Pediatric Pulmonology最新文献

Childhood Tuberculosis-Advances in Treatment and Prevention. 儿童结核病——治疗和预防的进展。

IF 2.7 3区医学

Pediatric Pulmonology Pub Date : 2025-01-07 DOI: 10.1002/ppul.27375

Sandra Kwarteng Owusu

引用次数: 0

Reconsidering the Diagnosis: Abnormal Sweat Chloride Tests in Non-CF Bronchiectasis. 重新考虑诊断：非cf支气管扩张异常的汗液氯化物试验。

IF 2.7 3区医学

Pediatric Pulmonology Pub Date : 2025-01-07 DOI: 10.1002/ppul.27471

Reyna L Huang, Matthew T Snyder, Nuzhat Fahmida, Dana P Albon

{"title":"Reconsidering the Diagnosis: Abnormal Sweat Chloride Tests in Non-CF Bronchiectasis.","authors":"Reyna L Huang, Matthew T Snyder, Nuzhat Fahmida, Dana P Albon","doi":"10.1002/ppul.27471","DOIUrl":"https://doi.org/10.1002/ppul.27471","url":null,"abstract":"Introduction: While the diagnosis of cystic fibrosis (CF) is often straightforward and reliant on correlation between genetic testing and clinical signs and symptoms, there is a subset where the distinction is not nearly as clearcut. This has previously been reported in patients identified through newborn screening but not meeting full CF diagnostic criteria, earning the label of CF Screen Positive, Inconclusive Diagnosis (CFSPID) instead. A homologous diagnostic category in adults is named CF Transmembrane Conductance Regulator-Related Disorder (CFTR-RD).Methods: Through a retrospective chart review, this study reports on a relatively large adult cohort (n = 23) that presented to pulmonology clinic at a single center with intermediate or positive sweat chloride tests but non-diagnostic full CFTR gene analysis.Results: Median sweat chloride result was 48 mmol/L, and a majority of the cohort had chronic lung disease with atypical pathogens on sputum culture, including Pseudomonas aeruginosa, non-tuberculous Mycobacteria, Acinetobacter species, amongst others.Conclusions: This clinical picture suggests CFTR dysfunction or similar mechanism in the absence of an identified genetic cause. Alternate chloride channels and their respective genes or candidates of genetic modifiers to the CF-phenotype could be targets of further research in this cohort or similar patients. Such genetic modifiers include loci that have been implicated in inflammation, the CFTR interactome, and/or co-/post-translational modification of CFTR.","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":" ","pages":"e27471"},"PeriodicalIF":2.7,"publicationDate":"2025-01-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142953069","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

Sickle Lung Disease Long-Term Consequences and Prevention. 镰状肺疾病的长期后果和预防。

IF 2.7 3区医学

Pediatric Pulmonology Pub Date : 2025-01-07 DOI: 10.1002/ppul.27374

Sandra Kwarteng Owusu

引用次数: 0

Growth, Body Composition, and Strength of Children With Cystic Fibrosis Treated With Elexacaftor/Tezacaftor/Ivacaftor (ETI). elexaftor /Tezacaftor/Ivacaftor （ETI）治疗囊性纤维化儿童的生长、身体组成和力量

IF 2.7 3区医学

Pediatric Pulmonology Pub Date : 2025-01-06 DOI: 10.1002/ppul.27463

Thomas Boat, Md Monir Hossain, Aisaku Nakamura, Michelle Hjelm, William Hardie, Matthew Wackler, Alyssa Amato, Carolyn Dress

