Pediatric Pulmonology最新文献

{"title":"Preliminary Psychometric Characteristics of the Cystic Fibrosis Coping Self-Efficacy (CF-CSE) Scale.","authors":"Deborah Friedman, Alexandra L Quittner, Jianghua He, Nivedita Chaudhary, Tara M D Mullen, Amanda S Bruce, Yelizaveta Sher, Beth A Smith, Anna M Georgiopoulos","doi":"10.1002/ppul.71164","DOIUrl":"10.1002/ppul.71164","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to examine the preliminary psychometric characteristics of the Cystic Fibrosis Coping Self-Efficacy Scale (CF-CSE), a new measure of coping self-efficacy assessing confidence in one's ability to cope with challenges of living with cystic fibrosis (CF).</p><p><strong>Methods: </strong>Measure development included cognitive testing with people with CF who participated in a pilot trial of CF-CBT, a CF-specific cognitive behavioral therapy skills-based intervention. Data were then collected from a baseline assessment in a multi-center randomized-controlled trial of CF-CBT for adults with mild symptoms of depression and/or anxiety (N = 60). Multitrait analysis was used to evaluate how individual CF-CSE items loaded on five hypothesized subscales, followed by assessment of subscale internal consistency and construct validity.</p><p><strong>Results: </strong>Multitrait analysis supported retention of four subscales (21 items total; 4-7 items each) reflecting coping skill domains: Acceptance-Based Strategies/Self-Compassion, Cognitive Coping, Active Stress Management, and Coping with Daily Self-Care. Each scale had excellent internal consistency (Cronbach ⍺ = 0.81 to 0.90). Medium to large effect-size (ES) correlations were found for all CF-CSE subscales with self-reported Perceived Stress Scale scores, and medium to large ES correlations for all subscales with Cystic Fibrosis Questionnaire-Revised (CFQ-R) Emotional Functioning. There were medium ES correlations between the Coping with Daily Self-Care subscale and CFQ-R Social and Role Functioning and Health Perceptions.</p><p><strong>Conclusion: </strong>CF-CSE is a new CF-specific measure of coping self-efficacy with preliminary evidence of reliability and construct validity. It offers a promising strengths-based patient-reported outcome measure identifying targets for clinical intervention and evaluating outcomes in CF mental health care.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 6","pages":"e71164"},"PeriodicalIF":2.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144476262","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":"Anxiety in the Pediatric Cystic Fibrosis Population: Evaluation of a Younger Cohort.","authors":"Jemilla Strode Smith, Josie van Dorst, Michael J Coffey, Chee Y Ooi","doi":"10.1002/ppul.71181","DOIUrl":"10.1002/ppul.71181","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 6","pages":"e71181"},"PeriodicalIF":2.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12202716/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144507498","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":"Impact of Home Oxygen Therapy for Bronchopulmonary Dysplasia on Families With Neonates: A Survey Conducted in Japan.","authors":"Masaki Hasebe, Ryo Ogawa, Takeshi Arimitsu, Fumihiko Namba, Tomohiko Nakamura","doi":"10.1002/ppul.71179","DOIUrl":"https://doi.org/10.1002/ppul.71179","url":null,"abstract":"<p><strong>Introduction: </strong>Bronchopulmonary dysplasia (BPD) often necessitates home oxygen therapy (HOT) in children. Although HOT facilitates early hospital discharge and provides security, its impact on families remains unclear. This study examined family experiences with HOT to identify challenges and strategies for enhanced implementation.</p><p><strong>Methods: </strong>A web-based survey was conducted with 113 families of children with BPD who used HOT within the past 3 years. Questions included the preparation for HOT, postdischarge challenges, and home monitoring devices.</p><p><strong>Results: </strong>HOT enabled early discharge; however, 42% families felt anxious upon leaving the hospital. Postdischarge life was harder than expected for 72% families, with mobility (82%) and equipment handling (82%) being major concerns. Coordination with home nursing services (55%) was helpful, whereas trial home stays (17%) and home visits (17%) were less commonly cited as beneficial. Home monitoring devices, used by 92% families, led to false alarms (75%). Despite such challenges, 99% considered it vital. A total of 26% adjusted oxygen flow independently, raising safety concerns.</p><p><strong>Discussion: </strong>The findings highlight the need for improved predischarge education, enhanced outpatient support, and technological advancements. Comprehensive preparation programs, improved home monitoring accuracy, and increased social awareness are essential to optimize HOT management and improve family quality of life.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 6","pages":"e71179"},"PeriodicalIF":2.