Pediatric Pulmonology最新文献

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A Rare Complication of Hematopoietic Stem Cell Transplantation in Childhood: Pleuroparenchymal Fibroelastosis. 儿童造血干细胞移植的罕见并发症:胸膜实质纤维弹性增生。
IF 2.4 3区 医学
Pediatric Pulmonology Pub Date : 2026-10-01 DOI: 10.1002/ppul.71855
Esin Gizem Olgun, Mukaddes Ağırtıcı, Semra Küçük Öztürk, Suat Fitöz, Nazan Çobanoğlu
{"title":"A Rare Complication of Hematopoietic Stem Cell Transplantation in Childhood: Pleuroparenchymal Fibroelastosis.","authors":"Esin Gizem Olgun, Mukaddes Ağırtıcı, Semra Küçük Öztürk, Suat Fitöz, Nazan Çobanoğlu","doi":"10.1002/ppul.71855","DOIUrl":"https://doi.org/10.1002/ppul.71855","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 10","pages":"e71855"},"PeriodicalIF":2.4,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149113509","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}
引用次数: 0
Longitudinal Impact of Pseudomonas aeruginosa Versus Haemophilus influenzae Colonization on Lung Function Decline in Children With Primary Ciliary Dyskinesia. 铜绿假单胞菌与流感嗜血杆菌定殖对原发性纤毛运动障碍儿童肺功能下降的纵向影响
IF 2.4 3区 医学
Pediatric Pulmonology Pub Date : 2026-10-01 DOI: 10.1002/ppul.71861
Ömer Faruk İpek, Nagehan Emiralioğlu Ordukaya, Aslı Kuzu Kuşaklı, Meltem Akgül Erdal, Havva İpek Demir, Ebru Yalçın, Deniz Doğru, Uğur Özçelik
{"title":"Longitudinal Impact of Pseudomonas aeruginosa Versus Haemophilus influenzae Colonization on Lung Function Decline in Children With Primary Ciliary Dyskinesia.","authors":"Ömer Faruk İpek, Nagehan Emiralioğlu Ordukaya, Aslı Kuzu Kuşaklı, Meltem Akgül Erdal, Havva İpek Demir, Ebru Yalçın, Deniz Doğru, Uğur Özçelik","doi":"10.1002/ppul.71861","DOIUrl":"https://doi.org/10.1002/ppul.71861","url":null,"abstract":"<p><strong>Background: </strong>The association of Pseudomonas aeruginosa (PsA) and Haemophilus influenzae (HI) colonization with lung function in children with primary ciliary dyskinesia (PCD) remains insufficiently defined. We aimed to compare spirometric parameters between PsA- and HI-colonized children and evaluate longitudinal changes before, at, and after chronic colonization.</p><p><strong>Methods: </strong>This retrospective cohort included patients aged ≥ 5 years with definite or highly probable PCD and chronic PsA or HI colonization. Chronic colonization was defined using repeated sputum cultures over 12 months. Pulmonary function tests were analyzed 1 year before colonization, at colonization, and 1 year after colonization. FEV1, FVC, and FEF25-75 were reported as percent-predicted values and GLI 2012 z-scores. Individual pre-to-after-colonization change scores and bronchiectasis subgroup analyses were performed.</p><p><strong>Results: </strong>Among 290 patients with PCD, 40 met inclusion criteria: 10 with PsA and 30 with HI colonization. PsA-colonized patients were older at colonization than HI-colonized patients (17 [12-23] vs. 11 [9-15] years, p = 0.011), and bronchiectasis was more frequent in the PsA group (100% vs. 60%, p = 0.019). PsA colonization was associated with lower lung function across several spirometric measures. Both groups showed significant within-group declines in FEV1 and FEF25-75 over time. However, individual pre-to-after-colonization changes in FEV1 and FEF25-75 z-scores did not differ significantly between groups, including in the bronchiectasis subgroup analysis.</p><p><strong>Conclusions: </strong>PsA colonization was associated with lower lung function than HI colonization, but not with significantly greater longitudinal decline. These findings suggest that PsA may mark more advanced disease rather than independently accelerating functional loss. Larger prospective studies with non-colonized comparators are needed.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 10","pages":"e71861"},"PeriodicalIF":2.4,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149113512","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}
引用次数: 0
Postoperative Respiratory Events and Risk Factors After Hip Reconstruction in Children With Neurological Complex Chronic Conditions. 神经系统复杂慢性疾病儿童髋关节重建术后呼吸事件及危险因素
IF 2.4 3区 医学
Pediatric Pulmonology Pub Date : 2026-10-01 DOI: 10.1002/ppul.71851
