Pediatric Pulmonology最新文献

{"title":"Efficacy of Intravenous Methylprednisolone Pulses in Children With Postinfectious Bronchiolitis Obliterans.","authors":"Souad Ghattas, David Drummond, Alice Hadchouel","doi":"10.1002/ppul.71150","DOIUrl":"10.1002/ppul.71150","url":null,"abstract":"<p><strong>Objective: </strong>To assess the efficacy of intravenous methylprednisolone pulses in children with postinfectious bronchiolitis obliterans (PIBO).</p><p><strong>Study design/methods: </strong>This French monocentric retrospective study included 31 patients with PIBO. Respiratory symptoms, occurrence of exacerbations and hospitalizations up to 4 years, and the results of lung function tests were compared between patients who received methylprednisolone pulses (n = 6) and those who did not (n = 25). A similar analysis was performed in the subgroup of the severest patients at diagnosis (five and six patients per group respectively).</p><p><strong>Results: </strong>During their inaugural episode, patients in the treatment group were hospitalized longer (p = 0.005), more frequently in ICU (p = 0.013) and had longer oxygen needs than in the control group (p = 0.005). During their course, more patients in the treatment group required at least one nonscheduled hospitalization at 6 months of evolution than in the control group (p = 0.04). Regarding lung function tests, no significant difference was observed between the control and treatments groups. Comparing the severest patients at diagnosis in each group showed no significant difference in clinical nor functional evolution.</p><p><strong>Conclusion: </strong>In a retrospective monocentric cohort, intravenous methylprednisolone pulses were used in severe cases of PIBO. This treatment had no effect of the subsequent evolution of these patients as compared to not treated patients.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 6","pages":"e71150"},"PeriodicalIF":2.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12160995/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144275581","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":"Respiratory Manifestations in Pediatric Patients With Interleukin 6 Receptor Deficiency.","authors":"Noga Arwas, Eyal Kristal, Inbal Golan-Tripto, Aviv Goldbart, Guy Hazan, Amit Nahum, Shani Arotzker, Arnon Broides, Dvir Gatt","doi":"10.1002/ppul.71174","DOIUrl":"10.1002/ppul.71174","url":null,"abstract":"<p><p>Interleukin-6 receptor (IL-6R) deficiency is a rare immunodeficiency marked by impaired IL-6 signaling, affecting the inflammatory response and respiratory health. In this report, pediatric patients with IL-6R deficiency presented with a spectrum of respiratory symptoms, from mild to severe infections, but did not commonly develop early bronchiectasis. Due to the absence of typical inflammatory markers in lung infections, routine imaging is advised to monitor for potential complications, even in the absence of overt clinical signs.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 6","pages":"e71174"},"PeriodicalIF":2.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144326535","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":"Are Athletic Coaches Aware of Exercise-Induced Laryngeal Obstruction in Children and Adolescents?","authors":"Robert Brinton Fujiki, Kayla Lewis, Susan L Thibeault","doi":"10.1002/ppul.71173","DOIUrl":"10.1002/ppul.71173","url":null,"abstract":"<p><strong>Purpose: </strong>Onset of pediatric exercise induced laryngeal obstruction (EILO) often occurs in athletic contexts. This study investigated athletic coach awareness of EILO in children and adolescents. Strategies employed by coaches when working with athletes with EILO and exercise induced dyspnea (EID) of unknown etiology were also examined.</p><p><strong>Methods: </strong>EILO awareness was assessed using an anonymous electronic survey distributed to three types of athletic coaches: sport coaches, dance instructors, and physical education teachers. Survey distribution occurred via social media, Reddit, and sport coach/dance instructor association email listservs.</p><p><strong>Results: </strong>Three-hundred and two athletic coaches responded to the survey (mean age = 43.4 years). Overall, only 22.5% (N = 68) of survey respondents indicated familiarity with EILO. Twelve percent (N = 38) of coaches reported that they had worked directly with an athlete diagnosed with EILO. Neither sport coached, respondent age, nor years of experience predicted which coaches were familiar with EILO. Fifty-eight percent (N = 176) of athletic coaches indicated that they worked with athletes with EID of unknown etiology \"often.\" Coaches were significantly more confident working with athletes with EID as compared to EILO (p = 0.021). Coaches reported that working with athletes with dyspnea was common, however, athletes rarely disclosed breathing symptom etiology.</p><p><strong>Conclusion: </strong>Less that one fourth of athletic coaches reported any awareness of EILO. Coaches overwhelmingly indicated that better communication with athletes and increased knowledge of EILO would allow them to coach athletes more effectively. Future work should determine whether coordinating EILO and EID treatment with athletic coaches can improve treatment outcomes.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 6","pages":"e71173"},"PeriodicalIF":2.