utilization of FE-1 in CF (when to screen for EPI, how often to screen); Comparison of diagnostic accuracy of FE-1 versus other tests; Definition of ideal cut-off for FE-1 in evaluating EPI in CF; Utilization of FE-1 in pwCF managed on CFTR-directed therapies.</p><p><strong>Conclusion: </strong>All pwCF should have EPI evaluated at diagnosis. Among various tests, FE-1 is the most commonly utilized test for screening EPI. A value of < 200 μg/g of stool is consistent with EPI and is highly sensitive for EPI diagnosis. A value of < 100 μg/g is highly specific for severe EPI. Repeating FE-1 should be considered in the current era of modulators, especially with a change in clinical status.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 5","pages":"e71649"},"PeriodicalIF":2.3,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13142209/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147841127","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":"Challenges to Assessing the Prevalence of Cystic Fibrosis in the Caribbean.","authors":"Krystal L Rivera-Figueroa, Cole L Martin, Christina A Le, Wilfredo De Jesús Rojas, Leandra Cordero Oñate, Stephen G Aller","doi":"10.1002/ppul.71648","DOIUrl":"10.1002/ppul.71648","url":null,"abstract":"<p><p> : Cystic fibrosis (CF) is likely underdiagnosed in Caribbean populations due to non-representative cystic fibrosis transmembrane conductance regulator (CFTR) variant screening panels, limited newborn screening programs, and structural healthcare barriers. Data from 2022 indicate substantial populations with European ancestry in Puerto Rico (1.4 M, 42.7%) and the Dominican Republic (1.4 M, 57.9%), yet the true burden of CF in the broader Caribbean remains largely undocumented.</p><p><strong>Working hypothesis: </strong>Current diagnostic frameworks, largely based on European-derived CFTR variant distributions, fail to capture the true burden of CF in Caribbean populations, leading to underestimated prevalence and delayed or missed diagnoses.</p><p><strong>Objectives: </strong>To synthesize registry, clinical, and published data to identify barriers to accurately assessing CF prevalence in Caribbean populations.</p><p><strong>Study design and methods: </strong>This narrative literature review integrates CF registries, published data on CFTR variant distribution, population ancestry data, and clinical observations from CF centers in Puerto Rico and the Dominican Republic. Clinical insights were derived from pediatric patients evaluated at the Pediatric Rare Lung and Asthma Institute in Puerto Rico and the CF Clinic at Robert Reid Cabral Children's Hospital in the Dominican Republic.</p><p><strong>Results: </strong>CFTR variant patterns differ from those in the United States, with higher frequencies of rare variants such as p.Ala559Thr. Standard screening panels may miss these variants, contributing to underdiagnosis. Limited newborn screening, misdiagnosis, and restricted access to CFTR modulator therapies further exacerbate disparities.</p><p><strong>Conclusions: </strong>Structural, diagnostic, and genetic factors hinder accurate CF prevalence estimates in the Caribbean, highlighting the need for region-specific research, improved screening, and expanded access to therapies.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 5","pages":"e71648"},"PeriodicalIF":2.3,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13146133/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147841538","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":"Prevalence, Severity, and Associated Risk Factors of Obstructive Sleep Apnea in a Pediatric Aerodigestive Population.","authors":"Sandhya Kalavacherla, Tzyynong L Friesen, Jeremy Landeo-Gutierrez, Ethan D Frank, Thu Elizabeth Duong, Matthew T Brigger, Aparna Rao","doi":"10.1002/ppul.71630","DOIUrl":"https://doi.org/10.1002/ppul.71630","url":null,"abstract":"<p><strong>Objectives: </strong>To characterize obstructive sleep apnea (OSA) and its severity in a referred pediatric population undergoing polysomnography (PSG) evaluation at an aerodigestive clinic.</p><p><strong>Study design: </strong>We conducted a single institution, retrospective cohort study of 234 aerodigestive children who underwent diagnostic PSG for OSA over 5 years. Demographic, symptom, and respiratory characteristics were collected. Patients' medical complexity scores (MCS), a comorbidity burden metric on a scale of 0-7, were calculated via institution-specific criteria. Kruskal-Wallis rank sum and Fisher's exact tests compared sociodemographic and clinical characteristics by OSA severity. Logistic multivariable regression models identified factors associated with a moderate/severe OSA diagnosis.