Pediatric Pulmonology最新文献

{"title":"Respiratory Function in Ventilated Newborn Infants Nursed Prone and Supine.","authors":"Konstantina Barka, Eleni Papachatzi, Sotirios Fouzas, Gabriel Dimitriou, Theodore Dassios","doi":"10.1002/ppul.71075","DOIUrl":"10.1002/ppul.71075","url":null,"abstract":"<p><strong>Objectives: </strong>Prone positioning has been associated with improved oxygenation in ventilated newborn infants but the physiological basis of this improvement has not been previously studied. We aimed to test the hypothesis that respiratory function measured by composite physiological indices would be improved in the prone compared to the supine position.</p><p><strong>Study design: </strong>Prospective observational study of ventilated newborns in a tertiary neonatal unit studied prone and supine at random order.</p><p><strong>Methodology: </strong>The ventilation to perfusion ratio (V_A/Q) and right to left shunt were non-invasively calculated using the oxyhemoglobin dissociation curve method. The gradient of the arterial to end tidal carbon dioxide (PaCO₂ - EtCO₂ gradient) was calculated to describe changes in the alveolar dead space.</p><p><strong>Results: </strong>Forty-six (26 male) infants with a median (IQR) gestational age of 34.8 (33.1-36.3) weeks and birth weight of 2.34 (1.77-2.87) kg were studied after 5 (2-10) hours of invasive ventilation. The V_A/Q was significantly higher in the prone position [0.57 (0.52-0.63)] compared to supine [0.53 (0.46-0.62), p = 0.001]. Right to left shunt was significantly lower in prone [7 (0-12) %] compared to supine [9 (1-16) %, p = 0.003]. The PaCO₂ - EtCO₂ gradient was significantly lower in prone [6.3 (3.8-8.4) mmHg] compared to supine [12.1 (7.1-16.0) mmHg].</p><p><strong>Conclusions: </strong>The prone position in ventilated neonates was associated with improved ventilation to perfusion matching and lower intrapulmonary shunting and alveolar dead space compared to supine.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 4","pages":"e71075"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11960726/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143754105","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":"Cardiac Echocardiography Findings in Previously Healthy Hospitalized Children With Bronchiolitis - a Retrospective Study.","authors":"George Shallufi, Yael Seider, Merav Zucker-Toledano, Kamal Masarweh, Michal Gur, Lea Bentur, Ronen Bar-Yoseph","doi":"10.1002/ppul.71079","DOIUrl":"10.1002/ppul.71079","url":null,"abstract":"<p><strong>Objective: </strong>Children less than two years of age with a prolonged or severe course of bronchiolitis are usually referred for Echocardiographic evaluation during hospitalization. Our aim was to assess the yield of Echocardiography (Echo) in these children and to assess possible characteristics predicting positive Echo findings.</p><p><strong>Study design: </strong>This retrospective study over 10 years included all children under two years of age admitted with acute bronchiolitis. Data was collected using the MDClone query tool. Children with abnormal Echo findings (positive Echo) were compared to a control group (comprised of all other admitted patients with bronchiolitis, and to bronchiolitis patients referred for cardiologic evaluation and found to have normal Echo).</p><p><strong>Results: </strong>Echocardiography was performed in 251/2137 eligible patients with a clinical diagnosis of bronchiolitis. Twenty-six of 251 had abnormal findings, 20 of mild and six of moderate to severe clinical significance. Younger age at diagnosis, Arab ethnicity, higher respiratory rate, lower pH, higher pCO₂, longer hospital length of stay, PICU admission and mechanical ventilation were more likely to be associated with positive Echo findings.</p><p><strong>Conclusion: </strong>Although Echocardiographic assessment of healthy infants during bronchiolitis is normal in the vast majority of children, the noninvasive, non-irradiating nature of the assessment, combined with the possible detrimental repercussions of a missed congenital cardiac anomaly, suggests performing Echocardiography during an atypical or severe course of bronchiolitis. However, larger studies are required to assess the yield of Echocardiography in bronchiolitis.