Pediatric Pulmonology最新文献

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Re-Entry High-Altitude Pulmonary Edema in a Child. 儿童再入高原肺水肿一例。
IF 2.4 3区 医学
Pediatric Pulmonology Pub Date : 2026-09-01 DOI: 10.1002/ppul.71811
Andrea Carolina Munive Gnecco, Isabella Andrea Bolaños Bermúdez, Luis Fernando Pulido, Gustavo Adolfo Triana Rodríguez
{"title":"Re-Entry High-Altitude Pulmonary Edema in a Child.","authors":"Andrea Carolina Munive Gnecco, Isabella Andrea Bolaños Bermúdez, Luis Fernando Pulido, Gustavo Adolfo Triana Rodríguez","doi":"10.1002/ppul.71811","DOIUrl":"10.1002/ppul.71811","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 9","pages":"e71811"},"PeriodicalIF":2.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13505238/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148813759","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
Evaluation of Pulmonary and Otological Manifestations in Primary Ciliary Dyskinesia and Their Effect on Quality of Life. 原发性纤毛运动障碍的肺部和耳部表现及其对生活质量的影响。
IF 2.4 3区 医学
Pediatric Pulmonology Pub Date : 2026-09-01 DOI: 10.1002/ppul.71810
Muruvvet Yanaz, Ela Erdem Eralp, Cansu Yilmaz Yegit, Aynur Gulieva, Mine Kalyoncu, Merve Selcuk Balci, Seyda Karabulut, Nuru Aliyev, Nezafet Ozturk, Almala Pınar Ergenekon, Yasemin Gokdemir, Ali Cemal Yumusakhuylu, Ayca Ciprut, Bulent Karadag
{"title":"Evaluation of Pulmonary and Otological Manifestations in Primary Ciliary Dyskinesia and Their Effect on Quality of Life.","authors":"Muruvvet Yanaz, Ela Erdem Eralp, Cansu Yilmaz Yegit, Aynur Gulieva, Mine Kalyoncu, Merve Selcuk Balci, Seyda Karabulut, Nuru Aliyev, Nezafet Ozturk, Almala Pınar Ergenekon, Yasemin Gokdemir, Ali Cemal Yumusakhuylu, Ayca Ciprut, Bulent Karadag","doi":"10.1002/ppul.71810","DOIUrl":"10.1002/ppul.71810","url":null,"abstract":"<p><strong>Objective: </strong>Otitis media and conductive hearing loss are common in patients with primary ciliary dyskinesia (PCD). We primarily aimed to assess the impact of otologic features of individuals with PCD on health-related quality of life (QOL), and our secondary aims are characterization of the coexisting pulmonary and sinonasal findings.</p><p><strong>Methods: </strong>Demographic data, spirometry values, a health-related quality of life questionnaire for PCD patients (QOL-PCD), physical examination data of sinonasal and otologic systems, the sinonasal outcome test-22, and audiologic tests were recorded in this cross-sectional study. The pure tone audiometry, distortion product otoacoustic emission (DPOAE), tympanogram, and the Turkish matrix test (TUR-matrix) evaluating speech understanding in noise were performed.</p><p><strong>Results: </strong>Fifty-six patients with PCD were included in the study. The scores of the social, upper, and lower respiratory domains of the QOL-PCD were significantly lower in adults than in children and adolescents (p-value < 0.001, 0.02, and 0.01, respectively). The hearing and the TUR-matrix were pathologic in 26 (46.4%) and 36 (64.3%) cases, respectively. Patients with pathological hearing had significantly worse results in regards to the FEV<sub>1</sub> z-score (-1.7 vs. -0.8, p = 0.01), the TUR-matrix test (20 vs. 16 cases, p = 0.001), the DPOAE (25 vs. 11 cases, p < 0.001), and the tympanogram (26 vs. 12 cases, p < 0.001) than patients with normal hearing.</p><p><strong>Conclusion: </strong>Sinopulmonary involvement and hearing manifestations are very common in patients with PCD, and speech understanding in noise is often impaired. These manifestations impact QOL in various areas.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 9","pages":"e71810"},"PeriodicalIF":2.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13507764/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148819220","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
Neonatal Course and Long-Term Outcome in Children With Congenital Chylothorax. 先天性乳糜胸患儿的新生儿病程和长期预后。
IF 2.4 3区 医学
Pediatric Pulmonology Pub Date : 2026-09-01 DOI: 10.1002/ppul.71820
N Beullens, K Smets, M De Lausnay, P Schelstraete, S Van Daele, J Willekens, J Van Dorpe, H Schaballie
