Pediatric Pulmonology最新文献

{"title":"Therapies Used by Children With Primary Ciliary Dyskinesia: A Natural History Study.","authors":"Robert A Gardner, Thomas W Ferkol, Stephanie D Davis, Margaret Rosenfeld, Scott D Sagel, Sharon D Dell, Carlos E Milla, Lang Li, Feng-Chang Lin, Kelli M Sullivan, Maimoona A Zariwala, Michael R Knowles, Margaret W Leigh","doi":"10.1002/ppul.27412","DOIUrl":"10.1002/ppul.27412","url":null,"abstract":"<p><strong>Introduction: </strong>Primary ciliary dyskinesia (PCD) management has not been systematically evaluated and is largely empirical.</p><p><strong>Methods: </strong>Pediatric participants with PCD were enrolled in a prospective, longitudinal, multicenter, observational study. Therapies were recorded at annual visits and categorized by type. Age-related trends in prevalence of therapies were described by serial cross-sectional analyses. Generalized estimating equations analyzed covariates affecting prevalence of certain therapies and whether these covariates impacted oral antibiotic courses.</p><p><strong>Results: </strong>A total of 137 participants completed 897 visits over 13 years. All but one received ≥ 1 antibiotic courses during study participation, most often cephalosporins (74%) or amoxicillin-clavulanate (73%). Thirty-one percent reported chronic azithromycin use. Per participant, there was an average of 2.3 (SD = 2.2) oral antibiotic courses annually. The rate of reported antibiotic courses at the 6 United States sites was 2.6 times higher compared to the Canadian site (p < 0.001). As patients got older, they were more likely to report use of amoxicillin-clavulanate (p < 0.001), chronic azithromycin (p < 0.001), fluroquinolones (p < 0.001), inhaled steroids with long-acting beta-agonists (p = 0.010), and hypertonic saline (p < 0.001). Compared to outer dynein arm defects, those with inner dynein arm/microtubular disorganization defects reported increased use of chronic azithromycin (p = 0.011) and inhaled steroids (p = 0.015).</p><p><strong>Discussion: </strong>Older participants and those with inner dynein arm/microtubular disorganization defects reported more therapies likely due to disease progression and more severe phenotypes, respectively. We report that a wide range of therapies are used in PCD without disease-specific studies defining benefits and risks.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":" ","pages":"e27412"},"PeriodicalIF":2.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11750599/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142687982","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":"Labor Status at Delivery and Lung Function in Extremely Prematurely Born Young Adults.","authors":"Sean Armstrong, Christopher Harris, Mohadeseh Kazemi, Alan Lunt, Janet Peacock, Anne Greenough","doi":"10.1002/ppul.27440","DOIUrl":"10.1002/ppul.27440","url":null,"abstract":"<p><strong>Background: </strong>There has been conflicting evidence regarding the impact of mode of delivery on respiratory outcomes in later childhood and adulthood. It is possible labor status, rather than mode of delivery, influences later respiratory morbidity. We hypothesized that extremely premature infants born to mothers in labor would have better lung function at follow-up than those born to mothers not in labor.</p><p><strong>Methods: </strong>We reviewed data from the United Kingdom High-Frequency Oscillation Study. Lung function testing was performed on young people aged 16-18 years born before 29 weeks of gestation. Linear mixed models were used to adjust lung function for maternal and neonatal factors and for the clustering due to multiple births.</p><p><strong>Results: </strong>One hundred and fifty subjects underwent lung function testing. Young adults born to mothers in labor had better mean Forced Expiratory Flow₇₅ (FEF₇₅) compared to those born to mothers not in labor (adjusted difference 0.50 [95% CI: 0.02, 0.99]). Similar significant differences were noted in FEF₅₀ (0.45 [-0.05, 0.85]), and FEF_25-75 (0.53 [0.05, 1.01]).</p><p><strong>Conclusion: </strong>Our study demonstrates that amongst individuals born very prematurely, those whose mothers were in labor before delivery had better small airway function at 16-19 years of age.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":" ","pages":"e27440"},"PeriodicalIF":2.