Andrea Carolina Munive Gnecco, Isabella Andrea Bolaños Bermúdez, Luis Fernando Pulido, Gustavo Adolfo Triana Rodríguez
{"title":"Re-Entry High-Altitude Pulmonary Edema in a Child.","authors":"Andrea Carolina Munive Gnecco, Isabella Andrea Bolaños Bermúdez, Luis Fernando Pulido, Gustavo Adolfo Triana Rodríguez","doi":"10.1002/ppul.71811","DOIUrl":"10.1002/ppul.71811","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 9","pages":"e71811"},"PeriodicalIF":2.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13505238/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148813759","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":"Evaluation of Pulmonary and Otological Manifestations in Primary Ciliary Dyskinesia and Their Effect on Quality of Life.","authors":"Muruvvet Yanaz, Ela Erdem Eralp, Cansu Yilmaz Yegit, Aynur Gulieva, Mine Kalyoncu, Merve Selcuk Balci, Seyda Karabulut, Nuru Aliyev, Nezafet Ozturk, Almala Pınar Ergenekon, Yasemin Gokdemir, Ali Cemal Yumusakhuylu, Ayca Ciprut, Bulent Karadag","doi":"10.1002/ppul.71810","DOIUrl":"10.1002/ppul.71810","url":null,"abstract":"<p><strong>Objective: </strong>Otitis media and conductive hearing loss are common in patients with primary ciliary dyskinesia (PCD). We primarily aimed to assess the impact of otologic features of individuals with PCD on health-related quality of life (QOL), and our secondary aims are characterization of the coexisting pulmonary and sinonasal findings.</p><p><strong>Methods: </strong>Demographic data, spirometry values, a health-related quality of life questionnaire for PCD patients (QOL-PCD), physical examination data of sinonasal and otologic systems, the sinonasal outcome test-22, and audiologic tests were recorded in this cross-sectional study. The pure tone audiometry, distortion product otoacoustic emission (DPOAE), tympanogram, and the Turkish matrix test (TUR-matrix) evaluating speech understanding in noise were performed.</p><p><strong>Results: </strong>Fifty-six patients with PCD were included in the study. The scores of the social, upper, and lower respiratory domains of the QOL-PCD were significantly lower in adults than in children and adolescents (p-value < 0.001, 0.02, and 0.01, respectively). The hearing and the TUR-matrix were pathologic in 26 (46.4%) and 36 (64.3%) cases, respectively. Patients with pathological hearing had significantly worse results in regards to the FEV<sub>1</sub> z-score (-1.7 vs. -0.8, p = 0.01), the TUR-matrix test (20 vs. 16 cases, p = 0.001), the DPOAE (25 vs. 11 cases, p < 0.001), and the tympanogram (26 vs. 12 cases, p < 0.001) than patients with normal hearing.</p><p><strong>Conclusion: </strong>Sinopulmonary involvement and hearing manifestations are very common in patients with PCD, and speech understanding in noise is often impaired. These manifestations impact QOL in various areas.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 9","pages":"e71810"},"PeriodicalIF":2.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13507764/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148819220","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}
N Beullens, K Smets, M De Lausnay, P Schelstraete, S Van Daele, J Willekens, J Van Dorpe, H Schaballie
{"title":"Neonatal Course and Long-Term Outcome in Children With Congenital Chylothorax.","authors":"N Beullens, K Smets, M De Lausnay, P Schelstraete, S Van Daele, J Willekens, J Van Dorpe, H Schaballie","doi":"10.1002/ppul.71820","DOIUrl":"10.1002/ppul.71820","url":null,"abstract":"<p><strong>Background: </strong>Congenital chylothorax is a rare pulmonary developmental disorder with high associated morbidity and mortality. A limited number of cases have been described, and clinical manifestations and outcomes vary.</p><p><strong>Methods: </strong>This study aims to delineate the neonatal and long-term outcome of patients with congenital chylothorax by retrospective and prospective data collection at Ghent University Hospital in Belgium between 1992 and 2022.