Objective: To compare the efficacy and safety of flow-driver-generated synchronized nasal intermittent positive-pressure ventilation (SNIPPV) and biphasic positive airway pressure (BiPAP) after extubation in preterm infants.
Hypothesis: SNIPPV delivered via a flow-driver device improves post-extubation outcomes compared with non-synchronized BiPAP.
Study design: This single-center, retrospective, observational cohort study was conducted in the neonatal intensive care unit of Nagoya University Hospital, Nagoya, Japan.
Patient selection: Preterm infants born at < 34 weeks of gestation who underwent endotracheal ventilation after birth and were subsequently supported with either BiPAP (from October 2017 to March 2020) or SNIPPV (from April 2020 to September 2022) after extubation. Infants with major anomalies or surgical ventilator dependence were excluded.
Methodology: Data on demographic and clinical characteristics, respiratory outcomes, and complications were collected. The primary outcome was reintubation within 72 h. Secondary outcomes were bronchopulmonary dysplasia (BPD), respiratory support duration, and major complications.
Results: Sixty-seven infants were included (BiPAP n = 37, SNIPPV n = 30). Reintubation within 72 h occurred in 6.7% and 29.7% of infants in the SNIPPV and BiPAP groups, respectively (p = 0.028). The incidence of respiratory distress-related failure was significantly lower in the SNIPPV group (3.3% vs. 24.3%, p = 0.019). There were no significant differences in BPD at 36 or 40 weeks, oxygen therapy duration, or complications such as necrotizing enterocolitis, retinopathy of prematurity, or severe neurological injury.
Conclusion: Flow-driver-generated SNIPPV significantly reduces early extubation failure in preterm infants compared to BiPAP without increasing the rate of adverse outcomes. This is a practical and effective alternative to noninvasive respiratory support.
目的:比较流驱动同步鼻腔间歇正压通气(SNIPPV)和双相气道正压通气(BiPAP)在早产儿拔管后的疗效和安全性。假设:与非同步BiPAP相比,通过流量驱动装置输送的SNIPPV可改善拔管后的结果。研究设计:这项单中心、回顾性、观察性队列研究在日本名古屋名古屋大学医院新生儿重症监护室进行。患者选择:方法:收集人口统计学和临床特征、呼吸结局和并发症的数据。主要结局是72小时内重新插管。次要结局是支气管肺发育不良(BPD)、呼吸支持持续时间和主要并发症。结果:纳入67例婴儿(BiPAP n = 37, SNIPPV n = 30)。SNIPPV组和BiPAP组患儿72 h内再插管率分别为6.7%和29.7% (p = 0.028)。SNIPPV组呼吸窘迫相关衰竭发生率显著降低(3.3% vs. 24.3%, p = 0.019)。36周或40周的BPD、氧疗时间、坏死性小肠结肠炎、早产儿视网膜病变或严重神经损伤等并发症无显著差异。结论:与BiPAP相比,由血流驱动产生的SNIPPV可显著减少早产儿早期拔管失败,且不增加不良结局的发生率。这是一种实用和有效的替代无创呼吸支持。
{"title":"Flow-Driver-Generated Synchronized Nasal Intermittent Positive-Pressure Ventilation Versus Biphasic Positive Airway Pressure After Extubation in Preterm Infants.","authors":"Toshihiko Suzuki, Kazuto Ueda, Akinobu Taniguchi, Takashi Maeda, Ryuichi Tanaka, Ryosuke Miura, Yukako Muramatsu, Yoshiaki Sato","doi":"10.1002/ppul.71410","DOIUrl":"10.1002/ppul.71410","url":null,"abstract":"<p><strong>Objective: </strong>To compare the efficacy and safety of flow-driver-generated synchronized nasal intermittent positive-pressure ventilation (SNIPPV) and biphasic positive airway pressure (BiPAP) after extubation in preterm infants.</p><p><strong>Hypothesis: </strong>SNIPPV delivered via a flow-driver device improves post-extubation outcomes compared with non-synchronized BiPAP.</p><p><strong>Study design: </strong>This single-center, retrospective, observational cohort study was conducted in the neonatal intensive care unit of Nagoya University Hospital, Nagoya, Japan.