Pediatric Pulmonology最新文献

{"title":"Upcoming events of interest.","authors":"","doi":"10.1002/ppul.71207","DOIUrl":"https://doi.org/10.1002/ppul.71207","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 7","pages":"e71207"},"PeriodicalIF":2.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144584536","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":"Congenital Pulmonary Airway Malformations: Experience of a Tunisian Tertiary Referral Center Over a Five-Year Period.","authors":"Hajer Chourou, Rim Ben Aziza, Manel Bourcheda, Yosra Sdiri, Houyem Khiari, Samia Kacem, Said Jlidi","doi":"10.1002/ppul.71188","DOIUrl":"10.1002/ppul.71188","url":null,"abstract":"<p><strong>Background: </strong>Congenital pulmonary airway malformations (CPAMs) are rare developmental lung anomalies. The optimal management of asymptomatic neonates remains a subject of debate. This study presents the experience of a Tunisian tertiary referral in the prenatal and postnatal diagnosis and management of CPAM.</p><p><strong>Methods: </strong>We conducted a 5-year retrospective descriptive study (2019-2024) study in the Neonatology and Intensive Care Unit of the Maternity and Neonatology Center of Tunis. All neonates diagnosed with CPAM were included.</p><p><strong>Results: </strong>Thirteen neonates with CPAM were managed. The mean maternal age was 31.4 yearsAntenatal diagnosis was made in 77% (10/13) of cases. Polyhydramnios was observed in two cases, hydrops fetalis in one, and dextrocardia in one. No antenatal interventions were performed. Cesarean section was performed in 10 cases, including four for fetal indications. Three neonates developed severe respiratory distress requiring mechanical ventilation; two died before surgical intervention, and one underwent lobectomy via thoracotomy at 9 months via thoracotomy, with an uneventful recovery. Histopathological examination confirmed type II CPAM. The remaining 10 patients were managed conservatively, with favorable outcomes during a mean follow-up of 4 years.</p><p><strong>Conclusions: </strong>The management of CPAM requires a multidisciplinary team and begins in the antenatal period. Even asymptomatic patients require close and prolonged monitoring, as complications may arise later. Surgical intervention should be considered on a case-by-case basis.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 7","pages":"e71188"},"PeriodicalIF":2.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12243706/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144601188","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":"Variations in the Management of Canadian Patients With CFTR Related Metabolic Syndrome/Cystic Fibrosis Screen Positive, Inconclusive Diagnosis (CRMS/CFSPID).","authors":"Dana Chemali, Erin Fleischer, Ryan Uyeno, Tara Mullowney, April Price","doi":"10.1002/ppul.71200","DOIUrl":"https://doi.org/10.1002/ppul.71200","url":null,"abstract":"<p><strong>Background: </strong>With routine newborn screening for cystic fibrosis (CF) now considered standard of care, the designation of CFTR Related Metabolic Syndrome (CRMS) or CF screen positive, indeterminate diagnosis (CFSPID) has been established. The majority of CRMS/CFSPID patients remain asymptomatic; however, 3.8%-44% of these patients may progress to a diagnosis of CF. This raises the question of how to optimally manage CRMS/CFSPID patients. We set out to gain a better understanding of the past practices employed at CF centers across Canada in the care of patients with a diagnosis of CRMS/CFSPID.</p><p><strong>Methods: </strong>An invitation to participate in an online survey was disseminated to CF centers in Canada through the REDCap database. The survey was completed in 2018. It included questions addressing patient population, timing of follow-up of CRMS/CFSPID patients, and details around specific investigations ordered.</p><p><strong>Results: </strong>Twelve out of 20 qualifying clinics completed the survey. The total patient population compiled included 1412 patients, of which 171 (12%) were classified as CRMS/CFSPID. There was wide variability in the timing of follow-up with a median (IQR) of 6 (5.25-12) months and a range of 3-12 months. There was also wide variability in the timing of repeat investigations such as sweat chloride, respiratory cultures, chest x-rays and spirometry.