{"title":"Growth, Body Composition, and Strength of Children With Cystic Fibrosis Treated With Elexacaftor/Tezacaftor/Ivacaftor (ETI).","authors":"Thomas Boat, Md Monir Hossain, Aisaku Nakamura, Michelle Hjelm, William Hardie, Matthew Wackler, Alyssa Amato, Carolyn Dress","doi":"10.1002/ppul.27463","DOIUrl":"https://doi.org/10.1002/ppul.27463","url":null,"abstract":"Objective: We prospectively monitored rates of change for growth, body mass and composition, muscle strength, and FEV1 in 6-11-year-olds initiating ETI therapy, comparing them to those of US reference children. We assessed factors potentially contributing to rate of change and report ranges of individual variation.Methods: Body composition was assessed using bioelectrical impedance analysis (BIA), and rates of change were analyzed using linear mixed effects regression models.Results: At enrollment, median BMI-Z was 0.6 (IQR: -0.2, 1.1), percent body fat (PBF) was 22.7 (14.0, 31.5), and percent predicted(pp) FEV1 was 100 (90, 106). During ETI treatment, mean Z scores for annualized change rates of BMI (0.02 ± 0.07; p = 0.74), FMI (0.02 ± 0.08; p = 0.76), and FFMI (-0.03 ± 0.07; p = 0.68) were not different from zero. The most rapid weight gain occurred in girls (p = 0.01), 10-11-year-olds (p < 0.001), and those previously treated with a modulator (p = 0.005). Individual rates of change varied widely; PBF increased for 15 children (range: 0.7 to 10.0) and decreased for 12 (range: -0.7 to -9.5). Changes in body mass and composition were not significantly associated with changes in ppFEV1; regression coefficients were positive for FFMI (0.83) and SMMI (1.07) and negative for FMI (-0.29).Conclusion: Healthy, well-nourished children with CF, as a group, experienced growth and body composition changes similar to those of US children, added muscle mass, and often added more FM than FFM during ETI therapy. Individual variation underscores the need for body composition monitoring and interventions that promote healthy physical maturation for all during ETI therapy.","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":" ","pages":"e27463"},"PeriodicalIF":2.7,"publicationDate":"2025-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142932488","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 laryngeal cysts mimicking laryngomalacia. 模仿喉瘤的先天性喉囊肿。

IF 2.7 3区医学

Pediatric Pulmonology Pub Date : 2025-01-01 Epub Date: 2024-09-27 DOI: 10.1002/ppul.27298

Lara Merino-Mateo, Rocío Morante, María López, Ignacio Jiménez, Juan L Antón-Pacheco

引用次数: 0

Outpatient clinical care for bronchopulmonary dysplasia: A survey of the BPD collaborative. 支气管肺发育不良的门诊临床护理：支气管肺发育不良协作组调查。

IF 2.7 3区医学

Pediatric Pulmonology Pub Date : 2025-01-01 Epub Date: 2024-10-11 DOI: 10.1002/ppul.27296

Paul E Moore, Lystra P Hayden, Natalie M Villafranco, Demet Toprak, Jessica L Rice, Lawrence M Rhein, Antonia P Popova, Robin L McKinney, Winston M Manimtim, Jonathan C Levin, Khanh V Lai, Susan C Gage, Manvi Bansal, Christopher D Baker, Eric D Austin, Amit Agarwal, Sharon A McGrath-Morrow, Joseph M Collaco

{"title":"Outpatient clinical care for bronchopulmonary dysplasia: A survey of the BPD collaborative.","authors":"Paul E Moore, Lystra P Hayden, Natalie M Villafranco, Demet Toprak, Jessica L Rice, Lawrence M Rhein, Antonia P Popova, Robin L McKinney, Winston M Manimtim, Jonathan C Levin, Khanh V Lai, Susan C Gage, Manvi Bansal, Christopher D Baker, Eric D Austin, Amit Agarwal, Sharon A McGrath-Morrow, Joseph M Collaco","doi":"10.1002/ppul.27296","DOIUrl":"10.1002/ppul.27296","url":null,"abstract":"Background: Bronchopulmonary dysplasia, a sequela of preterm birth, is the most common chronic respiratory disorder in infancy, and the second most common in children. Despite this, clinical care remains highly variable with guidelines supported by limited evidence, and do not provide specific guidance for timing of clinical follow-up, echocardiography, modalities of pulmonary function testing, etc. OBJECTIVE/METHODS: To further our understanding of care delivery for BPD, we sought to describe outpatient care patterns at tertiary care centers through survey data from 27 well-established BPD programs.Results: We observed variability in referral patterns to outpatient BPD clinics, ancillary services provided, indications for follow-up echocardiograms, availability of lung function testing, and criteria for discharge from care.Conclusion: More comprehensive and detailed clinical guidelines similar to other pulmonary diseases such as asthma and cystic fibrosis should be developed to help standardize care and may improve long term outcomes.","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":" ","pages":"e27296"},"PeriodicalIF":2.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142400955","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