</p><p><strong>Patient selection: </strong>Preterm infants born at < 34 weeks of gestation who underwent endotracheal ventilation after birth and were subsequently supported with either BiPAP (from October 2017 to March 2020) or SNIPPV (from April 2020 to September 2022) after extubation. Infants with major anomalies or surgical ventilator dependence were excluded.</p><p><strong>Methodology: </strong>Data on demographic and clinical characteristics, respiratory outcomes, and complications were collected. The primary outcome was reintubation within 72 h. Secondary outcomes were bronchopulmonary dysplasia (BPD), respiratory support duration, and major complications.</p><p><strong>Results: </strong>Sixty-seven infants were included (BiPAP n = 37, SNIPPV n = 30). Reintubation within 72 h occurred in 6.7% and 29.7% of infants in the SNIPPV and BiPAP groups, respectively (p = 0.028). The incidence of respiratory distress-related failure was significantly lower in the SNIPPV group (3.3% vs. 24.3%, p = 0.019). There were no significant differences in BPD at 36 or 40 weeks, oxygen therapy duration, or complications such as necrotizing enterocolitis, retinopathy of prematurity, or severe neurological injury.</p><p><strong>Conclusion: </strong>Flow-driver-generated SNIPPV significantly reduces early extubation failure in preterm infants compared to BiPAP without increasing the rate of adverse outcomes. This is a practical and effective alternative to noninvasive respiratory support.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 12","pages":"e71410"},"PeriodicalIF":2.3,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12666728/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145649025","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}
Introduction: Leucine-rich α-2 glycoprotein (LRG) is an acute-phase reactant protein which reflects inflammation differently from C-reactive protein. We investigated the value of cord blood LRG levels for predicting bronchopulmonary dysplasia (BPD) in preterm infants.
Methods: This prospective cohort study included 64 infants born at 22-31 weeks' gestation (22 and 42 in the BPD and non-BPD arms, respectively), and between-group comparisons of LRG and interleukin (IL)-6 levels in the cord blood, neonatal LRG, and immunoglobulin (Ig)M were undertaken. Accounting for gestational age, inverse probability weighted generalized estimating equation assessed the biomarkers' independent effects on BPD.
Results: In the BPD group, the incidences of histological chorioamnionitis, funisitis, neonatal steroid treatment, and ventilator duration were significantly higher (p = 0.023, 0.043, 0.011, and < 0.001, respectively) whereas the Apgar score 1st minute, 5th minute, birthweight, and gestational age were significantly lower (p = 0.001, 0.011, < 0.001, < 0.001, respectively). After adjustment, umbilical artery (UA)-LRG (odds ratio [OR]: 2.678, 95% confidence interval [CI]: 1.225-0.5.857; p = 0.014) and umbilical vein (UV)-IL-6 (OR: 1.258, 95% CI: 1.020-1.552; p = 0.032) remained independent risk factors for BPD, whereas UV-LRG, UA-IL-6, IgM, and neonatal-LRG showed no significant associations.
Conclusions: UA-LRG may serve as a biomarker for predicting BPD and identify infants who are at a higher risk of BPD and could benefit more from corticosteroids or other therapies. UA-LRG's superior predictive accuracy over UV-LRG is attributable to the fact that cytokines in the amniotic fluid might cause fetal inflammation in utero before the development of histologically chorioamnionitis.