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 4","pages":"e71079"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11975167/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143795879","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":"Time Toxicity: Quantifying Healthcare Utilization Attributable to Pediatric Home Ventilation.","authors":"Emily D Johnson, Renee D Boss, Woo Yeon Park, Khyzer B Aziz","doi":"10.1002/ppul.71081","DOIUrl":"https://doi.org/10.1002/ppul.71081","url":null,"abstract":"<p><strong>Objective: </strong>Determine the \"time toxicity,\" or time burden patients experience when pursuing health-related interventions, of pediatric invasive home ventilation.</p><p><strong>Study design: </strong>Retrospective chart review.</p><p><strong>Methods: </strong>We identified all patients from a single neonatal intensive care unit who received a tracheostomy with/without home ventilation (T + /-HV) from 2016 to 2024. After matching the gestational week distribution of this cohort, five controls were randomly selected for each T + /-HV patient. Healthcare encounters in the electronic medical record were compared for the cohort versus controls. All encounters on a single calendar day were counted as 1 day of healthcare utilization.</p><p><strong>Results: </strong>We identified 62 patients with T + /-HV and 310 controls. Mortality was 35.5% among the T + /-HV cohort and 9% among controls. Median inpatient hospitalizations (7.0 vs. 2.0), total healthcare encounters (64.0 vs. 15.5), days of healthcare utilization (296 vs. 46), and healthcare utilization ratio (0.4 vs. 0.1) were all significantly higher for the T + /-HV cohort compared with controls. Differences were greatest in the first year of life. Among survivors in the T + /-HV cohort, decannulated patients had significantly greater healthcare utilization than those not decannulated.</p><p><strong>Conclusions: </strong>This proof-of-concept analysis quantified \"time toxicity\" for pediatric invasive home ventilation and demonstrated that neonates who receive a tracheostomy during their initial admission spent over half of all days in their first 2 years of life interacting with the healthcare system. \"Time toxicity\" could add objective information to better prepare families for what life looks like with a child using a ventilator at home.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 4","pages":"e71081"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143780865","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":"Pancreatic Status Is Not a Risk Factor for Cystic Fibrosis-Related Bone Disease.","authors":"Miri Dotan, Maya Trau, Meir Mei-Zahav, Huda Mussaffi, Yulia Gendler, Hannah Blau, Dario Prais","doi":"10.1002/ppul.71078","DOIUrl":"10.1002/ppul.71078","url":null,"abstract":"<p><strong>Background: </strong>As the life expectancy of people with cystic fibrosis (PwCF) increases, understanding long-term complications, including CF-related bone disease (CFBD), is crucial.</p><p><strong>Objective: </strong>This study aimed to longitudinally characterize CFBD and to compare the bone status of pancreatic sufficient (PS) and pancreatic insufficient (PI) PwCF.</p><p><strong>Methods: </strong>This longitudinal analysis included PwCF older than 8 years of age who had at least one dual-energy X-ray absorptiometry test between 2008 and 2021. Data were collected on serum parameters of bone metabolism, nutritional history, habitual activity, and fractures in addition to other demographic and clinical characteristics.</p><p><strong>Results: </strong>The study included 80 PwCF: 32 (40%) were PS and 48 (60%) PI. Normal dual-energy X-ray absorptiometry results were found in 42 (53%) patients: 16 (50%) in the PS group and 26 (54%) in the PI group (p = 0.72). Three (9%) of the PS group and seven (15%) of the PI group had at least one Z-score below -2 (p = 0.49). The longitudinal bone density decline over a mean of 4.8 years was similar in the two groups. In a logistic regression analysis, pancreatic insufficiency was not found to be a risk factor for CFBD. Female sex was the only significant risk factor for a pathological Z-score.</p><p><strong>Conclusions: </strong>The prevalence and severity of CFBD were not found to correlate with pancreatic sufficiency. The similar prevalence of CFBD between patients with PS and PI suggests that screening, and eventually treatment, should be offered to all PwCF, irrespective of pancreatic status.