</p><p><strong>Results: </strong>Eighteen patients with congenital chylothorax were included, seven of whom had a pathology-confirmed diagnosis of congenital pulmonary lymphangiectasia. Of 16 patients with prenatal diagnosis, four underwent an intra-uterine intervention. Thirteen patients were born prematurely. The most common comorbidities were hydrops (8/18), pulmonary hypertension (3/18), and reflux disease (6/18). One patient was diagnosed with Noonan syndrome. The majority of patients (14/18) required mechanical ventilation. Treatment included pleural drainage (15/18), total parenteral nutrition (14/18), medium-chain triglyceride diet (11/18), somatostatin (8/18), and surgical intervention (3/18), with a median hospital stay of 59 days. Three patients died during or after the neonatal period. Follow-up ranged from 6 months to 30 years, with most patients reporting good general health but variable respiratory complaints. Five out of eight respiratory function tests showed mild abnormalities. Follow-up chest CT showed persistent changes in all but two patients up to a maximum age of 16 years. Health-related quality of life was similar to healthy controls.</p><p><strong>Conclusion: </strong>Congenital chylothorax demonstrates a variable neonatal course. Long-term outcomes are generally favorable in patients without comorbidities, although mild pulmonary function and chest CT abnormalities persist in the majority of patients.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 9","pages":"e71820"},"PeriodicalIF":2.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13539660/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148888263","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 Patterns of Children's Interstitial and Diffuse Lung Disease (chILD) in Colombia, 2016-2024.","authors":"Andrea Parra Buitrago, Jefferson Antonio Buendía","doi":"10.1002/ppul.71832","DOIUrl":"10.1002/ppul.71832","url":null,"abstract":"<p><strong>Background: </strong>Children's interstitial and diffuse lung disease (chILD) comprise a heterogeneous group of rare disorders associated with substantial morbidity and mortality. Population-based epidemiological data remain scarce in low- and middle-income countries, limiting health system planning and policy development. We aimed to estimate the prevalence and temporal trends of chILD in Colombia using nationwide administrative healthcare data.</p><p><strong>Methods: </strong>We conducted a retrospective, population-based study using Colombia's Registro Individual de Prestación de Servicios de Salud (RIPS) from 2016 to 2024. chILD cases were identified using ICD-10 codes for chILD in individuals aged 0-18 years. Prevalence was estimated overall, by year, sex, age group, and diagnosis. Crude and WHO age-standardized prevalence rates were calculated per 100,000 population. Temporal trends were assessed using Poisson regression and LOESS smoothing.</p><p><strong>Results: </strong>A total of 4608 chILD -related healthcare encounters were identified. The average annual crude prevalence was 3.32 per 100,000 children (age-standardized: 3.39 per 100,000). Prevalence was highest in infants younger than 1 year and showed a secondary increase during adolescence. No relevant sex differences were observed. A significant annual increase in reported cases was detected (IRR 1.022; 95% CI 1.011-1.034), with a marked decline during the COVID-19 pandemic. Alveolar and fibrotic interstitial lung disease categories accounted for most cases.</p><p><strong>Conclusions: </strong>This first nationwide study provides robust real-world evidence on the epidemiology of chILD in Colombia, revealing substantial healthcare utilization and distinct age-related patterns. Administrative data represents a valuable complementary tool for surveillance of rare pediatric lung diseases.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 9","pages":"e71832"},"PeriodicalIF":2.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13572882/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148963097","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":"A Case Report of a Novel LTBP4 Variant in Urban-Rifkin-Davis Syndrome Presenting With Lobar Overinflation in an Adolescent Girl.","authors":"Kkomal C Suvarna, Kalyana Prabhakaran, Sathya Srivatsav, Taruna Yadav, Prawin Kumar, Jagdish Prasad Goyal","doi":"10.1002/ppul.71769","DOIUrl":"10.1002/ppul.71769","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71769"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13439482/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148674092","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}