{"title":"Umbilical Artery Leucine-Rich α-2 Glycoprotein as a Predictor of Bronchopulmonary Dysplasia in Preterm Infants.","authors":"Genichiro Sotodate, Satoshi Serada, Fumiaki Takahashi, Atsushi Matsumoto, Yukiko Toya, Shigekuni Tsuchiya, Minoru Fujimoto, Tetsuji Naka, Manami Akasaka","doi":"10.1002/ppul.71425","DOIUrl":"10.1002/ppul.71425","url":null,"abstract":"<p><strong>Introduction: </strong>Leucine-rich α-2 glycoprotein (LRG) is an acute-phase reactant protein which reflects inflammation differently from C-reactive protein. We investigated the value of cord blood LRG levels for predicting bronchopulmonary dysplasia (BPD) in preterm infants.</p><p><strong>Methods: </strong>This prospective cohort study included 64 infants born at 22-31 weeks' gestation (22 and 42 in the BPD and non-BPD arms, respectively), and between-group comparisons of LRG and interleukin (IL)-6 levels in the cord blood, neonatal LRG, and immunoglobulin (Ig)M were undertaken. Accounting for gestational age, inverse probability weighted generalized estimating equation assessed the biomarkers' independent effects on BPD.</p><p><strong>Results: </strong>In the BPD group, the incidences of histological chorioamnionitis, funisitis, neonatal steroid treatment, and ventilator duration were significantly higher (p = 0.023, 0.043, 0.011, and < 0.001, respectively) whereas the Apgar score 1st minute, 5th minute, birthweight, and gestational age were significantly lower (p = 0.001, 0.011, < 0.001, < 0.001, respectively). After adjustment, umbilical artery (UA)-LRG (odds ratio [OR]: 2.678, 95% confidence interval [CI]: 1.225-0.5.857; p = 0.014) and umbilical vein (UV)-IL-6 (OR: 1.258, 95% CI: 1.020-1.552; p = 0.032) remained independent risk factors for BPD, whereas UV-LRG, UA-IL-6, IgM, and neonatal-LRG showed no significant associations.</p><p><strong>Conclusions: </strong>UA-LRG may serve as a biomarker for predicting BPD and identify infants who are at a higher risk of BPD and could benefit more from corticosteroids or other therapies. UA-LRG's superior predictive accuracy over UV-LRG is attributable to the fact that cytokines in the amniotic fluid might cause fetal inflammation in utero before the development of histologically chorioamnionitis.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 12","pages":"e71425"},"PeriodicalIF":2.3,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145715319","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}
Anna Šmejkalová, Václav Koucký, Josef Sýkora, Hana Hrdinová, Jana Bartošová, Jan Baxa, Marcela Kreslová
{"title":"Pica-Induced Airway Obstruction in Paediatric Cystic Fibrosis: An Unexpected Culprit.","authors":"Anna Šmejkalová, Václav Koucký, Josef Sýkora, Hana Hrdinová, Jana Bartošová, Jan Baxa, Marcela Kreslová","doi":"10.1002/ppul.71420","DOIUrl":"10.1002/ppul.71420","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 12","pages":"e71420"},"PeriodicalIF":2.3,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145654530","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}
Robin L McKinney, Antonia P Popova, Milenka Cuevas Guaman, Matthew J Kielt, Paul E Moore, Sharon A McGrath-Morrow, Joseph M Collaco
Objectives: Bronchopulmonary dysplasia (BPD) is the most common complication of preterm birth, which can have life-long effects. Commonly used definitions of BPD severity are based on respiratory support at 36 weeks post-menstrual age. The utility of these definitions for tertiary referral centers outside the initial birth hospitalization is unclear.
Study design: Retrospective cohort study of infants born < 34 weeks gestation in the BPD Collaborative registry using three common definitions of BPD severity at 36 weeks post menstrual age and associated their severity with outpatient respiratory outcomes during the first 3 years of life.
Results: BPD severity by all three definitions was applied to 608 patients. Although the definitions had different distributions of BPD severity for the cohort, a majority of participants (75.7%) had moderate/Grade 2 or severe/Grade 3 BPD irrespective of the definition. Using clustered logistic regression models, we found no association between any of the definitions of BPD and outpatient acute care use or the BPD control score.