</p><p><strong>Results: </strong>A total of 86 children underwent diagnostic PSG. The median age at first study was 2 years; 53% were male, 27% Non-Hispanic White, and 49% Hispanic. OSA was diagnosed in 92% of those who completed a PSG, and the OSA severity was mild in 45.3%, moderate in 26.7%, and severe in 19.8%. Median MCS was 4 with no significant differences between OSA severity groups. Female sex (OR 2.51), age > 9 years (OR 3.22), and genetic/syndromic diagnoses (OR 2.44) were significantly associated with moderate/severe OSA. Multimodal treatments with pharmacologic therapy, respiratory support, and surgery were most effective in mitigating disease progression compared to single-modalities (p = 0.002).</p><p><strong>Conclusions: </strong>OSA was highly prevalent in this pediatric aerodigestive cohort, with increased likelihood of moderate/severe disease in females, older children, and those with genetic/syndromic diagnoses. We observed no association between OSA severity and higher medical complexity. Consequently, OSA screening can be considered in all aerodigestive children.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 4","pages":"e71630"},"PeriodicalIF":2.3,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147778130","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":"Premature Infants With Pulmonary Complications Exhibit Decreased Skin Maturity Early After Birth: A Cross-Sectional Study.","authors":"Samanta Cris Monteiro Frota, Luiza Eduarda Silva de Macedo, Larissa Queiroz Oliveira, Ingrid Fonseca Damasceno Bezerra, Anna Christina do Nascimento Granjeiro Barreto, Ingrid Guerra Azevedo, Roberta Lins Gonçalves, Daniele Soares-Marangoni, Gabriela Silveira Neves, Carolina Daniel de Lima-Alvarez, Silvana Alves Pereira","doi":"10.1002/ppul.71562","DOIUrl":"10.1002/ppul.71562","url":null,"abstract":"<p><strong>Introduction: </strong>Early identification of respiratory risk in preterm newborns (PTNB) remains challenging, as direct assessment of pulmonary maturity is often unavailable or requires invasive or resource-intensive methods. Non-invasive biomarkers capable of indicating physiological maturity shortly after birth could improve early clinical decision-making in neonatal care. Because skin and lung development share common biological pathways, skin maturity assessed by optical reflectance may serve as an indirect marker of pulmonary development.</p><p><strong>Objective: </strong>This study evaluated the association between skin maturity estimated by skin reflectance and respiratory outcomes in PTNB during the first 48 hours of life.</p><p><strong>Methods: </strong>A cross-sectional study was conducted with PTNB born admitted within 48?h. Exclusion criteria included cutaneous malformations, or conditions interfering with dermal assessment. Skin maturity was measured using the non-invasive Preemie-Test® device. Respiratory outcomes, such as chest radiographic abnormalities, pneumothorax, atelectasis, and pulmonary hemorrhage, were defined by clinical and radiological criteria.</p><p><strong>Results: </strong>A total of 82 PTNB were included, 54.9% male. Skin reflectance ranged from 0.9 to 2.1?nm. Lower skin maturity was significantly associated with radiographic abnormalities (p = 0.01), pneumothorax (p = 0.01), pulmonary hemorrhage (p = 0.01), atelectasis (p = 0.01), and need for invasive ventilation (p = 0.01).</p><p><strong>Conclusions: </strong>These findings suggest skin reflectance may serve as a rapid, objective, and non?invasive screening tool for neonatal respiratory risk.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 4","pages":"e71562"},"PeriodicalIF":2.3,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13051638/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147623553","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":"Improving Pancreatic Function With Elexacaftor/Tezacaftor/Ivacaftor.","authors":"Madison Pranske, Alizay Paracha, Carson Loncarich, Amy D Hendrix-Dicken, Joseph Walter, Michelle Condren","doi":"10.1002/ppul.71616","DOIUrl":"10.1002/ppul.71616","url":null,"abstract":"<p><strong>Background: </strong>Due to the dysfunction and destruction of pancreatic cells in individuals with cystic fibrosis (CF), most require lifelong exogenous pancreatic enzyme supplementation. The objective of this study was to determine if elexacaftor/tezacaftor/ivacaftor (ETI) treatment improves pancreatic function in individuals with CF.