Pediatric Pulmonology最新文献

{"title":"Effects of an Exercise Intervention on Exercise Capacity in Adults With Cystic Fibrosis: A Quasi-Experimental Study Comparing Individuals Treated With and Without Elexacaftor/Tezacaftor/Ivacaftor.","authors":"Wolfgang Gruber, Jana Koop, Franziska A Haegele, Christian Falkenberg, Strefan Dewey, Burkhard Weisser, Anja Bosy-Westphal","doi":"10.1002/ppul.71076","DOIUrl":"https://doi.org/10.1002/ppul.71076","url":null,"abstract":"<p><strong>Background: </strong>The effects of CFTR modulators, particularly elexacaftor/tezacaftor/ivacaftor (ETI), on exercise capacity in people with cystic fibrosis (pwCF) remain unclear, with no data available on their impact within the context of an exercise intervention. Therefore, this study aimed to assess the effects of an exercise intervention on exercise capacity in adults with CF, comparing those treated with and without ETI.</p><p><strong>Methods: </strong>A total of 56 adult pwCF participated in this quasi-experimental study as part of a rehabilitation program, which included a 3.5-week exercise intervention. The program involved five weekly 45-min sessions, including endurance training on a cycle ergometer. VO₂ _peak and W_peak were the primary outcomes used to assess changes in exercise capacity.</p><p><strong>Results: </strong>The intervention significantly increased VO₂ _peak and W_peak in all pwCF, regardless of ETI use, with similar improvements between groups. PwCF with lower baseline fitness (VO₂ _peak ≤ 81%pred) showed greater improvements than those with higher fitness (VO₂ _peak ≥ 82%pred). ppFEV1 remained unchanged, while BMI increased in both groups. Notably, the ETI group spent significantly more time in physical activity (PA) at hard and very hard intensities compared to the non-ETI group. Additionally, a positive correlation was observed between PA intensity and VO₂ _peak and W_peak in the ETI group.</p><p><strong>Conclusion: </strong>Independent of ETI treatment, adult pwCF improve their exercise capacity by participating in a regular exercise program. ETI treatment appears to enhance time spent in higher PA intensities. Despite the effectiveness of CFTR modulators, regular PA and exercise remain essential to maintain and improve exercise capacity in pwCF.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 4","pages":"e71076"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143754091","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":"Changes in Respiratory Patterns From Pressure Control Ventilation to Neurally Adjusted Ventilatory Assist Assessed by Electrical Impedance Tomography.","authors":"Marika Rahtu, Inéz Frerichs, Tobias H Becher, Tytti Pokka, Andreas D Waldmann, Thalia Papadouri, Anton H van Kaam, Peter C Rimensberger, Richard Bayford, Outi Peltoniemi, Merja Kallio","doi":"10.1002/ppul.71077","DOIUrl":"https://doi.org/10.1002/ppul.71077","url":null,"abstract":"<p><strong>Introduction: </strong>Neurally Adjusted Ventilatory Assist (NAVA) is increasingly used as ventilatory support for preterm infants. Changes in ventilation distribution and respiratory patterns during the transition from patient-triggered time-cycled Pressure Controlled Ventilation (PCV) to NAVA have not yet been studied. This study aimed to evaluate the impact of ventilation mode (PCV and NAVA, respectively) on ventilation distribution and breathing patterns in preterm infants with Electrical Impedance Tomography.</p><p><strong>Methods: </strong>This study included 16 premature infants at Oulu University Hospital who participated in the observational CRADL project and were on NAVA. EIT data was retrospectively assessed by choosing a 1-min stable recording before and after the change from one to the other mode. The primary endpoint was changes in global and regional ventilation parameters (ΔZ), and the secondary endpoints were Centers of Ventilation (CoVs), the amount of silent spaces, and the duration of inspiratory and respiratory cycle times.</p><p><strong>Results: </strong>A larger variation in the global tidal impedance variation (p < 0.05) and the respiratory cycle time (p < 0.05) was observed on NAVA than on PCV. Sighs, which were determined as a breath impedance change twice the size of an average breath on PCV, were more frequent during NAVA than PCV (5.1% vs 0.8%, respectively). Mean global or regional impedance variations or silent spaces did not differ between PCV and NAVA.</p><p><strong>Conclusion: </strong>NAVA allowed more variable breathing patterns during invasive respiratory support than patient-triggered PCV. However, variability in the respiratory cycle did not lead to systematic changes in ventilation distribution or silent spaces.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 4","pages":"e71077"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143764603","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":"Outbreak of Post-Infectious Bronchiolitis Obliterans (PIBO) After Adenovirus Infection: A Case Series and Review of the Literature.","authors":"A Traunero, S Ghirardo, M Aldeco, P Pascolo, S Basilicata, L Mazzari, M Maschio, A Amaddeo, U Krivec","doi":"10.1002/ppul.71080","DOIUrl":"10.1002/ppul.71080","url":null,"abstract":"<p><strong>Background: </strong>Post-infectious bronchiolitis obliterans (PIBO) is a rare chronic pediatric pulmonary disease characterized by irreversible fibrotic narrowing of the small airways. Treatment options remain uncertain with limited success.