{"title":"Neonatal Course and Long-Term Outcome in Children With Congenital Chylothorax.","authors":"N Beullens, K Smets, M De Lausnay, P Schelstraete, S Van Daele, J Willekens, J Van Dorpe, H Schaballie","doi":"10.1002/ppul.71820","DOIUrl":"10.1002/ppul.71820","url":null,"abstract":"<p><strong>Background: </strong>Congenital chylothorax is a rare pulmonary developmental disorder with high associated morbidity and mortality. A limited number of cases have been described, and clinical manifestations and outcomes vary.</p><p><strong>Methods: </strong>This study aims to delineate the neonatal and long-term outcome of patients with congenital chylothorax by retrospective and prospective data collection at Ghent University Hospital in Belgium between 1992 and 2022.</p><p><strong>Results: </strong>Eighteen patients with congenital chylothorax were included, seven of whom had a pathology-confirmed diagnosis of congenital pulmonary lymphangiectasia. Of 16 patients with prenatal diagnosis, four underwent an intra-uterine intervention. Thirteen patients were born prematurely. The most common comorbidities were hydrops (8/18), pulmonary hypertension (3/18), and reflux disease (6/18). One patient was diagnosed with Noonan syndrome. The majority of patients (14/18) required mechanical ventilation. Treatment included pleural drainage (15/18), total parenteral nutrition (14/18), medium-chain triglyceride diet (11/18), somatostatin (8/18), and surgical intervention (3/18), with a median hospital stay of 59 days. Three patients died during or after the neonatal period. Follow-up ranged from 6 months to 30 years, with most patients reporting good general health but variable respiratory complaints. Five out of eight respiratory function tests showed mild abnormalities. Follow-up chest CT showed persistent changes in all but two patients up to a maximum age of 16 years. Health-related quality of life was similar to healthy controls.</p><p><strong>Conclusion: </strong>Congenital chylothorax demonstrates a variable neonatal course. Long-term outcomes are generally favorable in patients without comorbidities, although mild pulmonary function and chest CT abnormalities persist in the majority of patients.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 9","pages":"e71820"},"PeriodicalIF":2.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13539660/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148888263","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
Prevalence Patterns of Children's Interstitial and Diffuse Lung Disease (chILD) in Colombia, 2016-2024. 2016-2024年哥伦比亚儿童间质性和弥漫性肺疾病(儿童)患病率模式
IF 2.4 3区 医学
Pediatric Pulmonology Pub Date : 2026-09-01 DOI: 10.1002/ppul.71832
Andrea Parra Buitrago, Jefferson Antonio Buendía
{"title":"Prevalence Patterns of Children's Interstitial and Diffuse Lung Disease (chILD) in Colombia, 2016-2024.","authors":"Andrea Parra Buitrago, Jefferson Antonio Buendía","doi":"10.1002/ppul.71832","DOIUrl":"10.1002/ppul.71832","url":null,"abstract":"<p><strong>Background: </strong>Children's interstitial and diffuse lung disease (chILD) comprise a heterogeneous group of rare disorders associated with substantial morbidity and mortality. Population-based epidemiological data remain scarce in low- and middle-income countries, limiting health system planning and policy development. We aimed to estimate the prevalence and temporal trends of chILD in Colombia using nationwide administrative healthcare data.</p><p><strong>Methods: </strong>We conducted a retrospective, population-based study using Colombia's Registro Individual de Prestación de Servicios de Salud (RIPS) from 2016 to 2024. chILD cases were identified using ICD-10 codes for chILD in individuals aged 0-18 years. Prevalence was estimated overall, by year, sex, age group, and diagnosis. Crude and WHO age-standardized prevalence rates were calculated per 100,000 population. Temporal trends were assessed using Poisson regression and LOESS smoothing.</p><p><strong>Results: </strong>A total of 4608 chILD -related healthcare encounters were identified. The average annual crude prevalence was 3.32 per 100,000 children (age-standardized: 3.39 per 100,000). Prevalence was highest in infants younger than 1 year and showed a secondary increase during adolescence. No relevant sex differences were observed. A significant annual increase in reported cases was detected (IRR 1.022; 95% CI 1.011-1.034), with a marked decline during the COVID-19 pandemic. Alveolar and fibrotic interstitial lung disease categories accounted for most cases.</p><p><strong>Conclusions: </strong>This first nationwide study provides robust real-world evidence on the epidemiology of chILD in Colombia, revealing substantial healthcare utilization and distinct age-related patterns. Administrative data represents a valuable complementary tool for surveillance of rare pediatric lung diseases.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 9","pages":"e71832"},"PeriodicalIF":2.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13572882/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148963097","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