Elke Huenaerts, Myriam Vreys, François Vermeulen, Marijke Proesmans, Natalie Lorent, Mieke Boon
{"title":"Physiotherapy in Primary Ciliary Dyskinesia-What Is the Evidence?: A Narrative Review and Personal Experience.","authors":"Elke Huenaerts, Myriam Vreys, François Vermeulen, Marijke Proesmans, Natalie Lorent, Mieke Boon","doi":"10.1002/ppul.71790","DOIUrl":"10.1002/ppul.71790","url":null,"abstract":"<p><p>Primary ciliary dyskinesia (PCD) is a rare, hereditary disorder characterized by impaired motile ciliary function, resulting in abnormal mucociliary clearance (MCC). This leads to persistent infection and inflammation, progressive airway damage, and bronchiectasis. Physiotherapy, particularly airway clearance therapy (ACT), is considered a cornerstone of PCD management to compensate for the mechanical MCC deficit, aiming to reduce infection risk, prevent disease progression, and improve quality of life (QOL). However, evidence supporting specific physiotherapy regimens in PCD remains limited. Current practice varies widely across centers and countries, with no standardized guidelines. Common ACT methods include positive expiratory pressure (PEP) with or without oscillation, autogenic and postural drainage, active cycle of breathing technique (ACBT), intrapulmonary percussive ventilation (IPV), and high-frequency chest wall oscillation (HFCWO). Comparative studies show no clear superiority among techniques. Evidence for mucolytic therapy, such as hypertonic saline or rhDNAse, is weak or controversial. Scarce data demonstrate potential benefits of exercise interventions in improving ventilatory capacity and QOL. Age-specific considerations are crucial: ACT should be started early, be adapted to developmental stages, and be combined with strategies to enhance adherence. Despite physiotherapy being standard care, randomized controlled trials are challenging due to ethical constraints, potentially reducing a therapy that is already believed to be helpful. Future priorities include developing international guidelines and conducting high-quality studies to optimize treatment strategies. In conclusion, physiotherapy plays a crucial role in PCD management, but current recommendations rely largely on expert opinion and extrapolation from related conditions like cystic fibrosis (CF) and non-CF bronchiectasis (BRECT), underscoring the need for robust evidence.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71790"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13477620/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148759534","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}
Allyson Tank, Layne Silver, Michael C Spaeder, Colin Rogerson, Alice Shanklin
{"title":"Effectiveness of Continuous Levalbuterol Versus Albuterol During a National Shortage: A Retrospective Observational Cohort Study.","authors":"Allyson Tank, Layne Silver, Michael C Spaeder, Colin Rogerson, Alice Shanklin","doi":"10.1002/ppul.71779","DOIUrl":"10.1002/ppul.71779","url":null,"abstract":"<p><strong>Introduction: </strong>Bronchospasm requiring continuous inhaled albuterol is a common reason for pediatric intensive care unit (PICU) admission. Albuterol's racemic composition may contribute to adverse effects; therefore, levalbuterol [(R)-albuterol] may be used. This study aimed to determine if continuous inhaled levalbuterol is associated with a shorter duration of continuous inhaled bronchodilator therapy and less tachycardia compared to albuterol in PICU patients.</p><p><strong>Methods: </strong>This study utilized two periods of exclusive continuous inhaled levalbuterol use due to an albuterol shortage. PICU encounters receiving ≥ 12 h of continuous inhaled levalbuterol during those periods were compared to encounters receiving albuterol in the interim. Therapy duration and % change in median heart rate (HR) at 6-h epochs were compared between groups, and multilevel mixed effects models were created.</p><p><strong>Results: </strong>In 686 encounters, there was no difference in therapy duration between levalbuterol and albuterol groups (31.3 vs. 28.7 h, p = 0.22). There was no difference in % change in median HR at 6 (-1.0% vs. -1.3%, p = 0.54), 12 (-1.6% vs. -2.7%, p = 0.28), or 18 h (-2.5% vs. -4.1%, p = 0.38) from therapy initiation. Levalbuterol was not associated with therapy duration adjusting for age, technology dependence, respiratory rate category, and number of continuous intravenous bronchodilators (p = 0.13). Levalbuterol was not associated with % change in median HR at 12 h following therapy initiation after adjusting for age and respiratory rate category (p = 0.99).