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11748109/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142829662","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":"Long Term Ventilation in Pediatric Central Apnea: Etiologies and Therapeutic Approach over a Decade.","authors":"Santiago Presti, Martino Pavone, Elisabetta Verrillo, Maria Giovanna Paglietti, Anna Del Colle, Salvatore Leonardi, Renato Cutrera","doi":"10.1002/ppul.27400","DOIUrl":"10.1002/ppul.27400","url":null,"abstract":"<p><strong>Objective: </strong>This retrospective study aimed to analyze the clinical characteristics, ventilatory strategies, and effectiveness of ventilation in pediatric patients with central apneas treated at the Sleep Medicine and Long-Term Ventilation Unit of the Bambino Gesù Children's Hospital in Rome from 2012 to 2022.</p><p><strong>Methods: </strong>Among all ventilated patients at our Center from January 2012 to December 2022, we retrospectively included children with a cAHI ≥ 1 events/h on baseline poly(somno)graphic study. Additional parameters assessed included the underlying disease, type of ventilation (non-invasive vs. invasive), age at ventilation onset, ventilation mode, and transcutaneous capnometry parameters. To assess the effectiveness of ventilation on central apneas, we compared the cAHI at baseline and on ventilation.</p><p><strong>Results: </strong>Sixty-seven patients met the inclusion criteria for central apnea (cAHI > 1 events/h). Diagnoses included hypoxic-ischemic encephalopathy, 15 (22.4%); Ondine syndrome, 14 (20.9%); polymalformative syndrome, 10 (14.9%); Prader-Willi syndrome, 8 (11.9%); brain tumor, 6 (9.0%); Down syndrome, 4 (6.0%); ROHHAD syndrome, 2 (3.0%); other infrequent pathologies were, Arnold-Chiari II, primary central apnea, epilepsy, lisosomal diseases, hydrocephalus, myopathy, obesity, Rett Syndrome. Pressure-supported ventilation (PSV) was the most common mode used (45 out 67 patients, 67.2%), followed by pressure-controlled ventilation (PCV) (15 out 67 patients, 22.4%) and continuous positive airway pressure (CPAP) (7 out 67 patients, 10.4%). Statistically significant improvement (p < 0.05) in cAHI was observed in patients with polymalformative syndrome (3.5 vs. 0.3, p = 0.01), hypoxic-ischemic encephalopathy (3.1 vs. 0.1, p = < 0.01), and Prader-Willi syndrome (3.5 vs. 0.1, p = 0.03), while there was no significant improvementn in children with brain tumor (6.2 vs. 1.5, p = 0.21).</p><p><strong>Conclusion: </strong>Central apneas are present in children with various underlying pathologies. Ventilatory strategies tailored to the specific diagnosis and severity of central apneas yield significant improvements in cAHI. PSV was the preferred ventilation mode in this study and there was notable effectiveness across different diagnostic categories. PCV was employed in most severe cases. CPAP was exclusively used in patients with predominantly obstructive sleep apneas.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":" ","pages":"e27400"},"PeriodicalIF":2.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11758771/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142646652","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":"Effects of Elexacaftor-Tezacaftor-Ivacaftor on Nasal and Sinus Symptoms in Children With Cystic Fibrosis.","authors":"Guillaume Petit, Aurélie Coudert, Ruben Hermann, Eric Truy, Maxime Bonjour, Philippe Reix, Sonia Ayari","doi":"10.1002/ppul.27493","DOIUrl":"10.1002/ppul.27493","url":null,"abstract":"<p><strong>Background: </strong>New CFTR Modulator triple therapy Elexacaftor-Ivacaftor-Tezacaftor (ETI) prove efficacy in pulmonary outcomes. However, its impact on nasal sinus symptoms in children has not been specifically studied. The aim of this study is to evaluate the impact of this therapy on nasal sinus symptomatology in children aged 6-12 years.</p><p><strong>Methods: </strong>A prospective, single-center cohort study was conducted over a 12-month follow-up period in children aged 6-12 years at the initiation of ETI therapy. The primary outcome was evolution of the SN-5 score, a validated pediatric questionnaire measuring quality of life related to nasal sinus symptoms. A decrease of 0.5 points is considered clinically significant. Secondary outcomes included changes in clinical examination findings (obstructive turbinate hypertrophy, polyps, presence of pus in the middle meatus, and externalized mucocele), quality of life measured by the Visual Analog Scale (VAS), and number of antibiotic courses during the study period.</p><p><strong>Results: </strong>Twenty-six patients were included between March and September 2023, with no lost to follow-up. The initial mean SN-5 score was 2.88 (95% CI {1.91; 3.85}). After 1 year, the mean SN-5 score was significantly lower (1.41, 95% CI {1.00; 1.88}, Delta = 1.47, p < 0.001). The VAS related to symptoms also improved (Delta = 1.7, p < 0.001), and the number of antibiotic courses decreased (25 vs. 69, p < 0.001). A trend toward improvement in clinical examination parameters was observed.