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 4","pages":"e71078"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11962574/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143764604","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":"Genotype-Phenotype Correlation in a Group of Italian Patients With Primary Ciliary Dyskinesia.","authors":"Laura Petrarca, Valentina Guida, Raffaella Nenna, Alessandro De Luca, Marina Goldoni, Laura Bernardini, Maria Giulia Conti, Giuseppe Cimino, Enrica Mancino, Laura Masuelli, Piercarlo Poli, Fabio Midulla","doi":"10.1002/ppul.71057","DOIUrl":"https://doi.org/10.1002/ppul.71057","url":null,"abstract":"<p><strong>Introduction: </strong>Primary Ciliary Dyskinesia (PCD) is a rare genetic disorder characterized by abnormalities in the motile cilia. Diagnosis could be hard to make, but genetic analysis could be important for the diagnosis and for defining prognosis.</p><p><strong>Aim of the study: </strong>To evaluate the clinical, ultrastructural, and molecular characteristics of a cohort of PCD subjects.</p><p><strong>Materials and methods: </strong>The study cohort included PCD patients enrolled in two Italian centers. Clinical data were retrospectively collected consulting medical records. All patients underwent nasal brushing and peripheral blood sampling for ultrastructural analysis of motile cilia and genetic testing, respectively.</p><p><strong>Results: </strong>A total of 39 patients with PCD were enrolled (median age 25.5 years, range 2.5-54.3 years). All patients showed common clinical features, which included SIT in 22/39 (56.4%), chronic rhinitis in 31/39 (79.5%), chronic sinusitis in 26/37 (66.7%), chronic cough in 32/39 (82.1%), and neonatal respiratory distress in 46.2% (18/39). The genetic defect was identified in 27/39 patients (69.2%), while a diagnostic ultrastructure was found in 27/35 (77.1%). Assessing genotype-phenotype correlations, subjects with biallelic pathogenic variants in CCDC39 and CCDC40 genes had a significantly lower forced expiratory volume in the first second of exhalation value (p = 0.017) than subjects with pathogenic variants in DNAH5 or in other PCD-related genes.</p><p><strong>Conclusions: </strong>Our study further highlights the high heterogeneity of ultrastructural defects and genetics characterizing patients with PCD, as well as providing additional evidence that patients with biallelic pathogenic variants in CCDC39 or CCDC40 display a worse clinical phenotype than patients with pathogenic variants in other PCD genes.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 4","pages":"e71057"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143780845","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":"Asymptomatic Airway Foreign Body: A Counsel for Caution.","authors":"Srilakshmi Mula, Vigneshwaran Muthuvel, Sravani Madhileti, Ramya Srinivasa Rangan, Venkatesh Chandrasekaran, Dhandapany Gunasekaran","doi":"10.1002/ppul.71089","DOIUrl":"https://doi.org/10.1002/ppul.71089","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 4","pages":"e71089"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143803942","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Genetic Testing Utilization in the U.S. Registry for Childhood Interstitial and Diffuse Lung Diseases.","authors":"Laura A Voss, Rebekah J Nevel, Jennifer A Wambach, Lawrence M Nogee, Robin R Deterding, Alicia M Casey, Michael G O'Connor, Daniel I Craven, Jane B Taylor, Gail H Deutsch, Jade B Tam-Williams, Lea C Steffes, Steven K Brennan, Maria T Santiago, Sara C Sadreameli, Andrea F Heras, Michael R Powers, Antonia P Popova, Manvi Bansal, Aaron Hamvas, William A Gower, Fernando Urrego, Lisa R Young","doi":"10.1002/ppul.71073","DOIUrl":"10.1002/ppul.71073","url":null,"abstract":"<p><strong>Introduction: </strong>Childhood interstitial and diffuse lung diseases (chILD) comprise a diverse group of rare disorders. Identifying the underlying cause is crucial for treatment, prognosis, and estimating recurrence risk. The objective of this study was to assess the utilization of genetic testing for subjects enrolled in the United States National Registry for ChILD, a multicenter observational study.</p><p><strong>Methods: </strong>Genetic data from participating sites were reviewed and analyzed in relationship to clinical characteristics.