A Case Report of a Novel LTBP4 Variant in Urban-Rifkin-Davis Syndrome Presenting With Lobar Overinflation in an Adolescent Girl. 青春期女孩Urban-Rifkin-Davis综合征中一种新型LTBP4变异伴大叶过度膨胀的病例报告。
IF 2.4 3区 医学
Pediatric Pulmonology Pub Date : 2026-08-01 DOI: 10.1002/ppul.71769
Kkomal C Suvarna, Kalyana Prabhakaran, Sathya Srivatsav, Taruna Yadav, Prawin Kumar, Jagdish Prasad Goyal
{"title":"A Case Report of a Novel LTBP4 Variant in Urban-Rifkin-Davis Syndrome Presenting With Lobar Overinflation in an Adolescent Girl.","authors":"Kkomal C Suvarna, Kalyana Prabhakaran, Sathya Srivatsav, Taruna Yadav, Prawin Kumar, Jagdish Prasad Goyal","doi":"10.1002/ppul.71769","DOIUrl":"10.1002/ppul.71769","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71769"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13439482/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148674092","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
Physiotherapy in Primary Ciliary Dyskinesia-What Is the Evidence?: A Narrative Review and Personal Experience. 物理治疗原发性纤毛运动障碍的证据是什么?:叙述回顾与个人经验。
IF 2.4 3区 医学
Pediatric Pulmonology Pub Date : 2026-08-01 DOI: 10.1002/ppul.71790
Elke Huenaerts, Myriam Vreys, François Vermeulen, Marijke Proesmans, Natalie Lorent, Mieke Boon
{"title":"Physiotherapy in Primary Ciliary Dyskinesia-What Is the Evidence?: A Narrative Review and Personal Experience.","authors":"Elke Huenaerts, Myriam Vreys, François Vermeulen, Marijke Proesmans, Natalie Lorent, Mieke Boon","doi":"10.1002/ppul.71790","DOIUrl":"10.1002/ppul.71790","url":null,"abstract":"<p><p>Primary ciliary dyskinesia (PCD) is a rare, hereditary disorder characterized by impaired motile ciliary function, resulting in abnormal mucociliary clearance (MCC). This leads to persistent infection and inflammation, progressive airway damage, and bronchiectasis. Physiotherapy, particularly airway clearance therapy (ACT), is considered a cornerstone of PCD management to compensate for the mechanical MCC deficit, aiming to reduce infection risk, prevent disease progression, and improve quality of life (QOL). However, evidence supporting specific physiotherapy regimens in PCD remains limited. Current practice varies widely across centers and countries, with no standardized guidelines. Common ACT methods include positive expiratory pressure (PEP) with or without oscillation, autogenic and postural drainage, active cycle of breathing technique (ACBT), intrapulmonary percussive ventilation (IPV), and high-frequency chest wall oscillation (HFCWO). Comparative studies show no clear superiority among techniques. Evidence for mucolytic therapy, such as hypertonic saline or rhDNAse, is weak or controversial. Scarce data demonstrate potential benefits of exercise interventions in improving ventilatory capacity and QOL. Age-specific considerations are crucial: ACT should be started early, be adapted to developmental stages, and be combined with strategies to enhance adherence. Despite physiotherapy being standard care, randomized controlled trials are challenging due to ethical constraints, potentially reducing a therapy that is already believed to be helpful. Future priorities include developing international guidelines and conducting high-quality studies to optimize treatment strategies. In conclusion, physiotherapy plays a crucial role in PCD management, but current recommendations rely largely on expert opinion and extrapolation from related conditions like cystic fibrosis (CF) and non-CF bronchiectasis (BRECT), underscoring the need for robust evidence.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71790"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13477620/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148759534","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