</p><p><strong>Conclusions: </strong>With current evidence showing that a considerable number of CRMS/CFSPID patients may progress to CF, ensuring these patients are identified as early as possible and followed in a consistent manner is essential. The Cystic Fibrosis Foundation and European Cystic Fibrosis Society have recently developed guidelines regarding the care of these patients. This survey describes historical practices for follow up of CRMS/CFSPID patients to help inform the development of Canadian consensus guidelines.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 7","pages":"e71200"},"PeriodicalIF":2.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144650103","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 Reference Interval for the Attainment of Comparable Term Functional Levels of Pulmonary Oxygenation Capacity in Healthy Preterm Infants: A Single-Center Retrospective Study.","authors":"Masako Yasui, Tohru Ogihara, Shigeo Yamaoka, Jun Shinohara, Yutaro Kawamura, Masamine Kijima, Yoshihiko Fukuda, Daisuke Nishioka, Yuri Ito, Akira Ashida","doi":"10.1002/ppul.71182","DOIUrl":"10.1002/ppul.71182","url":null,"abstract":"<p><strong>Objective: </strong>We aimed to identify the reference interval (RI) of the postmenstrual age (PMA) at which lung oxygenation capacity is sufficiently stable in healthy preterm infants.</p><p><strong>Design and setting: </strong>A single-center, retrospective study involving a cohort of infants born at gestational age (GA) < 34 weeks who were discharged without respiratory support and experienced no short-term adverse respiratory outcomes.</p><p><strong>Outcome measures: </strong>The PMA when oxygen supplementation was discontinued (Stop-O₂), and subsequent SpO₂ stabilization at ≥ 96% with room air spontaneous breathing for three consecutive days (Stable-SpO₂).</p><p><strong>Results: </strong>Of the 243 eligible infants, 176 meeting the Stable-SpO₂ criteria before discharge were analyzed. The upper limit of the RI (97.5th percentile) for Stop-O₂ was 40.2 weeks PMA and that for Stable-SpO₂ was 41.7. Limited to infants with GA < 30 weeks, the 97.5th percentile of Stop-O₂ and Stable-SpO₂ was 41.9 and 42.9 weeks PMA, respectively. When both indices plotted against GA were divided by K-means clustering, one distinct cluster straddled a wide range of GA with delayed achievement of Stop-O₂ (39.6, median weeks PMA) or Stable-SpO₂ (40.6). The SpO₂ status when Stable-SpO₂ criteria was fulfilled (mean SpO₂, 97.3%; time spent with SpO₂ > 90%, 97.7%) was nearly comparable to that of healthy term infants shortly after birth.</p><p><strong>Conclusions: </strong>The lung oxygenation capacity in most healthy preterm infants was near-term levels by 42 weeks PMA. Our data might be useful for determining the optimal timing for assessing respiratory function as well as the presence or absence of bronchopulmonary dysplasia in preterm infants.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 7","pages":"e71182"},"PeriodicalIF":2.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12247153/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144609027","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":"Trends in Neighborhood Opportunity, Hospitalizations, and Costs for Pediatric Asthma.","authors":"Jonathan M Gabbay, Benjamin V M Bajaj, Michael D Fishman, Cara S Guenther, Samantha Levano, Florinda Islamovic, Kevin P Fiori, Ann Chen Wu, Jennifer M Perez, Robert J Graham","doi":"10.1002/ppul.71185","DOIUrl":"https://doi.org/10.1002/ppul.71185","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate changes in (1) admissions and (2) costs of hospitalization over time relative to Child Opportunity Index (COI) levels for pediatric patients with asthma exacerbations.</p><p><strong>Study design: </strong>We conducted a retrospective study using the PHIS database from 2016 to 2024 for children aged 2 to 18 years who presented to children's hospitals with an asthma exacerbation. Outcomes were odds of admission and costs (per hospitalization) over time relative to COI levels. Mixed-effects regression models with interaction terms were used for analyses.