</p><p><strong>Results: </strong>Of 609 children enrolled from 22 centers, genetic testing was performed for 55.5% (n = 338). Genetic testing results were positive (diagnostic) for 22.8% (n = 77), negative for 60.7% (n = 205), and uncertain for 16.6% (n = 56). Most testing was performed through gene panels (55.9%), followed by exome sequencing (ES) or whole genome sequencing (WGS) (26.9%), single gene testing (24.6%), and/or chromosomal microarray (11.8%). For participants with positive (diagnostic) genetic testing results, the majority were diagnosed through gene panel (33.8%; n = 26) or single gene testing (32.5%; n = 25). The most common diagnosis confirmed by genetic testing was SFTPC-associated surfactant metabolism dysfunction. Of the 59 subjects with unclassified ILD, only 22% (n = 13) had undergone ES or WGS, 61% (n = 36) had received panel testing, and 27% (n = 16) did not have any genetic testing reported.</p><p><strong>Conclusion: </strong>The utilization of genetic testing has been variable in infants and children enrolled in the ChILD Registry. Additional efforts are needed to develop genetic testing recommendations for children with suspected ILD. Furthermore, there is opportunity for broader utilization of ES/WGS and genetic discovery for children with lung disease of unclear etiology.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 4","pages":"e71073"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11960725/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143754096","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":"Automatic Analysis of Lung Sounds in 3-Year-Old Children.","authors":"Hiroyuki Mochizuki, Takashi Matsushita, Kota Hirai, Fumio Niimura, Hiroyuki Furuya, Yoshiyuki Yamada, Atsushi Uchiyama","doi":"10.1002/ppul.71071","DOIUrl":"https://doi.org/10.1002/ppul.71071","url":null,"abstract":"<p><strong>Introduction: </strong>In recent years, with the advent of artificial intelligence, clear progress has been made in the clinical application of lung sound analysis techniques.</p><p><strong>Objective: </strong>Using a new software program to analyze pediatric lung sounds using machine learning (ML), we conducted a lung sound survey study of 139 healthy 3-year-old children.</p><p><strong>Subjects and methods: </strong>All cases were surveyed using the ATS-DLD questionnaire, which mainly included items related to a history of wheezing, diagnosis of asthma, and history of respiratory syncytial virus (RSV) infection, allergies and environment. The characteristics of the lung sounds were examined, along with the results of the questionnaire and lung sound parameters.</p><p><strong>Results: </strong>Children with a history of wheezing showed a higher maximum inspiratory frequency (FAP₀), lower basal power (PAP₀) (p < 0.001 and p < 0.001, respectively), and lower RPF_50p and RPF_75p (p = 0.003 and p = 0.003, respectively), suggesting the enhancement of the high-pitched region of the lung sound spectrum. A similar tendency was observed in children with a history of asthma or RSV infection. Furthermore, in the group of children with a history of wheezing, those with a history of acute respiratory tract infection (ARI) within 1 week were found to have an enhancement of the high-pitched region relative to those without history of ARI.</p><p><strong>Conclusions: </strong>By utilizing a new analysis software program using ML, we found that 3-year-old children with a history of wheezing or suspected asthma had characteristic lung sounds even when healthy.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 4","pages":"e71071"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143754090","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Changes in Respiratory Patterns From Pressure Control Ventilation to Neurally Adjusted Ventilatory Assist Assessed by Electrical Impedance Tomography.","authors":"Marika Rahtu, Inéz Frerichs, Tobias H Becher, Tytti Pokka, Andreas D Waldmann, Thalia Papadouri, Anton H van Kaam, Peter C Rimensberger, Richard Bayford, Outi Peltoniemi, Merja Kallio","doi":"10.1002/ppul.71077","DOIUrl":"https://doi.org/10.1002/ppul.71077","url":null,"abstract":"<p><strong>Introduction: </strong>Neurally Adjusted Ventilatory Assist (NAVA) is increasingly used as ventilatory support for preterm infants. Changes in ventilation distribution and respiratory patterns during the transition from patient-triggered time-cycled Pressure Controlled Ventilation (PCV) to NAVA have not yet been studied. This study aimed to evaluate the impact of ventilation mode (PCV and NAVA, respectively) on ventilation distribution and breathing patterns in preterm infants with Electrical Impedance Tomography.