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12186470/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144476260","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":"Feasibility and Utility of Multiple Breath Washout Measured Scond and Sacin in Preschool Children With Cystic Fibrosis.","authors":"James Lynch, Renee Jensen, Michelle Shaw, Marie-Pier Dumas, Nicholas Karsli, Felix Ratjen","doi":"10.1002/ppul.71180","DOIUrl":"10.1002/ppul.71180","url":null,"abstract":"<p><strong>Background: </strong>The lung clearance index (LCI) is a sensitive measure of global ventilation inhomogeneity but does not describe the gas mixing mechanisms that lead to inhomogeneity. Multiple breath washout normalized phase III slope (SnIII) metrics may complement LCI when assessing lung disease; Scond describes convection-dependent inhomogeneity and Sacin reflects diffusion convection-interaction-dependent inhomogeneity respectively. We aim to determine the feasibility and utility of Scond and Sacin in preschool children with CF.</p><p><strong>Methods: </strong>We retrospectively assessed Scond and Sacin in successful MBW tests performed on preschool children. Scond and Sacin were calculated by visual breath-by-breath analysis according to ATS/ERS MBW consensus statement, with the exception that minimum SnIII was at least 30% of expired volume rather than between 65% and 95% of expired volume.</p><p><strong>Results: </strong>We analyzed MBW tests for 40 healthy controls and 40 participants with CF, with a mean (range) baseline age of 4.1 years (2.6-5.9). Of the 372 successful MBW tests analyzed, 77% had reportable SnIII indices. Scond was elevated in CF relative to controls (∆ 0.037; 95% CI: 0.026-0.047; p < 0.001). Both within- and between-test variability was higher for Scond and Sacin than for LCI. More clinically stable test occasions were above the upper limit of normal for Scond than for LCI (64% vs. 50%, p = 0.02).</p><p><strong>Conclusions: </strong>MBW metrics Scond and Sacin are feasible in young children. While they differentiate preschool children with CF from healthy controls, SnIII indices are more variable than LCI, limiting their interpretability.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 6","pages":"e71180"},"PeriodicalIF":2.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144476261","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":"Real-World Improvements of Lung Clearance Index and Ventilation Distribution Efficiency in Children With Cystic Fibrosis After Elexacaftor/Tezacaftor/Ivacaftor Initiation.","authors":"Esben Herborg Henriksen, Rikke Mulvad Sandvik, Mette Frahm Olsen, Tacjana Pressler, Thomas Bryrup, Christian Leo-Hansen, Bibi Uhre Nielsen, Christine Højte, Marianne Skov, Frederik Buchvald, Inger Hee Mabuza Mathiesen, Tavs Qvist, Hanne Vebert Olesen, Sune Rubak, Camilla Bjørn Jensen, Daniel Faurholt-Jepsen, Kim Gjerum Nielsen","doi":"10.1002/ppul.71166","DOIUrl":"10.1002/ppul.71166","url":null,"abstract":"<p><strong>Introduction: </strong>Elexacaftor/tezacaftor/ivacaftor (ETI) is a breakthrough therapy for cystic fibrosis (CF). We aimed to assess ETI's real-world impact on peripheral airway disease assessed as ventilation distribution inhomogeneity using nitrogen multiple breath washout (N₂MBW) in children aged 6-17 years. Additionally, we compared the two outcomes, lung clearance index (LCI), and ventilation distribution efficiency (VDE), as VDE is considered to adjust for a theoretical overestimation of lung disease when using LCI.</p><p><strong>Methods: </strong>This nationwide study included data from N₂MBW performed during routine clinical care. Linear mixed effect regression was used to assess changes in LCI and VDE after 12 months of ETI treatment. Subgroup analyses included baseline age (6-11 vs. 12-1 years) and disease severity (normal-moderate vs. severe-very severe).</p><p><strong>Results: </strong>We included 131 children (78% homozygous for F508del mutation, mean [SD] age 11.5 [3.4]), and 339 N₂MBW tests. The median (range) number of tests per child was 3 (1-10). The estimated mean (95% CI) 12-months post-ETI improvement in LCI and VDE were 1.7 units (-2.1; -1.2, p < 0.001) and 2.1%-point (1.6; 2.6, p < 0.001), respectively. Similar LCI and VDE improvements were observed across age groups. Using VDE, fewer children were categorized with very severe lung disease, and the ETI-effect did not differ between the severity groups, unlike LCI.</p><p><strong>Conclusion: </strong>Our research demonstrates that ETI treatment significantly improves lung function, as measured by N₂MBW, in Danish children and adolescents with CF. VDE improvements were consistent across age and disease severity groups. In contrast, LCI revealed larger effect estimates for those with severe to very-severe lung impairment.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 6","pages":"e71166"},"PeriodicalIF":2.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12180290/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144333669","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":"An Ultra Protective Ventilation Bundle in an Infancy Severe pARDS Post Liver Transplantation.","authors":"Biyuan Xie, Long Xiang","doi":"10.1002/ppul.71172","DOIUrl":"https://doi.org/10.1002/ppul.71172","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 6","pages":"e71172"},"PeriodicalIF":2.