</p><p><strong>Objective: </strong>To delineate the characteristics of patients diagnosed with PIBO in Ljubljana (Slovenia) and Trieste (Italy) in 2023.</p><p><strong>Methods: </strong>We retrospectively assessed clinical records of PIBO patients from January to December 2023, capturing data on initial viral infection, clinical presentation, radiological features, treatments, and outcomes.</p><p><strong>Results: </strong>In 2023, 11 patients were identified, contrasting with only 6 cases in the previous 7 years. Common symptoms and signs included tachypnea, chronic wet cough, and diffuse crackles following adenovirus pneumonia. Most patients were previously healthy. Chest CT findings confirmed the diagnosis in all cases. Bronchoalveolar lavage showed elevated levels of neutrophils (46% to 90% of cells), and biopsies performed in 6 patients indicated predominantly lymphocytic inflammatory infiltrate and bronchiolar fibrosis. Nocturnal pulse oximetry revealed reduced mean SpO2 (median: 96.5% Q1: 93%, Q3: 98%) and reduced lower values (median: SpO2 89% Q1: 87%, Q3: 92.5%) with an increased oxygen desaturation index (1.1 to 11.2 events/hour). Treatment involved methylprednisolone (20-30 mg/kg) for three consecutive days monthly for 6 months, resulting in clinical improvement in nine patients and radiological improvement in seven patients.</p><p><strong>Conclusions: </strong>The post-pandemic surge in PIBO cases may stem from viral ecology changes, immunologic factors, and/or adenovirus genotypes, highlighting the need for further research into its etiology and management strategies.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 4","pages":"e71080"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11960595/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143754099","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":"Prominent Features and Treatment Outcomes of Childhood-Onset Eosinophilic Granulomatosis With Polyangiitis.","authors":"Xiaoyan Zhang, Hui Xu, Yuhong Guan, Yuelin Shen, Hui Liu, Xiang Zhang, Weihan Xu, Xiaohui Wen, Haiming Yang, Jinrong Liu, Shunying Zhao","doi":"10.1002/ppul.71063","DOIUrl":"https://doi.org/10.1002/ppul.71063","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 4","pages":"e71063"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143754102","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":"Respiratory Related Hospitalization in Children With Exposure to Meconium Aspiration or Staining.","authors":"Gabriel Côté-Corriveau, Ariane Lasry, Philippe Bégin, Marianne Bilodeau-Bertrand, Jessica Healy-Profitós, Nathalie Auger","doi":"10.1002/ppul.71085","DOIUrl":"10.1002/ppul.71085","url":null,"abstract":"<p><strong>Background: </strong>We investigated whether neonates exposed to meconium had a higher risk of respiratory hospitalization in childhood.</p><p><strong>Methods: </strong>We analyzed a longitudinal cohort of 1,271,563 children with 6,334,857 person-years of follow-up between birth and age 5 years in Quebec, Canada from 2006 to 2022. The exposure was meconium aspiration or meconium staining without aspiration at birth. The outcome was hospitalization for bronchiolitis, asthma, and other allergic conditions up to age 5 years. We estimated hazard ratios (HR) and 95% confidence intervals (CI) for the association between meconium exposure and these outcomes using Cox regression models adjusted for patient characteristics. We additionally examined associations according to gestational age.</p><p><strong>Results: </strong>A total of 6050 children had meconium aspiration (0.5%) and 131,238 were exposed to meconium without aspiration (10.3%). Compared with no exposure, meconium aspiration was associated with 1.24 times the risk of bronchiolitis (95% CI: 1.09-1.42), 1.43 times the risk of asthma (95% CI: 1.20-1.69), and 1.40 times the risk of other allergy hospitalization (95% CI: 1.11-1.76). Meconium aspiration was associated with these outcomes among children born at term (HR: 1.28, 95% CI: 1.14-1.44) and post-term (HR: 1.56, 95% CI: 1.26-1.94). There was no association for children born preterm. Meconium exposure without aspiration appeared to protect against the outcomes (HR: 0.92, 95% CI: 0.90-0.95), but the association disappeared when we accounted for gestational age.</p><p><strong>Conclusion: </strong>Meconium aspiration is associated with a higher risk of hospitalization for respiratory or atopic complications, but meconium exposure without aspiration is not associated with these outcomes.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 4","pages":"e71085"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143803957","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":"Challenges in the care of cystic fibrosis in low-to-middle income countries.","authors":"Sushil Kr Kabra","doi":"10.1002/ppul.27294","DOIUrl":"10.1002/ppul.27294","url":null,"abstract":"<p><p>Till few years ago cystic fibrosis (CF) was considered to be exclusively disease affecting Caucasian population. More recently CF has been reported from all over the world from different ethnic background. CF is an emerging illness in other parts of world (India with 1:2200 to 1:40,000 live birth). In low- and middle-income countries (LMICs) diagnosis of CF is missed due to ignorance and nonavailability of diagnostic tests. Diagnosis can be made using clinical features and basic lab tests. As of now there is no cure, but supportive care improved survival. However, medicines used in higher income countries are very expensive and not accessible to children with CF in LMICs. Cystic fibrosis transmembrane regulators modulators have emerged as significant advances in management. These are very expensive and beyond the reach of patients in LMICs. CF Fraternity in developed countries should work towards training of personnel and possibly making medications available in LMICs.