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144507500","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":"Impact of Modulator Therapy on the Chronic Colonization of Lower Respiratory Tract Pathogens in Children: Data From Cystic Fibrosis Registry of Turkey.","authors":"Birce Sunman, Ebru Yalçın, Meltem Akgül Erdal, Velat Şen, Azer Kılıç Başkan, Ayşe Ayzıt Kılınç, Hakan Yazan, Erkan Çakır, Ayşegül Doğan Demir, Gökçen Ünal, Suat Savaş, Sevgi Pekcan, Merve Korkmaz, Yakup Canıtez, Gökçen Kartal Öztürk, Figen Gülen, Elif Arık, Özlem Keskin, Ali Ersoy, Mehmet Köse, Mahir Serbes, Derya Ufuk Altıntaş, Abdurrahman Erdem Başaran, Ayşen Bingöl, Zeynep İlkşen Hocoğlu, Ayşe Tana Aslan, Demet Polat Yuluğ, Ali Özdemir, Satı Özkan Tabakçı, Dilber Ademhan Tural, Koray Harmancı, Beste Özsezen, Nazan Çobanoğlu, Sedef Narin Tongal, Gönül Çaltepe, Melih Hangül, Zeynep Gökçe Gayretli Aydın, Mehmet Kılıç, Mina Hızal, Nilay Baş, Gizem Özcan, Tuğba Şişmanlar Eyüboğlu, Nagehan Emiralioğlu, Güzin Cinel, Uğur Özçelik, Deniz Doğru","doi":"10.1002/ppul.71155","DOIUrl":"10.1002/ppul.71155","url":null,"abstract":"<p><strong>Background: </strong>Modulators have revolutionized cystic fibrosis (CF) management, but their effects on respiratory pathogens remain unclear. This study evaluated changes in lower respiratory tract pathogen detection after modulator therapy in children with CF, registered in the Cystic Fibrosis Registry of Turkey.</p><p><strong>Method: </strong>This retrospective, multicenter cohort study included children receiving modulator therapy between 2020 and 2022. Chronic respiratory tract colonization rates before and after therapy were compared, along with inhaler treatments, oral steroid, azithromycin use, pulmonary function tests, and hospitalizations for pulmonary exacerbations. The cohort was stratified by age, modulator type, and lung disease severity. Changes in microbiologic data over a 1-year period were also analyzed for children not receiving modulator therapy.</p><p><strong>Results: </strong>A total of 101 children (mean age 9.95 ± 4.44 years) were included. Following modulator therapy, respiratory cultures of 57 (56.4%) were negative. Among 32 children with chronic Pseudomonas aeruginosa (P. aeruginosa) colonization, 14 (44%) showed negative respiratory cultures after receiving modulator therapy (p = 0.039). Conversion to culture-negative status was significant for methicillin-sensitive Staphylococcus aureus (MSSA) (p = 0.022) and methicillin-resistant Staphylococcus aureus (MRSA) (p = 0.034), with ETI therapy yielding the highest conversion rates. Inhaled antibiotic use for chronic respiratory pathogens decreased significantly (p = 0.039), and spirometry parameters improved (p < 0.001). Among 1232 children not receiving modulators, 180 (14.6%) had negative respiratory cultures when examined at 1-year interval. In the same group, intermittent/chronic P. aeruginosa colonization was negative in 58 cases, while 85 developed new growth, following modulator therapy, with positivity rates rising from 16.3% to 18.5% (p = 0.030). No significant changes in other pathogen detection were observed.</p><p><strong>Conclusion: </strong>Modulators, particularly ETI, reduced respiratory pathogen detection and improved lung function in children with CF.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 6","pages":"e71155"},"PeriodicalIF":2.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12161001/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144275582","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 Intestinal Permeability in Children Diagnosed With Bronchiolitis in Pediatric Emergency Department: A Cross-Sectional Study.","authors":"Abdullah Akkuş, Ahmet Osman Kiliç, Fatih Ercan, Mehtap Yücel, Abdullah Yazar, Fatih Akin, Sevgi Pekcan, Hasan Çetin, Hanife Tuğçe Çağlar","doi":"10.1002/ppul.71171","DOIUrl":"10.1002/ppul.71171","url":null,"abstract":"<p><strong>Background: </strong>Bronchiolitis is the most common lower respiratory tract infection, with the most commonly isolated organism being respiratory syncytial virus (RSV). Despite several studies on intestinal permeability in various acute and chronic pulmonary diseases, studies examining these molecules in patients diagnosed with bronchiolitis are very limited. This study was conducted to examine zonulin and claudin-3 levels to evaluate intestinal permeability in children diagnosed with bronchiolitis.</p><p><strong>Materials and methods: </strong>A total of 98 children, 60 patients with bronchiolitis and 38 healthy controls were included in this study. After informed consent of patients and their parents were obtained, 5 mL of blood serum samples were collected upon admission, after confirmation of the diagnosis, which were kept frozen until being analyzed for zonulin and claudin-3 levels and subsequently analyzed and compared between the groups.</p><p><strong>Results: </strong>The median value of zonulin was 18.57 ng/mL in the patient group and 12.41 ng/mL in the control group. There was no significant difference in zonulin levels between the patient and control groups (p = 0.540). The mean claudin-3 (CLDN3) level was significantly lower in the patient group (3889.56) than the control group 4216.77 (p < 0.001). In the patient group, zonulin and claudin levels did not significantly differ by the need of mechanical ventilation and ICU administration (p > 0.05).</p><p><strong>Conclusion: </strong>In conclusion, CLDN3 levels were found to be significantly lower in patients with bronchiolitis than in the control group. This finding may indicate that low CLDN3 levels may be involved in pathogenesis of bronchiolitis, by impairing airway permeability Further studies with larger number of participants, investigating the effect of airway permeability on the pathogenesis of bronchiolitis, which compare zonulin and CLDN3 levels according to disease severity, are warranted.