Myungyeon Lee, Wonik Lee, Chang Ho Shin, Dong In Suh, Tae-Joon Cho, Ji Soo Park, Mi Hyun Song
{"title":"Postoperative Respiratory Events and Risk Factors After Hip Reconstruction in Children With Neurological Complex Chronic Conditions.","authors":"Myungyeon Lee, Wonik Lee, Chang Ho Shin, Dong In Suh, Tae-Joon Cho, Ji Soo Park, Mi Hyun Song","doi":"10.1002/ppul.71851","DOIUrl":"https://doi.org/10.1002/ppul.71851","url":null,"abstract":"<p><strong>Background: </strong>Children with neurological complex chronic conditions (NCCCs) frequently undergo hip reconstruction for hip displacement. Impaired airway clearance and respiratory muscle weakness may increase the risk of postoperative respiratory events. This study evaluated the incidence and predictors of postoperative respiratory events in this population.</p><p><strong>Methods: </strong>We retrospectively reviewed children with NCCCs who underwent hip reconstruction between 2015 and 2024. Baseline characteristics included age, sex, diagnosis, comorbidities, feeding method, ambulatory status, and respiratory support. Operative and anesthesia-related variables included the number of osteotomies, operative duration and scheduling, estimated blood loss, airway device, and arterial blood gas measurements. The primary outcome was postoperative desaturation (SpO<sub>2</sub> < 95%) requiring supplemental oxygen. Secondary outcomes included pulmonary complications and intensive care unit (ICU) utilization. Predictors were initially evaluated using an expanded multivariable logistic regression model, followed by a reduced model using Firth's penalized logistic regression.</p><p><strong>Results: </strong>Among 126 children (mean age, 7.7 ± 4.1 years), postoperative desaturation occurred in 30 (23.8%). Pulmonary complications included respiratory failure (n = 6), atelectasis (n = 5), pulmonary edema (n = 3), and pneumonia (n = 1). ICU care was required for 22 children (17.5%). Predictors of postoperative desaturation included congenital brain malformation (adjusted odds ratio [aOR], 21.76; 95% confidence interval [CI], 3.33-249.48), surgery performed on Friday (aOR, 3.35; 95% CI, 1.11-10.24), ≥ 3 osteotomies (aOR, 7.82; 95% CI, 1.83-36.36), and elevated intraoperative bicarbonate (HCO<sub>3</sub> <sup>-</sup> > 26 mEq/L; aOR, 6.49; 95% CI, 2.36-19.14).</p><p><strong>Conclusion: </strong>Postoperative desaturation was common after hip reconstruction in children with NCCCs, with a subset developing pulmonary complications or requiring ICU care. Identification of perioperative risk factors may improve risk stratification and support targeted respiratory monitoring and preventive strategies in this high-risk population.</p><p><strong>Level of evidence: </strong>Level III, retrospective cohort study.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 10","pages":"e71851"},"PeriodicalIF":2.4,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149113432","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}
引用次数: 0
Congenital Lung Malformations and Quantitative Assessment of Infant Tracheal-Bronchial Dimensions. 先天性肺畸形和婴儿气管支气管尺寸的定量评估。
IF 2.4 3区 医学
Pediatric Pulmonology Pub Date : 2026-10-01 DOI: 10.1002/ppul.71876
Antonio Mario Bulfamante, Ugo Maria Pierucci, Alessandro Campari, Salvatore Zirpoli, Carlotta Ardenghi, Michele Ceresola, Franco Pignatelli, Irene Paraboschi, Sara Costanzo, Anna Camporesi, Gloria Pelizzo
{"title":"Congenital Lung Malformations and Quantitative Assessment of Infant Tracheal-Bronchial Dimensions.","authors":"Antonio Mario Bulfamante, Ugo Maria Pierucci, Alessandro Campari, Salvatore Zirpoli, Carlotta Ardenghi, Michele Ceresola, Franco Pignatelli, Irene Paraboschi, Sara Costanzo, Anna Camporesi, Gloria Pelizzo","doi":"10.1002/ppul.71876","DOIUrl":"https://doi.org/10.1002/ppul.71876","url":null,"abstract":"<p><strong>Background: </strong>Congenital lung malformations (CLMs) are heterogeneous developmental anomalies caused by disrupted airway branching morphogenesis, but whether the central airways are also involved remains unclear. We quantified infant tracheobronchial dimensions in CLMs and compared them with published infant reference values.