Effectiveness of Continuous Levalbuterol Versus Albuterol During a National Shortage: A Retrospective Observational Cohort Study. 在全国短缺期间持续使用左旋布特罗与沙丁胺醇的有效性:一项回顾性观察队列研究。
IF 2.4 3区 医学
Pediatric Pulmonology Pub Date : 2026-08-01 DOI: 10.1002/ppul.71779
Allyson Tank, Layne Silver, Michael C Spaeder, Colin Rogerson, Alice Shanklin
{"title":"Effectiveness of Continuous Levalbuterol Versus Albuterol During a National Shortage: A Retrospective Observational Cohort Study.","authors":"Allyson Tank, Layne Silver, Michael C Spaeder, Colin Rogerson, Alice Shanklin","doi":"10.1002/ppul.71779","DOIUrl":"10.1002/ppul.71779","url":null,"abstract":"<p><strong>Introduction: </strong>Bronchospasm requiring continuous inhaled albuterol is a common reason for pediatric intensive care unit (PICU) admission. Albuterol's racemic composition may contribute to adverse effects; therefore, levalbuterol [(R)-albuterol] may be used. This study aimed to determine if continuous inhaled levalbuterol is associated with a shorter duration of continuous inhaled bronchodilator therapy and less tachycardia compared to albuterol in PICU patients.</p><p><strong>Methods: </strong>This study utilized two periods of exclusive continuous inhaled levalbuterol use due to an albuterol shortage. PICU encounters receiving ≥ 12 h of continuous inhaled levalbuterol during those periods were compared to encounters receiving albuterol in the interim. Therapy duration and % change in median heart rate (HR) at 6-h epochs were compared between groups, and multilevel mixed effects models were created.</p><p><strong>Results: </strong>In 686 encounters, there was no difference in therapy duration between levalbuterol and albuterol groups (31.3 vs. 28.7 h, p = 0.22). There was no difference in % change in median HR at 6 (-1.0% vs. -1.3%, p = 0.54), 12 (-1.6% vs. -2.7%, p = 0.28), or 18 h (-2.5% vs. -4.1%, p = 0.38) from therapy initiation. Levalbuterol was not associated with therapy duration adjusting for age, technology dependence, respiratory rate category, and number of continuous intravenous bronchodilators (p = 0.13). Levalbuterol was not associated with % change in median HR at 12 h following therapy initiation after adjusting for age and respiratory rate category (p = 0.99).</p><p><strong>Conclusion: </strong>Continuous inhaled levalbuterol was not associated with shorter therapy duration or lower HR compared to albuterol in PICU patients.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71779"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13448360/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148685432","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
Sleep-Disordered Breathing in Primary Ciliary Dyskinesia. 原发性纤毛运动障碍的睡眠呼吸障碍。
IF 2.4 3区 医学
Pediatric Pulmonology Pub Date : 2026-08-01 DOI: 10.1002/ppul.71783
Merve Selçuk Balcı, Ceren Ayça Yıldız, Ela Erdem Eralp, Refika Ersu
{"title":"Sleep-Disordered Breathing in Primary Ciliary Dyskinesia.","authors":"Merve Selçuk Balcı, Ceren Ayça Yıldız, Ela Erdem Eralp, Refika Ersu","doi":"10.1002/ppul.71783","DOIUrl":"10.1002/ppul.71783","url":null,"abstract":"<p><strong>Background: </strong>Primary Ciliary Dyskinesia (PCD) is a rare genetic disorder marked by impaired mucociliary clearance, chronic upper and lower airway infections, and progressive bronchiectasis. Sleep-disordered breathing (SDB) has emerged as an under-recognized comorbidity in this population.</p><p><strong>Objective: </strong>This review synthesizes current evidence on the prevalence, pathophysiology, diagnostic approaches, and management of SDB in PCD across pediatric and adult cohorts.</p><p><strong>Results: </strong>Reported prevalence of obstructive sleep apnea (OSA) in PCD ranges widely from ~33% to 100%, markedly exceeding general population norms; however, one large recent pediatric cohort reported a much lower prevalence (~1.2%), indicating that true disease magnitude remains uncertain. Upper-airway abnormalities (chronic rhinosinusitis, nasal polyposis, adeno-tonsillar hypertrophy) are almost universal in PCD, while lower-airway disease and ventilation-perfusion mismatch contribute to nocturnal hypoxemia even in the absence of apneas. Polysomnography (PSG) remains the diagnostic gold standard; subjective questionnaires (e.g., PSQI, SDSC, ESS) show poor correlation with PSG metrics in PCD. Sleep architecture is typically preserved, but sleep efficiency is mildly reduced and arousal indices are elevated, indicative of fragmented yet largely restorative sleep. Psychological and neurocognitive sequelae, including anxiety, depressive symptoms, and attention deficits, may be seen and are associated with SDB in PCD. First-line management focuses on optimization of the upper-airway (adenotonsillectomy, nasal irrigation, intranasal corticosteroids), while residual SDB may necessitate positive airway pressure (PAP) therapy. Optimizing lung function and infection control may further improve nocturnaloxygenation.