Conclusions: In this multi-center cohort study, we did not observe a significant association between BPD definitions and respiratory morbidity following the initial birth hospitalization. Alternative risk-based BPD definitions that can be integrated into the care of preterm infants following discharge need to be developed.
{"title":"Common Severity-Based Definitions of Bronchopulmonary Dysplasia Are not Associated With Outpatient Morbidities.","authors":"Robin L McKinney, Antonia P Popova, Milenka Cuevas Guaman, Matthew J Kielt, Paul E Moore, Sharon A McGrath-Morrow, Joseph M Collaco","doi":"10.1002/ppul.71415","DOIUrl":"10.1002/ppul.71415","url":null,"abstract":"<p><strong>Objectives: </strong>Bronchopulmonary dysplasia (BPD) is the most common complication of preterm birth, which can have life-long effects. Commonly used definitions of BPD severity are based on respiratory support at 36 weeks post-menstrual age. The utility of these definitions for tertiary referral centers outside the initial birth hospitalization is unclear.</p><p><strong>Study design: </strong>Retrospective cohort study of infants born < 34 weeks gestation in the BPD Collaborative registry using three common definitions of BPD severity at 36 weeks post menstrual age and associated their severity with outpatient respiratory outcomes during the first 3 years of life.</p><p><strong>Results: </strong>BPD severity by all three definitions was applied to 608 patients. Although the definitions had different distributions of BPD severity for the cohort, a majority of participants (75.7%) had moderate/Grade 2 or severe/Grade 3 BPD irrespective of the definition. Using clustered logistic regression models, we found no association between any of the definitions of BPD and outpatient acute care use or the BPD control score.</p><p><strong>Conclusions: </strong>In this multi-center cohort study, we did not observe a significant association between BPD definitions and respiratory morbidity following the initial birth hospitalization. Alternative risk-based BPD definitions that can be integrated into the care of preterm infants following discharge need to be developed.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 12","pages":"e71415"},"PeriodicalIF":2.3,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145655198","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}
Lieke C E Noij, Caroline L H Brackel, Mariëlle W Pijnenburg, Michiel A G E Bannier, Sanne F Kloosterman, Joost G van den Aardweg, Arjen Pelgröm, Irene Kuipers, Erik G J von Asmuth, Emilie P Buddingh, Miriam G Mooij, Angelique M A M Winkel, Lotte Haverman, Lorynn Teela, Anke H Maitland van der Zee, Johannes B van Goudoever, Simone Hashimoto, Suzanne W J Terheggen-Lagro
Background: Although rare, COVID-19 in children may lead to hospitalization due to severe respiratory symptoms, or a hyperinflammatory state called Multisystem Inflammatory Syndrome in Children (MIS-C). This study examined respiratory morbidity in children 5 to 12 months after hospitalization for MIS-C or COVID-19.
Methods: In this multi-center, prospective cohort study, children (0-17 years) with a history of hospitalization for MIS-C or COVID-19 in Dutch hospitals were invited for follow-up. Visits were scheduled in one of three academic hospitals, to evaluate current clinical status and health-related quality of life (HRQoL), and perform lung function tests and cardiopulmonary exercise testing (CPET).
Results: 72 children were included (43 MIS-C, 29 COVID-19), of whom 19% (5% and 41%, respectively) reported long-term respiratory symptoms including dyspnea and cough, a median of 8 months after hospitalization. Fatigue was the most common non-respiratory symptom. HRQoL was more frequently (60%) impaired in the COVID-19 group than the MIS-C group (14%). Spirometry (n = 48) and CPET (n = 40) were conducted in children aged > 4 and > 6 years, respectively. Spirometry was abnormal in 15% of the MIS-C group and 44% of COVID-19 group, CPET in 41% and 75%, respectively. Deconditioning patterns were the most common reason (30%) for abnormal CPET results.
