Pediatric Pulmonology最新文献

{"title":"Ventilatory Response to CO₂ During Exercise in Patients With Congenital Central Hypoventilation Syndrome.","authors":"Kenta Ikeda, Hisaya Hasegawa, Yosuke Yamada, Masanori Wasa, Shoichiro Kanabuchi","doi":"10.1002/ppul.71767","DOIUrl":"10.1002/ppul.71767","url":null,"abstract":"<p><strong>Background: </strong>Congenital central hypoventilation syndrome (CCHS) is characterized by a markedly impaired ventilatory response to CO₂ due to central chemoreceptor dysfunction. Although inadequate ventilation and hypercapnia during exercise have been reported, ventilatory response during exercise has not been quantitatively evaluated in CCHS. This study aimed to quantify ventilatory response during exercise and compare it with that at rest.</p><p><strong>Methods: </strong>Ten patients with genetically confirmed CCHS underwent cardiopulmonary exercise test (CPET) using a stepwise incremental treadmill protocol with breath-by-breath respiratory gas analysis. CPET was terminated when end-tidal CO₂ (EtCO₂) increased by at least 2% (15.2 mmHg) from baseline. Ventilatory response to CO₂ (VRCO₂) was calculated as Δminute volume (MV)/ΔEtCO₂/body weight (mL/min/mmHg/kg). VRCO₂ at rest was measured using the CO₂ rebreathing method. Data are presented as median (interquartile range [IQR]).</p><p><strong>Results: </strong>Age was 13.5 (12.3-15.5) years, and exercise duration was 5 min 53 s (4 min 47 s-6 min 56 s). Although MV increased during exercise, SpO₂ decreased and EtCO₂ increased, indicating hypoventilation. VRCO₂ during exercise was 2.27 (1.61-3.16), not significantly different from VRCO₂ at rest, 1.56 (1.07-2.83) (p = 0.85). VRCO₂ was markedly lower than values in healthy children (34.6 (29.3-42.8)). No patients reported dyspnea, and no serious adverse events occurred.</p><p><strong>Conclusions: </strong>VRCO₂ during exercise remains profoundly low in CCHS. No significant difference from VRCO₂ at rest was detected, suggesting persistent impairment of CO₂-driven ventilatory control despite increased metabolic demand. These findings provide insight into exercise-related hypoventilation in CCHS and underscore the importance of objective physiological assessment to support cautious and individualized clinical judgment rather than subjective symptoms.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71767"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13422129/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148620976","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 Nocturnal Non-Invasive Positive Pressure Support Adherence in Children and Young Adults With Duchenne Muscular Dystrophy.","authors":"John E Pascoe, Neepa Gurbani, Daniel Ignatiuk, Carolyn Grad, Danielle M Simmons, Mark Sketch, Olivia Rolsen, Lisa Mullen, Christine L Schuler, Hemant Sawnani","doi":"10.1002/ppul.71765","DOIUrl":"10.1002/ppul.71765","url":null,"abstract":"<p><strong>Background: </strong>Patients with Duchenne muscular dystrophy (DMD) have progressive muscular weakness, respiratory decline and often have poor adherence to nocturnal non-invasive positive airway pressure ventilation (NIPPV).</p><p><strong>Methods: </strong>In 2020, we sought to increase nocturnal NIPPV adherence (≥ 4 h use overnight on at least 65% of nights) from 33% to 75% by December 2024 at a pediatric neuromuscular center. We used quality improvement (QI) methods and identified key drivers (follow-up, education, staff engagement, consistent documentation). Interventions included: assuring timely follow-up (within ≤ 14 months), pre-visit planning, reminders to bring NIPPV equipment to clinic, addressing adherence barriers and partnering with psychologists. Improving overnight NIPPV adherence was the primary outcome measure. Process measures included the proportion of patients with: (1) NIPPV equipment in the clinic, (2) timely follow-up, and (3) a properly documented follow-up call. Measures were tracked on run charts.</p><p><strong>Results: </strong>Baseline median adherence improved from 33% to 60% in 8 months, and subsequently to 74% (n = 181). The proportion of patients with NIPPV machines in the clinic increased (53%-82%), as did the proportion of patients with timely follow-up (81%-95%). Common patient-reported (n = 123) adherence barriers included: \"sleeping well without NIPPV\" (20%), \"air pressure discomfort\" (14%) and \"falling asleep before putting it on\" (14%). Common parent-reported barriers were: \"forget to use\" (17%) and \"fall asleep before putting it on\" (15%).</p><p><strong>Conclusion: </strong>Nocturnal NIPPV adherence improved in youth with DMD using QI methods. Tracking adherence automatically via equipment records, further investigating barriers and minimizing discomfort with NIPPV may further improve adherence.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71765"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13425040/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148631131","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":"Complete Tracheal Rings and Congenital Diaphragmatic Hernia: A Case Series.","authors":"Brooke Gustafson, Bailey Young","doi":"10.1002/ppul.71795","DOIUrl":"10.1002/ppul.71795","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71795"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13477619/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148761085","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":"Viral Induced Hypersecretion of Mucous (VIper): Proposal for a Distinct Phenotype of Viral Induced Lower Respiratory Tract Infection in Children.","authors":"John Massie, Olivia Baenziger","doi":"10.1002/ppul.71801","DOIUrl":"10.1002/ppul.71801","url":null,"abstract":"<p><p>Some children with an acute viral lower respiratory tract illness present with a phenotype that overlaps with, but is distinct from, that of asthma. These children have shortness of breath with increased work of breathing, wet cough, hypoxia out of proportion to the work