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":" ","pages":"S48-S50"},"PeriodicalIF":2.7,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142366173","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":"Understanding primary ciliary dyskinesia.","authors":"Thomas Ferkol","doi":"10.1002/ppul.27360","DOIUrl":"10.1002/ppul.27360","url":null,"abstract":"<p><p>Primary ciliary dyskinesia (PCD) is a rare, inherited disease characterized by impaired motile ciliary function leading to chronic sinopulmonary disease, persistent middle ear effusions, laterality defects, and subfertility. Over fifty PCD-associated genes have also been identified, which have provided new insights into the processes involved into ciliary assembly, structure, and function. Historically, the diagnosis of PCD was based on the presence of ultrastructural defects in the ciliary axoneme but with identification of a growing number of disease-associated genes, genetic testing has become a first-line diagnostic tool. Other approaches have also evolved, that have improved our diagnostic capabilities. Treatments for PCD have lagged, and though our growing understanding of the genetic and pathophysiological bases of the disease of PCD may yield to better therapeutic strategies.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":" ","pages":"S86-S87"},"PeriodicalIF":2.7,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142505734","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":"Postinfectious bronchiolitis obliterans.","authors":"Alejandro Teper, Alejandro Colom","doi":"10.1002/ppul.27365","DOIUrl":"10.1002/ppul.27365","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":" ","pages":"S92-S93"},"PeriodicalIF":2.7,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142522560","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":"Etiology and clinical features of children with bronchiectasis-Insight from Chinese experience.","authors":"Peng Han, Kunling Shen","doi":"10.1002/ppul.27279","DOIUrl":"10.1002/ppul.27279","url":null,"abstract":"<p><p>Bronchiectasis is a clinical syndrome characterized by cough, sputum production, and the presence of abnormal thickening and dilation of the bronchial wall on imaging. There has been a remarkable resurgence in incidence and prevalence during the past 20 years. There was few data from children. In China, it has been estimated that 1.5% of women and 1.1% of men in the general population have physician-diagnosed bronchiectasis.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":" ","pages":"S37-S39"},"PeriodicalIF":2.7,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142854551","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":"Genetic Variants and Clinical Characteristics in the Diagnosis of Children Aged Under 18 Years With Cystic Fibrosis in a Brazilian State.","authors":"Luana da Silva Baptista Arpini, Fernanda Mayrink Gonçalves Liberato, Flavia Marini Paro, Sabrina da Silva Santos, Gina Torres Rego Monteiro","doi":"10.1002/ppul.71048","DOIUrl":"https://doi.org/10.1002/ppul.71048","url":null,"abstract":"<p><strong>Objective: </strong>To describe variants in the CFTR gene and demographic and clinical characteristics of individuals with Cystic Fibrosis (CF) from a Brazilian State pediatric reference center upon diagnosis.</p><p><strong>Methods: </strong>Cross-sectional retrospective cohort study of individuals with CF under 18 years old treated in a referral center between 2007 and 2023. Data was derived from medical records. A descriptive analysis of the variables at diagnosis, including pathogenic genetic variants in the CFTR gene, clinical findings, and demographic data.</p><p><strong>Results: </strong>The population of 110 patients was predominantly male (54.5%) and white (66.4%). Age at diagnosis ranged from 13 days to 17 years (median = 2 months), 78.2% were diagnosed at < 2 years, and 50.9% were diagnosed following neonatal screening. The most frequent clinical manifestations at diagnosis were steatorrhea (70.0%), persistent respiratory symptoms (58.2%), and malnutrition (36.4%). The most common CFTR variants were F508del (49.0%), G542X (7.3%), and 3120+1G>A (5.9%). Eighty-four (76.4%) were eligible to use at least 1 of the 4 CFTR modulator therapies available in Brazil, and 29 were eligible for 3 therapies because they are homozygous for F508del.</p><p><strong>Conclusion: </strong>Identification of CFTR variants at diagnosis can provide many benefits to patients, such as early interventions and CFTR modulator therapy, and is feasible in Brazil. Because each country may have different distributions of CFTR variants, it is essential to evaluate these distributions as we advance methodologies for gene variant detection, particularly in the contexts of newborn screening and diagnostic testing.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 3","pages":"e71048"},"PeriodicalIF":2.7,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143658053","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}