Conclusion: Long-term respiratory sequelae and fatigue occurred after both MIS-C and severe COVID-19, but respiratory symptoms and impaired HRQoL were more frequent after COVID-19. Lung function and CPET abnormalities in children with COVID-19 often corresponded with symptoms. Children with MIS-C often showed CPET abnormalities without respiratory complaints or lung function changes.
{"title":"Pulmonary Sequelae of Severe Acute COVID-19 and Multisystem Inflammatory Syndrome (MIS-C) in Dutch Children.","authors":"Lieke C E Noij, Caroline L H Brackel, Mariëlle W Pijnenburg, Michiel A G E Bannier, Sanne F Kloosterman, Joost G van den Aardweg, Arjen Pelgröm, Irene Kuipers, Erik G J von Asmuth, Emilie P Buddingh, Miriam G Mooij, Angelique M A M Winkel, Lotte Haverman, Lorynn Teela, Anke H Maitland van der Zee, Johannes B van Goudoever, Simone Hashimoto, Suzanne W J Terheggen-Lagro","doi":"10.1002/ppul.71426","DOIUrl":"10.1002/ppul.71426","url":null,"abstract":"<p><strong>Background: </strong>Although rare, COVID-19 in children may lead to hospitalization due to severe respiratory symptoms, or a hyperinflammatory state called Multisystem Inflammatory Syndrome in Children (MIS-C). This study examined respiratory morbidity in children 5 to 12 months after hospitalization for MIS-C or COVID-19.</p><p><strong>Methods: </strong>In this multi-center, prospective cohort study, children (0-17 years) with a history of hospitalization for MIS-C or COVID-19 in Dutch hospitals were invited for follow-up. Visits were scheduled in one of three academic hospitals, to evaluate current clinical status and health-related quality of life (HRQoL), and perform lung function tests and cardiopulmonary exercise testing (CPET).</p><p><strong>Results: </strong>72 children were included (43 MIS-C, 29 COVID-19), of whom 19% (5% and 41%, respectively) reported long-term respiratory symptoms including dyspnea and cough, a median of 8 months after hospitalization. Fatigue was the most common non-respiratory symptom. HRQoL was more frequently (60%) impaired in the COVID-19 group than the MIS-C group (14%). Spirometry (n = 48) and CPET (n = 40) were conducted in children aged > 4 and > 6 years, respectively. Spirometry was abnormal in 15% of the MIS-C group and 44% of COVID-19 group, CPET in 41% and 75%, respectively. Deconditioning patterns were the most common reason (30%) for abnormal CPET results.</p><p><strong>Conclusion: </strong>Long-term respiratory sequelae and fatigue occurred after both MIS-C and severe COVID-19, but respiratory symptoms and impaired HRQoL were more frequent after COVID-19. Lung function and CPET abnormalities in children with COVID-19 often corresponded with symptoms. Children with MIS-C often showed CPET abnormalities without respiratory complaints or lung function changes.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 12","pages":"e71426"},"PeriodicalIF":2.3,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12706180/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145763573","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}
Emma R Lyons, Tim Vigers, Marisa Simoni, Courtney Lynn, Emily Muther
Introduction: Adolescents and young adults with CF (AYAwCF) are at increased risk of body image concerns due to a historical focus on weight gain, BMI, and adequate nutrition. Societal ideals create even greater risk. Elexacaftor/tezacaftor/ivacaftor (ETI) dramatically improved outcomes in CF, including contributing to weight gain. To date, no research has examined body image and adherence to ETI in AYAwCF. This study examines perspectives of AYAwCF on how body image may relate to ETI adherence.
Methods: Participants (n = 52) who were prescribed ETI for at least 6 months completed a series of questionnaires assessing: (1) body image concerns; (2) self-reported adherence to ETI; (3) perceptions related to ETI. Medical outcomes (body mass index [BMI], forced expiratory volume in 1 s [FEV1]), demographic information, and mental health screening data were collected via retrospective chart review.