</p><p><strong>Conclusion: </strong>ETI therapy appears to significantly improve nasal sinus symptoms in children aged 6-12 years, as evidenced by improved quality-of-life scales and reduced antibiotic use.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 1","pages":"e27493"},"PeriodicalIF":2.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11771559/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143059480","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":"Spirometry Versus Forced Oscillation to Assess Lung Function Outcome at 5 Years of Age.","authors":"Robert S Tepper, Kristin Milner, Julia Harris, Brianna Lee, Michelle Cunningham, Christina Tiller, Lyndsey E Shorey-Kendrick, Diane Schilling, Julie Brownsberger, Kelvin MacDonald, Annette Vu, Byung S Park, Eliot R Spindel, Cynthia D Morris, Cindy T McEvoy","doi":"10.1002/ppul.27415","DOIUrl":"10.1002/ppul.27415","url":null,"abstract":"<p><strong>Background: </strong>Spirometry is the gold standard for assessing airway function for clinical studies; however, obtaining high-quality data in young children remains challenging. Since the forced oscillation technique (FOT) requires less subject cooperations, there has been increasing interest in FOT, particularly in young children. We evaluated whether spirometry and FOT in young children provides comparable ability to detect a treatment effect.</p><p><strong>Methods: </strong>We recently reported in a randomized controlled trial that vitamin C compared to placebo treatment of mothers who smoked during pregnancy (MSDP) results in the offspring having significantly higher forced expiratory flows (FEFs) at 5-years of age, as well as significantly less wheeze at 4-6 years of age. In these same offspring, we also measured respiratory impedance using FOT at 8-Hz impedance at 3, 4, and 5 years of age.</p><p><strong>Results: </strong>Although spirometry demonstrated significantly increased FEFs in vitamin C compared to placebo-treatment group at 5 years of age (p < 0.001), we were not able to detect a similar treatment effect using FOT impedance.</p><p><strong>Conclusions: </strong>It may be challenging to obtain technically successful spirometry in preschool children; however, FEFs may provide a better outcome than single-frequency FOT impedance to assess improvements in airway function in these young subjects.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":" ","pages":"e27415"},"PeriodicalIF":2.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11758767/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142676665","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":"Outpatient clinical care for bronchopulmonary dysplasia: A survey of the BPD collaborative.","authors":"Paul E Moore, Lystra P Hayden, Natalie M Villafranco, Demet Toprak, Jessica L Rice, Lawrence M Rhein, Antonia P Popova, Robin L McKinney, Winston M Manimtim, Jonathan C Levin, Khanh V Lai, Susan C Gage, Manvi Bansal, Christopher D Baker, Eric D Austin, Amit Agarwal, Sharon A McGrath-Morrow, Joseph M Collaco","doi":"10.1002/ppul.27296","DOIUrl":"10.1002/ppul.27296","url":null,"abstract":"<p><strong>Background: </strong>Bronchopulmonary dysplasia, a sequela of preterm birth, is the most common chronic respiratory disorder in infancy, and the second most common in children. Despite this, clinical care remains highly variable with guidelines supported by limited evidence, and do not provide specific guidance for timing of clinical follow-up, echocardiography, modalities of pulmonary function testing, etc. OBJECTIVE/METHODS: To further our understanding of care delivery for BPD, we sought to describe outpatient care patterns at tertiary care centers through survey data from 27 well-established BPD programs.</p><p><strong>Results: </strong>We observed variability in referral patterns to outpatient BPD clinics, ancillary services provided, indications for follow-up echocardiograms, availability of lung function testing, and criteria for discharge from care.</p><p><strong>Conclusion: </strong>More comprehensive and detailed clinical guidelines similar to other pulmonary diseases such as asthma and cystic fibrosis should be developed to help standardize care and may improve long term outcomes.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":" ","pages":"e27296"},"PeriodicalIF":2.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142400955","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":"Disseminated Tuberculosis Involving Lungs, Bronchus, Abdominal Cavity, Intracalvarium, and Spinal Column.","authors":"Hao Wang, Bao-Ping Xu, Kun-Ling Shen","doi":"10.1002/ppul.27427","DOIUrl":"10.1002/ppul.27427","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":" ","pages":"e27427"},"PeriodicalIF":2.