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144317607","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":"Partial Resolution of Unilateral Tuberculous Lung Destruction: A Case Report of a 13-Years Old Patient.","authors":"Alessia Belli, Erica Ricci, Giuseppe Losurdo, Gilda Gizzi, Pietro Salvati, Francesca Rizzo, Oliviero Sacco, Elio Castagnola","doi":"10.1002/ppul.71176","DOIUrl":"https://doi.org/10.1002/ppul.71176","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 6","pages":"e71176"},"PeriodicalIF":2.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144326534","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":"Referral Rates and Diagnostic Evaluation for Primary Ciliary Dyskinesia in Patients With Laterality Defects Who Meet Primary Ciliary Dyskinesia Evaluation Criteria.","authors":"Alyssa K Salinas, Gerard Smits, Stephen B Spurgin, Tanya M Martínez-Fernandez, Yadira M Rivera-Sánchez","doi":"10.1002/ppul.71129","DOIUrl":"10.1002/ppul.71129","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Objective: &lt;/strong&gt;To determine if pediatric patients with laterality defects and meet the diagnostic criteria for primary ciliary dyskinesia (PCD) evaluation are being referred to and evaluated by pediatric pulmonologists. There are organ laterality defects in 50% of patients with PCD. Thus, patients with laterality defects are more likely to raise suspicion for PCD than those without laterality defects. We hypothesize that even in patients with laterality defects who meet PCD evaluation criteria, referral rates are low, leading to underdiagnosis (2).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Subjects and methods: &lt;/strong&gt;A retrospective chart review of patients with laterality defects who met two or more of the PCD evaluation criteria was conducted at Dallas Children's Health using the electronic medical record. The four PCD evaluation criteria were laterality defect, chronic daily cough, nasal congestion starting in infancy, and NRDS of unclear etiology requiring oxygen or positive pressure for 24 h or longer. Referral rates to pediatric pulmonary medicine and genetics were determined. Completion of an evaluation by these services was analyzed.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;The electronic medical record search for laterality defects identified 433 patients. Of the 369 patients who met the inclusion and exclusion criteria for analysis, 189 (51%) met only one inclusion criterion for PCD evaluation (laterality defect), and 49% met 2 or more of the criteria. Of the 79 patients who met the bare minimum of two criteria for PCD evaluation, 41% were referred to pulmonary, 18% to genetics, and only 16% were evaluated for PCD. Of the 27 patients who met all 4 PCD criteria for evaluation, 96% were referred to pulmonary, 85% to genetics, and 93% were evaluated for PCD. Among the 369 patients studied, there were no significant differences in the referral rate associated with patient demographics, including sex, race, ethnicity, and insurance type. The frequency of referrals increased with the number of PCD criteria.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;A substantial number of pediatric patients meeting two PCD referral criteria are not referred to pediatric pulmonologists, and a larger number are not being evaluated for PCD. As expected, patients with three or four PCD referral criteria have a higher rate of referral to pulmonary than those meeting the minimum two criteria. Many patients who meet all four criteria still need to be evaluated for PCD, even when referred to pulmonary or genetics. Most of the patients with laterality defects had congenital heart disease (CHD), and this was identified as the main etiology for respiratory symptoms for most of the patients who were not referred or evaluated. This highlights the importance of PCD education and awareness efforts for Pediatric specialists and subspecialists, including pediatric pulmonologists. It supports PCD being an underdiagnosed chronic condition and thus underscores the importance of acute clinical awareness","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 6","pages":"e71129"},"PeriodicalIF":2.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12139576/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144226182","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":"Flexible Bronchoscopy for Assessing and Managing Smoke Inhalational Injury in a Teenager.","authors":"Guiqing Wang, Xiaoping Jing, Haoxiang Gu","doi":"10.1002/ppul.71137","DOIUrl":"https://doi.org/10.1002/ppul.71137","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 6","pages":"e71137"},"PeriodicalIF":2.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144226178","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":"Prenatal Elexacaftor/Tezacaftor/Ivacaftor Treatment Prevents Meconium Ileus, but Not Exocrine Pancreatic Insufficiency and Lung Disease in Infants With Cystic Fibrosis.","authors":"Paulina E Aleksander, Stephanie Thee, Felix Doellinger, Styliani Zagkla, Marcus A Mall, Mirjam Stahl","doi":"10.1002/ppul.71168","DOIUrl":"https://doi.org/10.1002/ppul.71168","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 6","pages":"e71168"},"PeriodicalIF":2.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144317611","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}