Results: AYAwCF's perceptions of ETI adherence significantly differed based on body dissatisfaction; those with body dissatisfaction were more likely to endorse their weight and/or body image as a factor impacting ETI adherence, and those who wanted to be smaller were more likely to not want to take ETI due to weight gain. Differences between gender and body dissatisfaction emerged, showing that females more frequently reported wanting a smaller body. Among AYAwCF with elevated anxiety, those with body dissatisfaction had higher levels of anxiety than those without body dissatisfaction. AYAwCF with lower body esteem were more likely to have elevated symptoms of both depression and anxiety.
Discussion: This cross-sectional, single-site study explored the relationship between body image concerns for AYAwCF since the approval of ETI and their relationship to adherence. Results help identify areas for intervention.
{"title":"Body Image Perceptions and Elexacaftor/Tezacaftor/Ivacaftor (ETI) Use in Adolescents and Young Adults Living With Cystic Fibrosis.","authors":"Emma R Lyons, Tim Vigers, Marisa Simoni, Courtney Lynn, Emily Muther","doi":"10.1002/ppul.71418","DOIUrl":"10.1002/ppul.71418","url":null,"abstract":"<p><strong>Introduction: </strong>Adolescents and young adults with CF (AYAwCF) are at increased risk of body image concerns due to a historical focus on weight gain, BMI, and adequate nutrition. Societal ideals create even greater risk. Elexacaftor/tezacaftor/ivacaftor (ETI) dramatically improved outcomes in CF, including contributing to weight gain. To date, no research has examined body image and adherence to ETI in AYAwCF. This study examines perspectives of AYAwCF on how body image may relate to ETI adherence.</p><p><strong>Methods: </strong>Participants (n = 52) who were prescribed ETI for at least 6 months completed a series of questionnaires assessing: (1) body image concerns; (2) self-reported adherence to ETI; (3) perceptions related to ETI. Medical outcomes (body mass index [BMI], forced expiratory volume in 1 s [FEV1]), demographic information, and mental health screening data were collected via retrospective chart review.</p><p><strong>Results: </strong>AYAwCF's perceptions of ETI adherence significantly differed based on body dissatisfaction; those with body dissatisfaction were more likely to endorse their weight and/or body image as a factor impacting ETI adherence, and those who wanted to be smaller were more likely to not want to take ETI due to weight gain. Differences between gender and body dissatisfaction emerged, showing that females more frequently reported wanting a smaller body. Among AYAwCF with elevated anxiety, those with body dissatisfaction had higher levels of anxiety than those without body dissatisfaction. AYAwCF with lower body esteem were more likely to have elevated symptoms of both depression and anxiety.</p><p><strong>Discussion: </strong>This cross-sectional, single-site study explored the relationship between body image concerns for AYAwCF since the approval of ETI and their relationship to adherence. Results help identify areas for intervention.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 12","pages":"e71418"},"PeriodicalIF":2.3,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145655217","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}
Maya L Albanowski, Asher Prangley, Olivia M Stransky, Judy C Chang, Ashley Deal, Raelynn O'Leary, Rhiannon Phillips, Traci M Kazmerski
Background: More people with cystic fibrosis (PwCF) are surviving to adulthood and considering their reproductive futures starting in the teen years. MyVoice:CF is a web-based reproductive health decision support tool for adult female PwCF. Given the evolving cystic fibrosis (CF) population, we explored the role of MyVoice:CF and further reproductive health resources for adolescent female PwCF.
Methods: We conducted qualitative interviews with female adolescent and young adult (AYA) PwCF between age 13-26 years and their caregivers regarding their reproductive health experiences, the acceptability and usability of MyVoice:CF, and the desired additional resources for female AYA PwCF. We recorded and transcribed all interviews and analyzed them thematically using an inductive coding approach.