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142751307","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":"Persistent Airflow Limitation Prediction and Risk Factor Analysis Among Asthmatic Children: A Retrospective Cohort Study.","authors":"Shiqiu Xiong, Chunyu Tian, Mingjun Shao, Chuanhe Liu","doi":"10.1002/ppul.27381","DOIUrl":"10.1002/ppul.27381","url":null,"abstract":"<p><strong>Introduction: </strong>A minority of asthmatic children develop persistent airflow limitation (PAL), associated with an increased risk of chronic airflow obstruction and poor prognosis. This study aimed to identify risk factors for PAL and develop a prediction model to identify high-risk asthmatic children.</p><p><strong>Methods: </strong>This retrospective study included 2072 children (5-16 years) with asthma. After a 2-year follow-up, patients were categorized into non-PAL, reversible PAL (RPAL), and irreversible PAL (IPAL) groups. Logistic regression (LR) was used to identify independent risk factors for RPAL and IPAL. A prediction model based on multivariate LR was developed and validated to identify asthmatic children at high risk of developing PAL. A nomogram was created for visualization.</p><p><strong>Results: </strong>Among the 2072 asthmatic patients, 14.72% (n = 305) developed PAL. Asthma exacerbation history (OR 1.80, 95% CI 1.03-3.01) and poor adherence (OR 1.83, 95% CI 1.26-2.65) were independent risk factors of RPAL. Independent risk factors for IPAL were BMI over 19.0 kg/m² (OR: 1.81, 95% CI: 1.03-3.21) and a history of pneumonia (OR: 2.40, 95% CI: 1.30-4.26). The prediction model incorporated nine variables and showed good discriminatory ability, with AUC values of 0.79 (95% CI: 0.76-0.81) for the training set, 0.76 (95% CI: 0.76-0.77) for internal validation, and 0.73 (95% CI: 0.64-0.81) for temporal validation.</p><p><strong>Conclusion: </strong>Asthma exacerbation history and poor adherence were independent risk factors for developing RPAL. BMI over 19.0 kg/m² and a history of pneumonia were risk factors for IPAL. Our prediction model effectively identified asthmatic children at high risk of developing PAL.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":" ","pages":"e27381"},"PeriodicalIF":2.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142583978","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":"Diabetes remission in adults with cystic fibrosis commenced on Elexacaftor/Tezacaftor/Ivacaftor: A single center case-series.","authors":"Shanal Kumar, Angela G Matson","doi":"10.1002/ppul.27348","DOIUrl":"10.1002/ppul.27348","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":" ","pages":"e27348"},"PeriodicalIF":2.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142472187","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":"The Risk of Pneumothorax With Intrapleural Urokinase in Children With Parapneumonic Effusion.","authors":"Gili Kadmon, Adi Schoen, Elhanan Nahum, Avichai Weissbach, Eytan Kaplan, Tal Cohen, Gabriel Chodick, Oded Scheuerman","doi":"10.1002/ppul.27443","DOIUrl":"10.1002/ppul.27443","url":null,"abstract":"<p><strong>Aim: </strong>Fibrinolytic therapy is commonly used in children with parapneumonic effusion, to facilitate drainage of the effusions and recovery. However, data regarding complications of this treatment in children are limited. We aimed to determine the incidence of pneumothorax (PNX) associated with intrapleural urokinase.</p><p><strong>Methods: </strong>We analyzed retrospectively collected data of children with parapneumonic effusion who underwent chest drain insertion. The clinical course and complications, including the incidence of PNX, were compared between children who were and were not treated with urokinase.</p><p><strong>Results: </strong>The study group included 120 children, of whom 57 were treated with urokinase. Children who were and were not treated with urokinase did not differ in markers of disease severity or in the length of hospitalization. Among the patients treated with urokinase compared to those not treated, the incidence of PNX was higher (35% vs. 6%, p < 0.001) and the median duration of chest drain treatment was longer (6 vs. 4 days, p < 0.001).</p><p><strong>Conclusion: </strong>In our pediatric cohort, intrapleural urokinase was associated with a higher incidence of PNX and did not shorten the duration of hospitalization.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":" ","pages":"e27443"},"PeriodicalIF":2.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142818838","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}