</p><p><strong>Methods: </strong>This retrospective single-center study included 81 of 94 infants with CLMs assessed for eligibility. Exclusions were secretions preventing measurement (n = 3), motion artifacts (n = 7), unavailable preoperative computed tomography (CT) (n = 2), and missing diagnosis (n = 1). Chest CT was performed before 1 year of age and before surgery. Tracheobronchial cross-sectional areas (CSAs) were measured at five standardized levels, natural-log transformed, and compared with expected values from the Chalwadi et al. equations for age 0 completed years. Holm correction was applied to the five overall comparisons; diagnostic subgroup and regression analyses were exploratory.</p><p><strong>Results: </strong>Diagnoses were congenital pulmonary airway malformation (CPAM, n = 40), pulmonary sequestration (n = 27), congenital lobar emphysema (n = 7), bronchial atresia (n = 6), and bronchogenic cyst (n = 1). Compared with expected values, the overall cohort showed larger proximal tracheal CSA (log<sub>e</sub> 3.22 vs. 3.11, p = 0.021; Holm-adjusted p = 0.042), distal tracheal CSA (3.33 vs. 3.21, p = 0.010; Holm-adjusted p = 0.030), right main bronchus (2.91 vs. 2.72, p = 0.007; Holm-adjusted p = 0.028), and left main bronchus (2.57 vs. 2.41, p = 0.002; Holm-adjusted p = 0.010), with no mid-tracheal difference (3.20 vs. 3.26; p = 0.233; Holm-adjusted p = 0.233). CPAM showed enlargement of the proximal trachea (p = 0.037), distal trachea (p = 0.022), right main bronchus (p = 0.017), and left main bronchus (2.62 vs. 2.41, p = 0.017). Pulmonary sequestration showed no significant differences. Congenital lobar emphysema showed reduced mid-tracheal CSA (2.86 vs. 3.26, p = 0.014). Bronchial atresia showed enlarged proximal trachea (p = 0.027), distal trachea (p = 0.027), and left main bronchus (p = 0.017).</p><p><strong>Conclusions: </strong>CLMs are associated with quantifiable tracheobronchial alterations during infancy, with distinct subtype-specific patterns. These findings suggest that CLMs may represent disorders of global airway development rather than isolated parenchymal abnormalities.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 10","pages":"e71876"},"PeriodicalIF":2.4,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149195790","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}
引用次数: 0
Surgical Management of Left Main Bronchus Compression Due to Pulmonary Tuberculosis in Children: The Role of Posterior Aortopexy. 儿童肺结核所致左主支气管压迫的外科治疗:后主动脉固定术的作用。
IF 2.4 3区 医学
Pediatric Pulmonology Pub Date : 2026-10-01 DOI: 10.1002/ppul.71877
Pierre Goussard, Jacques Janson, Ernst Eber, Savvas Andronikou, Andre Gie, Carmen Jacobs, Lars Ebert, Janette Verster, Jin Kim, Zane Ismail, Pawel Schubert
{"title":"Surgical Management of Left Main Bronchus Compression Due to Pulmonary Tuberculosis in Children: The Role of Posterior Aortopexy.","authors":"Pierre Goussard, Jacques Janson, Ernst Eber, Savvas Andronikou, Andre Gie, Carmen Jacobs, Lars Ebert, Janette Verster, Jin Kim, Zane Ismail, Pawel Schubert","doi":"10.1002/ppul.71877","DOIUrl":"https://doi.org/10.1002/ppul.71877","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 10","pages":"e71877"},"PeriodicalIF":2.4,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149183900","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}
引用次数: 0
Assessment of Cystic Fibrosis Lung Disease Using Electrical Impedance Tomography. 使用电阻抗断层成像评估囊性纤维化肺疾病。
IF 2.4 3区 医学
Pediatric Pulmonology Pub Date : 2026-10-01 DOI: 10.1002/ppul.71856
Heather Boas, Laura Bennett, Jennifer Faerber, Erin C Donnelly, Matthew Gari, Gina E Sailman, Alyssa Pierdomenico, Natalie Napolitano, Daniel Dorgan, Howard B Panitch, Clement L Ren