</p><p><strong>Conclusion: </strong>Continuous inhaled levalbuterol was not associated with shorter therapy duration or lower HR compared to albuterol in PICU patients.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71779"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13448360/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148685432","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":"Sleep-Disordered Breathing in Primary Ciliary Dyskinesia.","authors":"Merve Selçuk Balcı, Ceren Ayça Yıldız, Ela Erdem Eralp, Refika Ersu","doi":"10.1002/ppul.71783","DOIUrl":"10.1002/ppul.71783","url":null,"abstract":"<p><strong>Background: </strong>Primary Ciliary Dyskinesia (PCD) is a rare genetic disorder marked by impaired mucociliary clearance, chronic upper and lower airway infections, and progressive bronchiectasis. Sleep-disordered breathing (SDB) has emerged as an under-recognized comorbidity in this population.</p><p><strong>Objective: </strong>This review synthesizes current evidence on the prevalence, pathophysiology, diagnostic approaches, and management of SDB in PCD across pediatric and adult cohorts.</p><p><strong>Results: </strong>Reported prevalence of obstructive sleep apnea (OSA) in PCD ranges widely from ~33% to 100%, markedly exceeding general population norms; however, one large recent pediatric cohort reported a much lower prevalence (~1.2%), indicating that true disease magnitude remains uncertain. Upper-airway abnormalities (chronic rhinosinusitis, nasal polyposis, adeno-tonsillar hypertrophy) are almost universal in PCD, while lower-airway disease and ventilation-perfusion mismatch contribute to nocturnal hypoxemia even in the absence of apneas. Polysomnography (PSG) remains the diagnostic gold standard; subjective questionnaires (e.g., PSQI, SDSC, ESS) show poor correlation with PSG metrics in PCD. Sleep architecture is typically preserved, but sleep efficiency is mildly reduced and arousal indices are elevated, indicative of fragmented yet largely restorative sleep. Psychological and neurocognitive sequelae, including anxiety, depressive symptoms, and attention deficits, may be seen and are associated with SDB in PCD. First-line management focuses on optimization of the upper-airway (adenotonsillectomy, nasal irrigation, intranasal corticosteroids), while residual SDB may necessitate positive airway pressure (PAP) therapy. Optimizing lung function and infection control may further improve nocturnaloxygenation.</p><p><strong>Conclusions: </strong>SDB in PCD represents a multifactorial and potentially modifiable contributor to disease burden. Incorporating sleep assessment into routine PCD care and fostering multicenter, genotype-informed research are imperative to enhance early recognition, management, and quality-of-life outcomes.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71783"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13470827/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148726998","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}
Senthilkumar Sankararaman, Terri Schindler, Aravind Thavamani, Catherine Pamer, Alison Marie Rodatt, Kimberly A McBennett, Erica Roesch, Alex H Gifford
{"title":"Utilization of Des-Gamma-Carboxy-Prothrombin in Estimating Vitamin K Status in People With Cystic Fibrosis: A Single Center Retrospective Review.","authors":"Senthilkumar Sankararaman, Terri Schindler, Aravind Thavamani, Catherine Pamer, Alison Marie Rodatt, Kimberly A McBennett, Erica Roesch, Alex H Gifford","doi":"10.1002/ppul.71777","DOIUrl":"10.1002/ppul.71777","url":null,"abstract":"<p><strong>Background: </strong>People with cystic fibrosis (CF) are at increased risk of fat-soluble vitamin (A, D, E, and K) deficiencies secondary to exocrine pancreatic insufficiency (EPI). Estimation of vitamin K deficiency in routine clinical practice is challenging, and no uniform consensus exists due to lack of an accurate clinical test for the evaluation of its status. We aimed to estimate vitamin K status in people with CF using des-gamma-carboxy-prothrombin (DCP).