</p><p><strong>Conclusions: </strong>SDB in PCD represents a multifactorial and potentially modifiable contributor to disease burden. Incorporating sleep assessment into routine PCD care and fostering multicenter, genotype-informed research are imperative to enhance early recognition, management, and quality-of-life outcomes.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71783"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13470827/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148726998","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
Utilization of Des-Gamma-Carboxy-Prothrombin in Estimating Vitamin K Status in People With Cystic Fibrosis: A Single Center Retrospective Review. 利用des - γ -羧基凝血酶原估计囊性纤维化患者维生素K水平:一项单中心回顾性研究
IF 2.4 3区 医学
Pediatric Pulmonology Pub Date : 2026-08-01 DOI: 10.1002/ppul.71777
Senthilkumar Sankararaman, Terri Schindler, Aravind Thavamani, Catherine Pamer, Alison Marie Rodatt, Kimberly A McBennett, Erica Roesch, Alex H Gifford
{"title":"Utilization of Des-Gamma-Carboxy-Prothrombin in Estimating Vitamin K Status in People With Cystic Fibrosis: A Single Center Retrospective Review.","authors":"Senthilkumar Sankararaman, Terri Schindler, Aravind Thavamani, Catherine Pamer, Alison Marie Rodatt, Kimberly A McBennett, Erica Roesch, Alex H Gifford","doi":"10.1002/ppul.71777","DOIUrl":"10.1002/ppul.71777","url":null,"abstract":"<p><strong>Background: </strong>People with cystic fibrosis (CF) are at increased risk of fat-soluble vitamin (A, D, E, and K) deficiencies secondary to exocrine pancreatic insufficiency (EPI). Estimation of vitamin K deficiency in routine clinical practice is challenging, and no uniform consensus exists due to lack of an accurate clinical test for the evaluation of its status. We aimed to estimate vitamin K status in people with CF using des-gamma-carboxy-prothrombin (DCP).</p><p><strong>Methodology: </strong>A single-center retrospective chart review was done between 2022 and 2024. Ninety-four (39 pediatric and 55 adult) people with CF were included for final review.</p><p><strong>Results: </strong>The median DCP value was 0.35 ng/mL (IQR 0.2-0.60; range was 0.1-11 ng/mL). Post hoc, we classified patients into low-DCP (0-90th percentile values in DCP or ≤ 2 ng/mL) and high-DCP (91-100th percentile in DCP or >2 ng/mL) groups. Eleven out of 94 (11.7%) patients had DCP > 2 ng/mL; three out of 11 (27%) had advanced CF liver disease (aCFLD), p = 0.011, and another three had other fat-soluble vitamin deficiencies, and low adherence to pancreatic enzymes and/or vitamin supplementation was suspected, p = 0.011. There were no significant differences in demographics (age, gender) or other comorbidities between the groups.</p><p><strong>Conclusion: </strong>DCP can be used for estimating vitamin K status, and the prevalence of suboptimal vitamin K status was 11.7%. DCP levels were higher in aCFLD, as well as those with suspected low adherence to pancreatic enzymes and/or vitamin supplementation. Prospective, multicenter clinical trials are needed to evaluate this further.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 8","pages":"e71777"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13462822/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148712403","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
Efficacy of High-Dose Versus Conventional-Dose Methylprednisolone in Reducing the Risk of Chronic Lung Disease in Chinese Children With Severe Mycoplasma pneumoniae Pneumonia: A Retrospective Study. 大剂量甲基强的松龙与常规剂量甲基强的松龙降低中国重症肺炎支原体肺炎儿童慢性肺部疾病风险的疗效:一项回顾性研究
IF 2.4 3区 医学
Pediatric Pulmonology Pub Date : 2026-08-01 DOI: 10.1002/ppul.71762
Yanyan Su, Yanjuan Yu, Lei Zhang, Yu Tang, Min Li, Chaohui Chen, Shasha Xu, Yanqiong Wang, Yuemei Liu, Haiming Yang, Yuelin Shen
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