{"title":"Assessment of Cystic Fibrosis Lung Disease Using Electrical Impedance Tomography.","authors":"Heather Boas, Laura Bennett, Jennifer Faerber, Erin C Donnelly, Matthew Gari, Gina E Sailman, Alyssa Pierdomenico, Natalie Napolitano, Daniel Dorgan, Howard B Panitch, Clement L Ren","doi":"10.1002/ppul.71856","DOIUrl":"10.1002/ppul.71856","url":null,"abstract":"<p><strong>Introduction: </strong>Electrical impedance tomography (EIT) is a non-invasive, effort-independent imaging technique for measurement of regional lung ventilation and function that does not utilize ionizing radiation. There are limited data on the use of EIT to assess lung disease in people with CF (PwCF).</p><p><strong>Methods: </strong>This was a cross-sectional observational cohort study. Study participants performed EIT, multiple breath washout (MBW) and spirometry in a single session. We analyzed EIT results to calculate the global inhomogeneity index (GII) and regional ventilation delay at 40% of maximal inspiratory impedance (RVD40). We used pairwise Spearman correlations to compare EIT measurements with the lung clearance index (LCI) and percent predicted forced expiratory volume in 1 s (FEV1pp). We generated receiver operator characteristic (ROC) curves to assess specificity and sensitivity for GII and RVD40 to predict an abnormal LCI.</p><p><strong>Results: </strong>31 participants ranging in age from 6 to 65 years performed EIT and spirometry; 23 performed research-quality MBW. The median GII and RVD40 were 39.1 and 5.52, respectively. The median LCI was 8.9. RVD40 correlated moderately with LCI (r = 0.49, p = 0.02) and negatively correlated with FEV1pp (r = -0.36, p = 0.04). There was a weak positive correlation between GII and LCI (r = 0.27, p = 0.21). GII demonstrated a significant negative correlation with FEV1pp (r = -0.44, p = 0.01).</p><p><strong>Discussion: </strong>We found that EIT measurements correlated with spirometry and MBW. RVD40 was more sensitive and specific for ventilation inhomogeneity as compared to GII. Our data suggest that EIT measurements made during tidal breathing could be a useful clinical test in the evaluation of CF lung disease, though further investigations are needed.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 10","pages":"e71856"},"PeriodicalIF":2.4,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149195711","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}
引用次数: 0
Temporal Dynamics and Clinical Relevance of Oscillatory Amplitude Variability During High-Frequency Oscillatory Ventilation With Volume Guarantee in Preterm Infants. 早产儿在有容积保证的高频振荡通气过程中振荡幅度变异性的时间动力学和临床相关性。
IF 2.4 3区 医学
Pediatric Pulmonology Pub Date : 2026-10-01 DOI: 10.1002/ppul.71859
Kamal Ali, Mesaed Alsenani, Saad Alshareedah, Mohamed Sufyani, Saleh Algarni, Piero Alberti, Abdulaziz Homedi, Saif Alsaif, Ibrahim Ali, Theodore Dassios, Anne Greenough
{"title":"Temporal Dynamics and Clinical Relevance of Oscillatory Amplitude Variability During High-Frequency Oscillatory Ventilation With Volume Guarantee in Preterm Infants.","authors":"Kamal Ali, Mesaed Alsenani, Saad Alshareedah, Mohamed Sufyani, Saleh Algarni, Piero Alberti, Abdulaziz Homedi, Saif Alsaif, Ibrahim Ali, Theodore Dassios, Anne Greenough","doi":"10.1002/ppul.71859","DOIUrl":"https://doi.org/10.1002/ppul.71859","url":null,"abstract":"<p><strong>Objective: </strong>To characterise temporal dynamics, variance structure, determinants, and predictive relevance of oscillatory amplitude variability during high-frequency oscillatory ventilation with volume guarantee (HFOV-VG) in preterm infants.</p><p><strong>Methods: </strong>Single-centre retrospective cohort study using minute-level ventilator data collected during the first 72 h of HFOV-VG. Amplitude variability was quantified using standard deviation, coefficient of variation (CV), and root mean square of successive differences (RMSSD). Temporal patterns, variance decomposition, and determinants of variability were assessed using mixed-effects modelling separating within- and between-infant effects. Predictive performance for death before discharge or moderate-to-severe bronchopulmonary dysplasia was evaluated using receiver operating characteristic analysis.</p><p><strong>Results: </strong>A total of 237,790 measurements from 60 infants were analysed. Amplitude CV declined from 0.122 (0.072-0.160) at 0-6 h to 0.063 (0.035-0.106) at 48-72 h, while RMSSD decreased from 2.10 (1.22-2.69) to 1.13 (0.64-1.94). Variability was predominantly within-infant (ICC 0.246-0.282), whereas mean amplitude, VThf/kg, and frequency were predominantly between-infant. Within-infant increases in oscillatory frequency were associated with higher variability, whereas increases in mean airway pressure and time from HFOV initiation were associated with lower variability. The composite outcome occurred in 47/60 infants. Early oscillatory amplitude variability demonstrated moderate discrimination (AUC 0.72, 95% CI 0.55-0.86), with an AUC of 0.82 (95% CI 0.66-0.95) when combined with oscillatory frequency and weight-normalised DCO<sub>2</sub>.