Results: We interviewed 16 AYAs and 11 caregivers. Key themes included: (1) AYA females with CF are considering their reproductive health and are curious about how CF will affect their future reproductive goals; (2) Participants found MyVoice:CF informative, user-friendly, and relevant for adolescents; (3) Suggested additions include information about puberty, healthy relationships, and learning how to self-advocate in interpersonal and medical settings; (4) AYA participants were particularly interested in hearing about reproductive health experiences of peers and being able to use MyVoice:CF to connect with other PwCF; (5) Participants shared the importance of open parent-child conversations regarding reproductive health and the use of MyVoice:CF to supplement these discussions.
Conclusion: Participants found MyVoice:CF acceptable and usable for adolescent female PwCF and as a tool to initiate conversations between AYAs, caregivers, and providers.
{"title":"Exploration of MyVoice: CF as a Reproductive Health Decision Support Tool for Adolescent Females With Cystic Fibrosis.","authors":"Maya L Albanowski, Asher Prangley, Olivia M Stransky, Judy C Chang, Ashley Deal, Raelynn O'Leary, Rhiannon Phillips, Traci M Kazmerski","doi":"10.1002/ppul.71438","DOIUrl":"10.1002/ppul.71438","url":null,"abstract":"<p><strong>Background: </strong>More people with cystic fibrosis (PwCF) are surviving to adulthood and considering their reproductive futures starting in the teen years. MyVoice:CF is a web-based reproductive health decision support tool for adult female PwCF. Given the evolving cystic fibrosis (CF) population, we explored the role of MyVoice:CF and further reproductive health resources for adolescent female PwCF.</p><p><strong>Methods: </strong>We conducted qualitative interviews with female adolescent and young adult (AYA) PwCF between age 13-26 years and their caregivers regarding their reproductive health experiences, the acceptability and usability of MyVoice:CF, and the desired additional resources for female AYA PwCF. We recorded and transcribed all interviews and analyzed them thematically using an inductive coding approach.</p><p><strong>Results: </strong>We interviewed 16 AYAs and 11 caregivers. Key themes included: (1) AYA females with CF are considering their reproductive health and are curious about how CF will affect their future reproductive goals; (2) Participants found MyVoice:CF informative, user-friendly, and relevant for adolescents; (3) Suggested additions include information about puberty, healthy relationships, and learning how to self-advocate in interpersonal and medical settings; (4) AYA participants were particularly interested in hearing about reproductive health experiences of peers and being able to use MyVoice:CF to connect with other PwCF; (5) Participants shared the importance of open parent-child conversations regarding reproductive health and the use of MyVoice:CF to supplement these discussions.</p><p><strong>Conclusion: </strong>Participants found MyVoice:CF acceptable and usable for adolescent female PwCF and as a tool to initiate conversations between AYAs, caregivers, and providers.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 12","pages":"e71438"},"PeriodicalIF":2.3,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145805151","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}
Background: Interstitial lung disease (ILD) has a significant impact on morbidity and mortality in juvenile idiopathic inflammatory myopathies (JIIM). Early and noninvasive detection methods are crucial to improve outcomes through timely diagnosis and intervention. Fractional exhaled nitric oxide (FeNO) is commonly used to assess airway inflammation; however, its utility for the diagnosis of ILD in JIIM remains uncertain. This study investigates the role of FeNO as a potential noninvasive biomarker for ILD in pediatric JIIM patients.
Methods: We enrolled 34 pediatric JIIM patients and classified them into ILD (n = 13) and non-ILD (n = 21) groups based on clinical and high-resolution computed tomography (HRCT) findings. Pulmonary function tests (PFTs), diffusing capacity for carbon monoxide (DLCO) and FeNO measurements were analyzed. Statistical analyses included correlations and receiver operating characteristic (ROC) analyses.