of breathing, incomplete resolution of hypoxia with supplemental oxygen, worsening of oxygen saturation after salbutamol and improvement of oxygen saturation with coughing. The pathophysiology appears to relate to relate to excess mucous secretion in the airways causing ventilation:perfusion (V/Q) mismatching, which is then exacerbated by salbutamol use. A descriptive label for this condition is Viral Induced hypersecretion of mucous (VIper). Since this has yet to receive formal recognition as a subtype of viral induced lower respiratory tract infection in children, there are no published therapeutic trials of management. This letter details the proposed pathophysiological mechanisms of mucous hypersecretion and V?Q mismatching. The value of considering VIper as a distinct subset of viral lower respiratory tract infection is to prevent harmful treatments and offer the opportunity for prospective trials of effective therapies.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71801"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13501211/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148809046","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 \"Early Life Food Desert Status Is Associated With Alpha and Gamma Tocopherol Levels and Infant Lung Function\".","authors":"Kishankumar Mahida, Snehal Rajendra Jagtap","doi":"10.1002/ppul.71754","DOIUrl":"10.1002/ppul.71754","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71754"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13402906/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148592841","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":"National Case-Series of Pediatric Mycoplasma pneumoniae Pleural Empyema.","authors":"Sophie Vennard, Don S Urquhart, Florian Gahleitner, James Stewart, Ross Langley, Ivory Huang, Anthony Brown, Jonathan Twynam-Perkins, Thomas Williams","doi":"10.1002/ppul.71753","DOIUrl":"10.1002/ppul.71753","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71753"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13403609/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148592953","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 \"Early Life Food Desert Status Is Associated With Alpha and Gamma-Tocopherol Levels and Infant Lung Function\".","authors":"Kotina Shridevi, Megha Doiphode, Rakhi Mishra, Archana Dhyani, Karen Jaison","doi":"10.1002/ppul.71752","DOIUrl":"10.1002/ppul.71752","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71752"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13402911/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148592901","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":"Pseudotumoral Tuberculosis in Children: Two Case Reports.","authors":"Ramakrishna Golla, Nisha Sahoo, Ketan Kumar, Krishna Mohan Gulla, Rashmi Ranjan Das","doi":"10.1002/ppul.71802","DOIUrl":"https://doi.org/10.1002/ppul.71802","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71802"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13487350/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148796592","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":"Primary Ciliary Dyskinesia: A Suggested Pathway to Transition From Pediatric to Adult Care.","authors":"Abel De Castro, Melanie Sue Collins, Raksha Jain, Jennifer B Walsh, Juliana Alba-Suárez, Yadira M Rivera-Sánchez","doi":"10.1002/ppul.71780","DOIUrl":"10.1002/ppul.71780","url":null,"abstract":"<p><p>Primary ciliary dyskinesia (PCD) is a rare, genetically heterogeneous disorder characterized by motile ciliary dysfunction that impairs mucociliary clearance. Symptoms of PCD can include unexplained neonatal respiratory issues, chronic sino-otopulmonary symptoms, recurrent infections, bronchiectasis, and subfertility. It is expected that the number of adult patients with PCD is four times higher than the number of pediatric patients. There are no established guidelines for transitioning individuals with PCD from pediatric to adult clinics. Although the exact prevalence is difficult to determine, it is estimated that up to 13% of adults with non-CF bronchiectasis have PCD. The Six Core Elements of Health Care Transition, developed by Got Transition, funded by the National Alliance to Advance Adolescent Health, and endorsed by the American Academy of Pediatrics, the American Academy of Family Physicians, and the American College of Physicians, can serve as a framework for transitioning PCD patients to adult care. Programs implementing the Six Core Elements have significantly reduced care dropouts during adolescence and early adulthood and increased engagement in care in early adulthood. PCD has been the subject of growing research aimed at improving diagnostic methods, treatments, and awareness, leading to earlier and more accurate diagnoses in both children and adults. Additionally, more adult PCD centers are seeking accreditation from the PCD Foundation and collaborating with pediatric centers to ensure continuity of care. Although many chronic diseases, such as cystic fibrosis (CF), inflammatory bowel disease (IBD), and, more recently, sickle cell disease, have established transition guidelines, PCD presents unique challenges that affect the transition process, as discussed in this work. We outline a pathway for transitioning pediatric patients with PCD to adult care.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71780"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13470829/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148722217","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":"Asthma Dysbiosis Index: A Universal Airway Microbiome Signature From Multi-Cohort Analysis.","authors":"Mehmet Demirci","doi":"10.1002/ppul.71796","DOIUrl":"10.1002/ppul.71796","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71796"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13479934/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148761455","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}