</p><p><strong>Conclusions: </strong>Oscillatory amplitude variability during HFOV-VG reflects dynamic ventilator-lung interactions. Its association with adverse respiratory outcome is hypothesis-generating and requires validation in larger independent cohorts.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 10","pages":"e71859"},"PeriodicalIF":2.4,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13634310/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149112983","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}
引用次数: 0
Effects of Gastroschisis Reduction on Pulmonary Function. 胃裂复位对肺功能的影响。
IF 2.4 3区 医学
Pediatric Pulmonology Pub Date : 2026-10-01 DOI: 10.1002/ppul.71860
Georgia O'Sullivan, Luke Jardine, Suzanne Bates, Peter Cunningham, Judith Hough
{"title":"Effects of Gastroschisis Reduction on Pulmonary Function.","authors":"Georgia O'Sullivan, Luke Jardine, Suzanne Bates, Peter Cunningham, Judith Hough","doi":"10.1002/ppul.71860","DOIUrl":"https://doi.org/10.1002/ppul.71860","url":null,"abstract":"<p><strong>Aim: </strong>Neonates who have gastroschisis may be at risk of a deterioration in their pulmonary function as the lesion is reduced. Electrical Impedance Tomography (EIT) is a non-invasive tool that can be used to examine respiratory function. The aim of this study was to examine the effects of bowel reduction in gastroschisis on respiratory function. We hypothesized that as the gastroschisis was reduced, End-Expiratory Lung Impedance (EELI) would also be reduced.</p><p><strong>Methods: </strong>A Sentec LuMon EIT monitor was used to measure End-Expiratory Lung Impedance (EELI) pre and post bowel reduction.</p><p><strong>Results: </strong>Eight patients, four female and four male, with an average gestational age of 36 + 6 weeks, and average birth weight of 2710 g were studied. Of the six patients that received a primary reduction, there was an average decrease in EELI of 17% in the first 48 h post reduction. The two patients that received staged silo reduction also saw a drop in EELI over the course of their reductions. Five patients took more than 2 days for their pulmonary function to return to their prereduction baseline.</p><p><strong>Conclusion: </strong>This data are consistent with a decrease in lung function with bowel reduction in gastroschisis. It also adds to the growing body of evidence of the utility of EIT in neonatal research.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 10","pages":"e71860"},"PeriodicalIF":2.4,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149195801","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}
引用次数: 0
Making the Diagnosis of Primary Ciliary Dyskinesia in Australia. 澳大利亚原发性纤毛运动障碍的诊断。
IF 2.4 3区 医学
Pediatric Pulmonology Pub Date : 2026-10-01 DOI: 10.1002/ppul.71863
Michelle Cai, Megan Frohlich, Louisa Owens, Kate Hardaker, Dominic A Fitzgerald, Raewyn Campbell, Bernadette Prentice, Lucy Morgan
{"title":"Making the Diagnosis of Primary Ciliary Dyskinesia in Australia.","authors":"Michelle Cai, Megan Frohlich, Louisa Owens, Kate Hardaker, Dominic A Fitzgerald, Raewyn Campbell, Bernadette Prentice, Lucy Morgan","doi":"10.1002/ppul.71863","DOIUrl":"10.1002/ppul.71863","url":null,"abstract":"<p><strong>Background and objective: </strong>Primary Ciliary Dyskinesia (PCD) is a genetic disorder associated with recurrent infections and irreversible airway damage. No single diagnostic test exists, necessitating use of a diagnostic algorithm that varies internationally. This retrospective study aimed to describe the clinical characteristics of patients, and algorithm used to diagnose PCD in New South Wales between 2014 and 2024.</p><p><strong>Methods: </strong>A retrospective review of patients diagnosed with PCD at Concord Repatriation General Hospital (CRGH) from 2014 to 2024 was performed.