</p><p><strong>Results: </strong>We identified 777,370 encounters, of which 208,415 (26.8%) were admitted. In adjusted models, odds of admission were significantly higher across all COI levels relative to encounters from very high COI levels for most years. Only encounters from very low COI neighborhoods showed a decreased average annual odds of admission relative to those from very high neighborhoods (adjusted annual percent change: -0.96% [95% CI: -1.59%, -0.33%], p = 0.003). Inflation-adjusted mean costs per hospitalization increased across all COI levels (p < 0.001 for all). The average annual cost increase for very low, low, moderate, and high COI levels was significantly lower compared to those from very high COI neighborhoods.</p><p><strong>Conclusions: </strong>Disparities in admissions by neighborhood opportunity continue to exist but appear to be converging over time. Hospitalization costs among COI levels are increasing, although at a faster rate for those from the highest-opportunity neighborhoods. Future research is needed to understand continued hospitalization disparities and drivers of rising costs to develop community- and individual-level targeted interventions.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 7","pages":"e71185"},"PeriodicalIF":2.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144601156","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 Effect of Limiting Anterior Chest Wall Movement With a Weighted Blanket on Comfort, Oxygenation, and Ventilation Homogeneity in Nonventilated Infants and Children Admitted to Hospital With Lower Respiratory Tract Infections: An Exploratory Pilot Study.","authors":"Eleonora Lozano-Ray, Andrew C Argent, Alison Lupton-Smith, Brenda M Morrow","doi":"10.1002/ppul.71186","DOIUrl":"10.1002/ppul.71186","url":null,"abstract":"<p><strong>Objectives: </strong>The objectives of this exploratory study were to describe the safety, comfort, and effects of applying a weighted blanket to restrict anterior chest movement on oxygenation, and regional ventilation in clinically stable, nonventilated infants, and children hospitalized with lower respiratory tract infections (LRTIs). Our hypothesis is that the application of a weighted blanket would not adversely affect comfort levels or oxygenation in nonventilated infants and children with LRTIs.</p><p><strong>Method: </strong>A prospective, exploratory interventional pilot study of nonventilated children and infants aged 2 months to 9 years of age admitted to hospital for the management of a LRTI. Comfort and pain scores, vital signs (heart rate [HR], respiratory rate [RR], oxygen saturation, and blood pressure), and electrical impedance tomography (EIT) measurements were captured at baseline, immediately and at 5 min, 10 min, and 30 min after application of the blanket.</p><p><strong>Results: </strong>Thirty children (median interquartile range [IQR] 8.31 months old [2.46-22.27]; 16 [53.30%] males), received the intervention, and were included in the analysis. Just over half of the children recruited were admitted with a clinical diagnosis of pneumonia (n = 17; 56.70%). No adverse events were recorded during the study intervention. Overall, oxygen saturation increased from median (IQR) 97% (91-100) to 100% (93-100) (p < 0.001); COMFORT-B score improved from median (IQR) 13 (6-14) to 11 (6-16) (p = 0.02) from baseline to 30 min postintervention and there was no significant change in the faces, legs, activity, cry, and consolability (FLACC) pain scale.</p><p><strong>Conclusion: </strong>The application of a weighted blanket in nonventilated infants and children with mild LRTI was well tolerated, with no adverse effects and was associated with improvements in comfort levels and SpO_2.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 7","pages":"e71186"},"PeriodicalIF":2.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12243703/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144601155","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":"Intravenous Bronchodilators in Pediatric Critical Asthma: A Systematic Review and Network Meta-Analysis.","authors":"Samer Abu-Sultaneh, Andrew G Miller, Sangita Basnet, Hannah J Craven, Abdallah Dalabih, Jose Enrique Irazuzta, Christine A Kapuscinski, Christopher J L Newth, Sana J Said, Elizabeth C Whipple, Lynda T Goodfellow, Benjamin R White, Narayan Prabhu Iyer","doi":"10.1002/ppul.71192","DOIUrl":"10.1002/ppul.71192","url":null,"abstract":"<p><strong>Introduction: </strong>Pediatric critical asthma is one of the most common pediatric illnesses in children admitted to the pediatric ward and pediatric intensive care unit (PICU). Adjunct intravenous (IV) bronchodilators are often used when initial management with systemic corticosteroids and inhaled short-acting beta agonists (SABA) fail to provide improvement in a patient's clinical condition. While the recent guidelines gave recommendations for the use of different IV bronchodilators compared to placebo, it did not include ranking on which one should be used as first-line or second-line agent. The aim of this network meta-analysis is to determine the effect of IV bronchodilators on patient-centered outcomes and rank medications based on their effectiveness in these outcomes.</p><p><strong>Methods: </strong>A systematic review was conducted using three databases MEDLINE, Embase, and CINAHL to identify randomized control trials examining the use of IV magnesium sulfate (MgSO₄), IV methylxanthines (aminophylline or theophylline), IV SABA (salbutamol, terbutaline) in pediatric critical asthma patients. Bayesian network metanalytic framework was used to compare the interventions. Results are reported as odds ratio (OR) or mean difference (MD) and 95% Credible Interval (CrI).</p><p><strong>Results: </strong>Twelve trials (n = 852) were included in the network meta-analysis. Largest reduction in hospital length of stay (LOS), PICU admission, and PICU LOS were noted with IV MgSO₄; (MD: -3.1 days, 95% CrI: -6.9 days to 0.13 days), (OR 0.21; 95% CrI 0.02, 1.3), and (MD: -4.0 days, 95% CrI: -7.1 days to -1.2 days) respectively. IV MgSO₄ was ranked first in three outcomes of interest with Surface Under the Cumulative Ranking curve (SUCRA) of 0.884 for hospital LOS, 0.919 for PICU admission, and 0.957 for PICU LOS. For preventing intubation, IV SABA was ranked the highest (SUCRA 0.995), but the only study with IV SABA had zero intubation events. In a sensitivity analysis that excluded studies with zero events, the intubation rate was lowest with IV MgSO₄ (OR 0.10; 95% CrI 0.003, 0.88) and it was ranked the best treatment (SUCRA 0.921).</p><p><strong>Conclusions: </strong>In this network meta-analysis comparing different IV adjunct bronchodilators, IV MgSO₄ was ranked first followed by IV SABA, and then IV methylxanthines. Given these findings and the favorable safety profile, ease of use, and low cost, IV MgSO₄ appears most promising the first adjunct IV bronchodilator, however, further large high-quality trials are still needed before it can be endorsed as routine first-line agent.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 7","pages":"e71192"},"PeriodicalIF":2.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12243718/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144601189","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 Developmental Consequences of Short Apneas and Periodic Breathing in Preterm Infants.","authors":"Rosemary S C Horne, Alicia K Yee, Leon S Siriwardhana, Lisa M Walter, Flora Y Wong","doi":"10.1002/ppul.71193","DOIUrl":"10.1002/ppul.71193","url":null,"abstract":"<p><strong>Objective: </strong>Preterm infants frequently experience short apneas which can occur in isolation or in a repetitive pattern termed periodic breathing. We assessed the consequences of the amount of time spent with short apneas on developmental outcomes at 2 years of age.</p><p><strong>Methods: </strong>Preterm infants (N = 23) born between 28 and 32 weeks gestational age were studied during daytime sleep in the supine position at 32-36 weeks post menstrual age (PMA), 36-40 weeks PMA, 3 months and 6 months corrected age. The percentage of total sleep time (TST) spent with apneas at each study was calculated. Infants were divided into those below and above the median cumulative time spent with apneas over the 4 studies (28.4% TST) and developmental assessments (Bayley Scales of Infant Development III, Early Childhood Behavior Questionnaire, Child Behavior Check List) at 2 years of age were compared with ANCOVA.</p><p><strong>Results: </strong>The above median group tended to have lower unadjusted scores for motor composite, social emotional composite and adaptive behavior composite on the Bayley's. After adjusting for confounders and %TST spent with apneas, the motor composite score was significantly lower in the above median group (p < 0.05). Perceptual Sensitivity was lower in the above median group (p < 0.05).