</p><p><strong>Methods: </strong>This study included 16 premature infants at Oulu University Hospital who participated in the observational CRADL project and were on NAVA. EIT data was retrospectively assessed by choosing a 1-min stable recording before and after the change from one to the other mode. The primary endpoint was changes in global and regional ventilation parameters (ΔZ), and the secondary endpoints were Centers of Ventilation (CoVs), the amount of silent spaces, and the duration of inspiratory and respiratory cycle times.</p><p><strong>Results: </strong>A larger variation in the global tidal impedance variation (p < 0.05) and the respiratory cycle time (p < 0.05) was observed on NAVA than on PCV. Sighs, which were determined as a breath impedance change twice the size of an average breath on PCV, were more frequent during NAVA than PCV (5.1% vs 0.8%, respectively). Mean global or regional impedance variations or silent spaces did not differ between PCV and NAVA.</p><p><strong>Conclusion: </strong>NAVA allowed more variable breathing patterns during invasive respiratory support than patient-triggered PCV. However, variability in the respiratory cycle did not lead to systematic changes in ventilation distribution or silent spaces.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 4","pages":"e71077"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143764603","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Reliability of Composite Autonomic Symptom Score (COMPASS)-31 in Congenital Central Hypoventilation Syndrome.","authors":"Benjamin Dudoignon, Plamen Bokov, Fatima Benterki, Nathalie Couque, Casey M Rand, Debra E Weese-Mayer, Christophe Delclaux","doi":"10.1002/ppul.71072","DOIUrl":"10.1002/ppul.71072","url":null,"abstract":"<p><strong>Rationale: </strong>Congenital central hypoventilation syndrome (CCHS) is a rare disorder characterized by alveolar hypoventilation and variable autonomic nervous system (ANS) dysfunction (ANSD) due to mutations in PHOX2B, a gene crucial for ANS neural crest lineage differentiation.</p><p><strong>Objectives and methods: </strong>Our prospective study aims were twofold: to (1) assess the relationships between the subjective Composite Autonomic Symptom Score (COMPASS)-31 and objective indices of ANSD obtained from heart rate variability analyses, ambulatory blood pressure (BP) monitoring, and CO₂ chemosensitivities and (2) describe the organ system ANSD, its relationship to PHOX2B genotype, and its consequences on quality of life (PedsQL) in children with CCHS.</p><p><strong>Results: </strong>Thirty-two PHOX2B mutation-confirmed subjects (median [range] age 9.2 years (4.4; 18.0), 15 girls) were enrolled. COMPASS-31 was assessed in 32 matched (sex and age, range: 4.3; 18.9 years) healthy controls. As compared to healthy controls, children with CCHS had increased vasomotor (p = 0.001), secretomotor (p = 0.021), gastrointestinal (p = 0.002) and pupillomotor (p = 0.028) scores and decreased orthostatic intolerance scores (p = 0.050). There was no difference in overall COMPASS-31 score between CCHS and controls (p = 0.083). However, in CCHS, overall COMPASS-31 scores correlated with high frequencies (HF) normalized (cardiac parasympathetic modulation: R = -0.53; p = 0.002), low frequencies (LF)/HF ratio (R = 0.56; p< 0.001), and both systolic and diastolic nighttime BP dipping (R = 0.45, p = 0.012 and R = 0.40, p = 0.028, respectively). No significant relationships between COMPASS-31 scores and chemosensitivity testing, PedsQL scores, or PHOX2B genotype were identified.</p><p><strong>Conclusions: </strong>COMPASS-31 identified some aspects of CCHS-related ANSD, and scores correlate with objective ANS function measures, supporting the potential utility of COMPASS-31 in CCHS.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 4","pages":"e71072"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11951072/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143731222","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}