</p><p><strong>Methods: </strong>A retrospective study was conducted utilizing CF Foundation Patient Registry data for individuals 0-21 years receiving care at the Tulsa CF Center. Data collected included demographic variables, fecal elastase (FE-1) values, BMI percentile, and CFTR mutations. Pancreatic sufficiency was defined as \"severe\" (< 100), \"moderate\" (100-200), and \"sufficient\" (> 200). Descriptive statistics, and Spearman Correlation post-hoc analysis (p < 0.05) were performed.</p><p><strong>Results: </strong>The majority of individuals with a post-ETI FE-1 value (n = 49) were homozygous for the F508del mutation (n = 38, 78%). After ETI, most individuals had severe pancreatic insufficiency (n = 39, 79.6%) followed by 10.2% (n = 5) with moderate insufficiency and 10.2% (n = 5) with sufficiency. The median time between ETI initiation and highest post-FE-1 ranged from 14.07 months (IQR: 8.78-14.07) for the pancreatic sufficient group to 18.87 months (IQR: 10.42-33.04) for the severe pancreatic insufficiency group. The five individuals achieving post-ETI sufficiency had a mean time of 10.95 months (SD = 6.18) between start of therapy and conversion to pancreatic sufficiency. Age at ETI start and FE-1 value were moderately negatively correlated (p = 0.0024) with pancreatic sufficient individuals having the youngest median initiation age (5.28 years, IQR: 2.95-5.54). All pancreatic sufficient individuals at the post-ETI assessment had homozygous F508del CFTR mutations (n = 5). Twenty-four individuals had both pre-and-post-ETI FE-1 values. Of those 24, two converted (8.3%) to moderate sufficiency, and three (12.5%) to pancreatic sufficient.</p><p><strong>Conclusion: </strong>While exploring the impacts of ETI therapy on pancreatic function, this study revealed a potential reversal of pancreatic insufficiency. The results suggest the importance of early initiation of therapy and standardized protocols to monitor FE-1 values over time.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 4","pages":"e71616"},"PeriodicalIF":2.3,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147634234","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":"Characterization of Pulmonary Dysfunction in Systemic Juvenile Idiopathic Arthritis Using Xenon and Proton MRI.","authors":"William J Garrison, Matthew M Willmering, Bilal I Masokano, Shipra Rai, Antoinette Wannes Daou, David J Roach, Esi M Morgan, Jason C Woods, Zackary I Cleveland, Emrah Gecili, R Paul Guillerman, Andrew H Schapiro, Grant S Schulert, Alexei A Grom, Christopher T Towe, Laura L Walkup","doi":"10.1002/ppul.71613","DOIUrl":"10.1002/ppul.71613","url":null,"abstract":"<p><strong>Introduction: </strong>Systemic juvenile idiopathic arthritis-associated lung disease (SJIA-LD) is increasingly recognized and associated with potentially life-threatening complications. Diagnosis is challenging as SJIA-LD is complex and frequently presents with subtle or no respiratory symptoms, necessitating CT imaging or other tests to detect budding dysfunction. We hypothesized that xenon and proton lung MRI could identify SJIA-LD-associated structural changes and functional deficits.</p><p><strong>Methods: </strong>Thirty-one participants aged 5-15 (16 healthy, 7 SJIA without diagnosed lung disease [SJIA-non-LD], 8 SJIA-LD) underwent MRI for this cross-sectional study. Participants underwent xenon ventilation MRI (14 healthy/6 SJIA-non-LD/7 SJIA-LD), xenon gas-exchange MRI (12 healthy/3 SJIA-non-LD/5 SJIA-LD), and/or proton ultrashort echo time (UTE) MRI (12 healthy/5 SJIA-non-LD/7 SJIA-LD). Ventilation defect percentage (VDP) and ratios of xenon signal in alveolar membrane (Membrane), red blood cells (RBC), and gas were calculated from xenon MR images. CTs and PFTs were obtained from participant medical records where available for comparison.</p><p><strong>Results: </strong>RBC/Membrane differed significantly (p = 0.014) between healthy individuals and SJIA-LD patients, and corresponded with DLCO results in a limited sample of participants with both measurements. VDP did not differ significantly between groups (p = 0.30), but was abnormally high in 3/7 SJIA-LD patients. SJIA-LD pattern findings in UTE MRI were detected in 4/7 SJIA-LD patients and in no healthy or SJIA-non-LD participants (p < 0.001).