</p><p><strong>Results: </strong>This study included 52 patients with median age of diagnosis 7.0 years. The diagnostic algorithm consists of nasal nitric oxide (nNO) in patients > 5 years, and nasal brushings for high-speed video microscopy (HSVMA), immunofluorescence (IF) and electron microscopy (EM). Whole exome sequencing is not routine and was performed in 33% of the cohort. The diagnosis was confirmed by a multidisciplinary team that considered the test results and clinical phenotype. Non-diagnostic results occurred in 14% (nNO), 15% (HSVMA), 41% (IF) and 30% (EM) of patients eventually diagnosed with PCD or PCD \"highly likely\". Cumulatively, 56% of patients had at least one non-diagnostic result. Of the 17 patients who underwent whole exome sequencing with a PCD panel, 41% had diagnostic genetic results.</p><p><strong>Conclusion: </strong>Ciliary studies continue to be the mainstay of PCD diagnosis at CRGH. Although genetic testing is not part of the CRGH diagnostic algorithm, the CRGH algorithm has similar diagnostic performance to the ERS and ATS guidelines.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 10","pages":"e71863"},"PeriodicalIF":2.4,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13655486/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149165901","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}
引用次数: 0
Rehearsal to Premiere: Transfer of Chest Tube Insertion Skills From Simulation to the Clinical Context. 从预演到首演:从模拟到临床的胸管插入技巧转移。
IF 2.4 3区 医学
Pediatric Pulmonology Pub Date : 2026-10-01 DOI: 10.1002/ppul.71872
Lydia I Healy, Jessica Ann White, Briseida Mema
{"title":"Rehearsal to Premiere: Transfer of Chest Tube Insertion Skills From Simulation to the Clinical Context.","authors":"Lydia I Healy, Jessica Ann White, Briseida Mema","doi":"10.1002/ppul.71872","DOIUrl":"https://doi.org/10.1002/ppul.71872","url":null,"abstract":"<p><strong>Background: </strong>Simulation-based mastery learning (SBML) can establish procedural competence in a standardized environment, but how learners subsequently transfer these skills in clinical practice is less understood. This study explored the transfer of chest tube insertion skills from simulation to clinical practice and contextual factors influencing this process.</p><p><strong>Methods: </strong>In this mixed-methods study, novice trainees completed an SBML curriculum in chest tube insertion using the Seldinger technique and were assessed using an Objective Structured Assessment of Technical Skills (OSATS). Their first clinical chest tube insertion was subsequently observed, with procedural participation documented and OSATS items assessed for components performed. Field notes captured contextual factors surrounding each encounter. Clinical performance was analyzed descriptively and field notes thematically.</p><p><strong>Results: </strong>Thirty-five trainees completed the simulation curriculum and 24 were evaluated clinically. Nine (37%) observed, 13 (54%) actively participated, and 2 (8%) completed the procedure independently. Despite all learners achieving mastery in simulation, clinical participation varied considerably. Contextual factors included patient acuity and urgency, learner confidence, supervisor judgment, parental presence, clinical team support, and application of skills from related procedures.</p><p><strong>Conclusions: </strong>Mastery in a standardized simulation environment provides an important foundation for procedural learning but does not fully capture how skills are transferred in clinical practice. Learner, supervisor, patient, family, and environmental factors influenced opportunities to perform and adapt simulation-acquired skills. These findings highlight the complexity of transfer and the need for simulation designs that build on technical mastery by preparing learners to adapt across variable clinical contexts.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 10","pages":"e71872"},"PeriodicalIF":2.4,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149195858","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}
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