</p><p><strong>Conclusions: </strong>In clinically stable very preterm infants, who had been discharged home with no concerns of respiratory instability, those infants who spent more time with short apneas, particularly periodic breathing, had reduced motor outcomes at 2 years of age. Our findings add to a growing literature suggesting that short apneas and periodic breathing are not benign.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 7","pages":"e71193"},"PeriodicalIF":2.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12243723/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144601190","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":"Antiphospholipid Syndrome With Pulmonary Embolism, Cerebral Venous Sinus and Cardiac Thrombosis in a 17-Year-Old.","authors":"Muhammed Tekinhatun, Mehmet Sait Duyu","doi":"10.1002/ppul.71202","DOIUrl":"https://doi.org/10.1002/ppul.71202","url":null,"abstract":"<p><p>A 17-year-old boy initially presented with syncope, fever, headache, and vomiting. Examination revealed neck stiffness and bilateral papilledema. Imaging identified superior sagittal and left transverse sinus thrombosis. One year later, he developed chest pain, seizures, and bilateral pulmonary emboli, with chocardiography demonstrated intracardiac thrombi. Surgical excision confirmed fibrinous thrombi. Despite anticoagulation, recurrent intracardiac thrombi and pulmonary hemorrhage occurred. Thrombophilia workup confirmed antiphospholipid syndrome (APS). This rare case highlights APS-related multisystemic thrombotic events and intracardiac thrombi in a pediatric patient, emphasizing the role of imaging in early diagnosis.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 7","pages":"e71202"},"PeriodicalIF":2.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144637755","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":"Repeatability of Multiple Breath Washout in Pediatric Primary Ciliary Dyskinesia.","authors":"Wallace B Wee, Layan M Bashi, Renee Jensen, Jonathan H Rayment, Teresa To, Felix Ratjen, Giles Santyr, Sharon D Dell","doi":"10.1002/ppul.71107","DOIUrl":"10.1002/ppul.71107","url":null,"abstract":"<p><strong>Background: </strong>Primary ciliary dyskinesia (PCD) is a motile ciliopathy characterized by abnormal mucociliary clearance and progressive lung disease. Spirometry is commonly used to monitor lung health and response to treatment, but it is known to be insensitive to early subclinical lung disease in PCD. Multiple breath washout is more sensitive than spirometry, but its repeatability in PCD has not been assessed.</p><p><strong>Objectives: </strong>To evaluate the (i) same-day and (ii) 28-day repeatability of lung clearance index 2.5% (LCI) in PCD.</p><p><strong>Methods: </strong>Participants > 6 years old with a confirmed PCD diagnosis were recruited from two Canadian PCD centers. Participants completed baseline lung function tests to measure their forced expiratory volume in 1-second, z-score (FEV1z), and LCI. Tests were repeated either on the same day or after 28 days. No clinical interventions were performed during the same-day repeat testing. Outpatient therapies were unchanged during 28-day repeat testing. Repeatability was assessed using intraclass correlation (ICC), and Bland-Altman plots (B&A).</p><p><strong>Results: </strong>Twenty-three participants were enrolled (same-day: 16; 28-day: 13). The same-day and 28-day repeat testing ICC for FEV1z were 0.9 and 0.92, and LCI were 0.95 and 0.71, respectively. Baseline testing showed that most participants had abnormal LCI (18 of 29 tests), even in those with FEV1z in the normal range. FEV1z and LCI exhibited a weak inverse correlation.</p><p><strong>Conclusions: </strong>LCI is a repeatable and sensitive lung function measure in PCD patients, and may be a suitable outcome metric for clinical trials, particularly in patients with early subclinical lung disease.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 7","pages":"e71107"},"PeriodicalIF":2.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12247151/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144609026","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}