</p><p><strong>Conclusion: </strong>Xenon and proton lung MRI can identify SJIA-LD-associated pulmonary abnormalities, with RBC/Membrane showing particular promise for characterization of pulmonary diffusion limitation in SJIA-LD. Future studies will evaluate lung MRI for SJIA-LD phenotyping and longitudinal monitoring of SJIA-associated lung function changes.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 4","pages":"e71613"},"PeriodicalIF":2.3,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13058687/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147634275","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":"Impact of COVID-19 and CFTR Modulators on Cystic Fibrosis: A Real-World Analysis of Care Patterns.","authors":"Alexandra C Hinton, Edmund H Sears, Sara Lopez-Pintado, Jonathan B Zuckerman","doi":"10.1002/ppul.71595","DOIUrl":"10.1002/ppul.71595","url":null,"abstract":"<p><strong>Introduction: </strong>Novel therapeutics and rapid expansion of telehealth have reshaped cystic fibrosis (CF) care; however, the impact on visit patterns and equitable access across the CF population remains unclear. We characterized changes in visit patterns from 2017 to 2022 and the association of sociodemographic and clinical factors with visit frequency in 2022.</p><p><strong>Methods: </strong>This observational cohort study analyzed 2017-2022 US CF Foundation Patient Registry data for people aged 6-65 years. We described trends in care utilization and used adjusted longitudinal mixed-effects models to evaluate predictors of between-visit interval (BVI).</p><p><strong>Results: </strong>The study included 28,340 people and 463,745 encounters. Average BVI increased 22.6% from 2019 to 2022, with larger increases among adults. The effect of COVID-related changes and highly effective modulator use on BVI (average increase in BVI of 13.5 and 9.3 days, respectively) were roughly additive for those with concomitant exposures. Starting elexacaftor/tezacaftor/ivacaftor (ETI) initially shortened BVI, followed by sustained increases. Non-White and Hispanic individuals had shorter BVI than their White, non-Hispanic counterparts. After adjustment, insurance changes and greater residential distance from CF centers were associated with longer BVI.</p><p><strong>Conclusion: </strong>Advances in CF therapeutics and COVID-19-related care adaptations have both markedly influenced visit frequency, indicating evolving care models responsive to the needs of people with CF. Persistent disparities by race, insurance status, and geographic location reveal ongoing challenges in achieving equitable access to routine CF care. Targeted strategies to address these inequities and enhance integrated care are essential to optimize outcomes for people with CF.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 4","pages":"e71595"},"PeriodicalIF":2.3,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13051524/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147623480","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":"Endobronchial Cryobiopsy of Pediatric Airway Masses-Discussing Its Role Through Two Case Reports.","authors":"Ramakrishna Golla, Nisha Sahoo, Krishna Mohan Gulla, Rashmi Ranjan Das, Manoj Kumar Panigrahi, Ranjan Kumar Patel, Mukund Namdev Sable, Pritinanda Mishra, Kanishka Das, Sundeep M C Kisku, Ketan Kumar","doi":"10.1002/ppul.71636","DOIUrl":"https://doi.org/10.1002/ppul.71636","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 4","pages":"e71636"},"PeriodicalIF":2.3,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147777763","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":"To the Editor, \"Prevalence of Adverse Childhood Experiences in Children With Cystic Fibrosis at a Single Center\".","authors":"Wadsworth A Williams, Brittany Bedford, Susanna A McColley","doi":"10.1002/ppul.71593","DOIUrl":"10.1002/ppul.71593","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 4","pages":"e71593"},"PeriodicalIF":2.3,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13034398/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147575080","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":"Repeatability of Multiple Breath Washout in Pediatric Primary Ciliary Dyskinesia.","authors":"Muhammad Ahmad, Mian Zain Hayat, Mohsin Tariq","doi":"10.1002/ppul.71617","DOIUrl":"10.1002/ppul.71617","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 4","pages":"e71617"},"PeriodicalIF":2.3,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147634242","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}