Results: The prevalence of ILD in our cohort was 38.2%. ILD patients had significantly reduced lung function parameters including forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC), total lung capacity (TLC), and diffusing capacity for carbon monoxide corrected for alveolar volume (DLCOcSB/VA) compared to the non-ILD group (p < 0.05). FeNO levels were significantly higher in patients with ILD (median 17 ppb vs. 10 ppb; p = 0.011). Furthermore, FeNO was negatively correlated with TLC, and ROC analysis showed discriminatory power in identifying ILD (AUC = 0.797; p = 0.011). Therefore, FeNO could be considered a valuable marker for detecting ILD.
Conclusion: FeNO correlates with impaired lung function in JIIM-associated ILD and thus shows considerable potential as a noninvasive biomarker to differentiate ILD in pediatric JIIM patients. Our results suggest that integrating FeNO measurements into clinical practice could improve early detection, facilitate timely intervention, and enhance clinical management. Importantly, this study is the first to specifically investigate the clinical utility of FeNO in JIIM patients with ILD. Further large-scale, prospective studies are warranted to validate the role of FeNO in predicting disease progression and therapeutic decisions.
{"title":"Clinical Significance of Fractional Exhaled Nitric Oxide (FeNO) Measurements in Juvenile Idiopathic Inflammatory Myopathy-Associated Interstitial Lung Disease.","authors":"Azer Kilic Baskan, Berrak Oztosun, Aybüke Gunalp, Elif Kilic Konte, Ayse Kalyoncu Ucar, Sebuh Kurugoglu, Ozgur Kasapcopur, Ayse Ayzit Kilinc Sakalli","doi":"10.1002/ppul.71409","DOIUrl":"10.1002/ppul.71409","url":null,"abstract":"<p><strong>Background: </strong>Interstitial lung disease (ILD) has a significant impact on morbidity and mortality in juvenile idiopathic inflammatory myopathies (JIIM). Early and noninvasive detection methods are crucial to improve outcomes through timely diagnosis and intervention. Fractional exhaled nitric oxide (FeNO) is commonly used to assess airway inflammation; however, its utility for the diagnosis of ILD in JIIM remains uncertain. This study investigates the role of FeNO as a potential noninvasive biomarker for ILD in pediatric JIIM patients.</p><p><strong>Methods: </strong>We enrolled 34 pediatric JIIM patients and classified them into ILD (n = 13) and non-ILD (n = 21) groups based on clinical and high-resolution computed tomography (HRCT) findings. Pulmonary function tests (PFTs), diffusing capacity for carbon monoxide (DLCO) and FeNO measurements were analyzed. Statistical analyses included correlations and receiver operating characteristic (ROC) analyses.</p><p><strong>Results: </strong>The prevalence of ILD in our cohort was 38.2%. ILD patients had significantly reduced lung function parameters including forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC), total lung capacity (TLC), and diffusing capacity for carbon monoxide corrected for alveolar volume (DLCOcSB/VA) compared to the non-ILD group (p < 0.05). FeNO levels were significantly higher in patients with ILD (median 17 ppb vs. 10 ppb; p = 0.011). Furthermore, FeNO was negatively correlated with TLC, and ROC analysis showed discriminatory power in identifying ILD (AUC = 0.797; p = 0.011). Therefore, FeNO could be considered a valuable marker for detecting ILD.</p><p><strong>Conclusion: </strong>FeNO correlates with impaired lung function in JIIM-associated ILD and thus shows considerable potential as a noninvasive biomarker to differentiate ILD in pediatric JIIM patients. Our results suggest that integrating FeNO measurements into clinical practice could improve early detection, facilitate timely intervention, and enhance clinical management. Importantly, this study is the first to specifically investigate the clinical utility of FeNO in JIIM patients with ILD. Further large-scale, prospective studies are warranted to validate the role of FeNO in predicting disease progression and therapeutic decisions.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 12","pages":"e71409"},"PeriodicalIF":2.3,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145637441","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}