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 6","pages":"e71171"},"PeriodicalIF":2.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12178109/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144326531","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":"Investigating the Interplay Between Having Cystic Fibrosis and Being a Member of the LGBTQIA+ Community: Protocol for the PRIDE CF Study.","authors":"Traci M Kazmerski, Kacie M Kidd, Raksha Jain, Ishaan Jathal, Olivia M Stransky, MinJae Lee, Rachel E Salyer, Vin Tangpricha, John Palla, Adrianne N Alpern, Mitchell R Lunn, Juno Obedin-Maliver, Jonathan Greenberg, Asher Prangley, Gregory S Sawicki","doi":"10.1002/ppul.71154","DOIUrl":"10.1002/ppul.71154","url":null,"abstract":"<p><p>The impact of the intersectional lived experience of having a chronic health condition and identifying as lesbian, gay, bisexual, transgender, queer, intersex, asexual or another sexual or gender minority (LGBTQIA+) on health and wellbeing is largely unknown. In this article, we describe the development and structure of PRIDE CF, an innovative, large-scale epidemiologic study using a mixed-methods team science approach to understand the experiences and health outcomes of people with cystic fibrosis (PwCF) who identify as a gender or sexual minority. Our four separate but symbiotic projects utilize the PRIDE CF cohort (n ~ 300) with the shared goal of better understanding the needs of LGBTQIA+ PwCF. We use a fully decentralized study design to recruit a national group of participants from a small population within a small population and strive to protect participants' confidentiality and privacy. Results will inform intervention development and testing to improve health for this population. This study is the first to comprehensively investigate the long-term impact and experiences of LGBTQIA+ identity on a person with any chronic condition and, thus, can serve as a model for future collaborations outside of cystic fibrosis.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 6","pages":"e71154"},"PeriodicalIF":2.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12178092/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144326533","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":"Acute Respiratory Failure in an Adolescent.","authors":"Alessia Morreale, Claudia Salemi, Maria Luisa Casciana, Manuela Putzu, Silvia Sordelli, Antonina Tirendi, Silvia Fasoli","doi":"10.1002/ppul.71183","DOIUrl":"https://doi.org/10.1002/ppul.71183","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 6","pages":"e71183"},"PeriodicalIF":2.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144476259","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":"Posttraumatic Tracheal Ballooning and Tracheoesophageal Window in a 16-Year-Old Girl.","authors":"Marco Piastra, Elisa Meacci, Jacopo Galli, Enzo Picconi, Tony Christian Morena, Vittoria Ferrari, Daniela Paioli, Giorgio Conti","doi":"10.1002/ppul.71170","DOIUrl":"https://doi.org/10.1002/ppul.71170","url":null,"abstract":"<p><strong>Background: </strong>Tracheoesophageal injury following blunt cervical trauma is a rarely described condition, especially in young patients.</p><p><strong>Methods: </strong>Imaging documentation and clinical management of a tracheoesophageal fistula (TEF) associated with significant enlargement of the tracheal lumen occurring in a 16-year-old girl after a road accident was reported.</p><p><strong>Results: </strong>Due to impaired neurological status, the girl was intubated and underwent a 2-week course of invasive mechanical ventilation. When weaning attempts were made, air leak and tracheal enlargement were noticed, consistent with a wide TEF. Surgical tracheostomy and feeding jejunostomy were performed to promote fistula healing and serial endoscopic evaluations were performed. After 3 months, surgical repair by a vascularized muscular flap allowed to establish esophageal and tracheal continuity, leading to a complete functional recovery.</p><p><strong>Conclusions: </strong>Posttraumatic TEF is a rare condition in adolescence and it requires a multi-disciplinary approach for diagnosis and treatment. In this case, a multi-step approach was adopted, eventually achieving a complete resolution.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 6","pages":"e71170"},"PeriodicalIF":2.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144485505","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":"Diagnosis and Management of Pulmonary Emboli Due to Atrial Masses in Two Children.","authors":"Mohammad Mahdavi, Hamidreza Pouraliakbar, Alireza Yaghoubi, Golnar M Hejri","doi":"10.1002/ppul.71152","DOIUrl":"https://doi.org/10.1002/ppul.71152","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 6","pages":"e71152"},"PeriodicalIF":2.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144226177","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 Inverted Steeple Sign, Tracheal Tug, and Biphasic Stridor: A Triad for Diagnosing Congenital Tracheal Stenosis.","authors":"Nikhil Rajvanshi, Aswanth Ks, T V Vijay Kumar","doi":"10.1002/ppul.71151","DOIUrl":"https://doi.org/10.1002/ppul.71151","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 6","pages":"e71151"},"PeriodicalIF":2.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144275584","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}