</p><p><strong>Methodology: </strong>A single-center retrospective chart review was done between 2022 and 2024. Ninety-four (39 pediatric and 55 adult) people with CF were included for final review.</p><p><strong>Results: </strong>The median DCP value was 0.35 ng/mL (IQR 0.2-0.60; range was 0.1-11 ng/mL). Post hoc, we classified patients into low-DCP (0-90th percentile values in DCP or ≤ 2 ng/mL) and high-DCP (91-100th percentile in DCP or >2 ng/mL) groups. Eleven out of 94 (11.7%) patients had DCP > 2 ng/mL; three out of 11 (27%) had advanced CF liver disease (aCFLD), p = 0.011, and another three had other fat-soluble vitamin deficiencies, and low adherence to pancreatic enzymes and/or vitamin supplementation was suspected, p = 0.011. There were no significant differences in demographics (age, gender) or other comorbidities between the groups.</p><p><strong>Conclusion: </strong>DCP can be used for estimating vitamin K status, and the prevalence of suboptimal vitamin K status was 11.7%. DCP levels were higher in aCFLD, as well as those with suspected low adherence to pancreatic enzymes and/or vitamin supplementation. Prospective, multicenter clinical trials are needed to evaluate this further.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71777"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13462822/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148712403","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}
Yanyan Su, Yanjuan Yu, Lei Zhang, Yu Tang, Min Li, Chaohui Chen, Shasha Xu, Yanqiong Wang, Yuemei Liu, Haiming Yang, Yuelin Shen
{"title":"Efficacy of High-Dose Versus Conventional-Dose Methylprednisolone in Reducing the Risk of Chronic Lung Disease in Chinese Children With Severe Mycoplasma pneumoniae Pneumonia: A Retrospective Study.","authors":"Yanyan Su, Yanjuan Yu, Lei Zhang, Yu Tang, Min Li, Chaohui Chen, Shasha Xu, Yanqiong Wang, Yuemei Liu, Haiming Yang, Yuelin Shen","doi":"10.1002/ppul.71762","DOIUrl":"10.1002/ppul.71762","url":null,"abstract":"<p><strong>Background: </strong>This study aimed to compare the clinical efficacy of high-dose versus conventional-dose methylprednisolone therapy and the prevalence of chronic lung disease (CLD) at the 6-month follow-up in Chinese children with severe Mycoplasma pneumoniae pneumonia (SMPP).</p><p><strong>Methods: </strong>A retrospective cohort study was performed on 82 children with SMPP admitted from January 2018 to January 2024. Patients were categorized into high-dose and conventional-dose groups based on initial glucocorticoid dosage. Clinical data, laboratory tests, chest imaging, and bronchoscopy findings were reviewed to compare pulmonary conditions and therapeutic efficacy. The prevalence of CLD, oxygen saturation levels, and pulmonary function were assessed at the 6-month follow-up.</p><p><strong>Results: </strong>The main clinical features of SMPP included persistent high fever, cough, excessive inflammation (C-reactive protein, erythrocyte sedimentation rate, procalcitonin, interleukin-6, lactate dehydrogenase, serum ferritin, and D-Dimer), as well as severe pulmonary involvement, including consolidation, pleural effusion, and lung necrosis on computed tomography, and mucus plugging on bronchoscopy. Disease severity was comparable between groups, but the high-dose group exhibited a shorter fever duration (9.8 vs. 16.1 days), faster defervescence after corticosteroid treatment (1.0 vs. 7.0 days), and reduced hospitalization (13.4 vs. 18.4 days) (all p < 0.001). At the 6-month follow-up, the high-dose group had a significantly lower prevalence of CLD (28.2% vs. 85.4%) (p < 0.001), higher oxygen saturation levels (p = 0.033) and better pulmonary function (p < 0.001).</p><p><strong>Conclusion: </strong>High-dose methylprednisolone therapy was more effective than conventional-dose treatment for SMPP patients. It was also associated with a lower prevalence of CLD, higher oxygen saturation levels, and better pulmonary function outcomes.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71762"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13420772/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148621038","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}