LARGE primary spontaneous pneumothorax (2023 French Guidelines definition): Do radiographic size matter in pediatric patients? A comparative analysis. 巨大原发性自发性气胸（2023 年法国指南定义）：儿科患者的影像学尺寸是否重要？对比分析。

IF 2.7 3区医学

Pediatric Pulmonology Pub Date : 2025-01-01 Epub Date: 2024-11-27 DOI: 10.1002/ppul.27290

Angelo Zarfati, Giovanni Rollo, Simone Frediani, Valerio Pardi, Ivan Pietro Aloi, Arianna Bertocchini, Antonella Accinni, Alessandro Inserra

{"title":"LARGE primary spontaneous pneumothorax (2023 French Guidelines definition): Do radiographic size matter in pediatric patients? A comparative analysis.","authors":"Angelo Zarfati, Giovanni Rollo, Simone Frediani, Valerio Pardi, Ivan Pietro Aloi, Arianna Bertocchini, Antonella Accinni, Alessandro Inserra","doi":"10.1002/ppul.27290","DOIUrl":"10.1002/ppul.27290","url":null,"abstract":"Purpose: Our aim was to explore the applicability of the definition of \"large\" primary spontaneous pneumothorax (PSP) of adult guidelines to pediatric patients.Methods: We performed a retrospective monocentric analysis of first-episodes of PSP (Period: January 2010-January 2023). We defined large PSP according to French SPLF/SMFU/SRLF/SFAR/SFCTCV Guidelines.Inclusion criteria: age 1-18 years-old, follow-up ≥ 1 year. We performed a comparative analysis between large and nonlarge PSP. We reported the categorical variables as frequencies, and continuous as median and range. We compared categorical variables using Chi-Square or Fisher test, and continuous using U Mann-Whitney test. p < 0.05 was considered significant.Results: The population consisted of 49 patients, 15 (30%) with large PSP. We found no significant differences at presentation between large and nonlarge PSP regarding age, sex, side, BMI, oxygen saturation, respiratory rate and hearth rate. Conservative first-line management was used significantly less for large PSP (26% vs. 82%, p = 0.0003), while drain more (46% vs. 8%, p = 0.005). The groups were similar regarding surgery. Large PSP had a significantly longer hospitalization (7 days vs. 4 days, p = 0.003). The incidence of first-line management failure was similar between the groups. During the follow-up, we found no significant differences regarding ipsilateral recurrence (33% vs. 17%, p = 0.275), and contralateral occurrence (0% vs. 5%, p = 1.000), need for drain (6% vs. 5%, p = 1.000), or surgery (33% vs. 14%, p = 0.246).Conclusions: In the first-line, large PSP were treated significantly less conservatively and more drained. Radiographic size of the pneumothorax does not seem to have an impact on first-line treatment failure and follow-up outcomes.","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":" ","pages":"e27290"},"PeriodicalIF":2.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142731679","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

Inhaled corticosteroid delivery is markedly affected by breathing pattern and valved holding chamber model. 吸入皮质类固醇的给药量明显受到呼吸模式和瓣膜保持室模型的影响。

IF 2.7 3区医学

Pediatric Pulmonology Pub Date : 2025-01-01 Epub Date: 2024-09-30 DOI: 10.1002/ppul.27300

Laura Ojanperä, Lauri Lehtimäki, Heini Huhtala, Péter Csonka

{"title":"Inhaled corticosteroid delivery is markedly affected by breathing pattern and valved holding chamber model.","authors":"Laura Ojanperä, Lauri Lehtimäki, Heini Huhtala, Péter Csonka","doi":"10.1002/ppul.27300","DOIUrl":"10.1002/ppul.27300","url":null,"abstract":"Background: There is a scarcity of high-quality research on the efficient delivery of inhaled corticosteroids using valved holding chambers (VHCs) in children.Methods: The delivered dose (DD) of fluticasone from a metered dose inhaler (pMDI) was tested using four VHCs: AeroChamber plus Flow-Vu (AC), Babyhaler (BH), EasyChamber (EC), and Optichamber Diamond (OD). The in vitro setup included an anatomical child throat model, Next Generation Impactor, and a breathing simulator to generate tidal breathing of a four and a 6-year-old child, and adult type single inhalation.Results: OD showed the lowest proportion of fluticasone trapped in the throat with all breathing patterns. AC showed similar fine particle dose (FPD) in the respirable range (1-5 µm) irrespective of the breathing pattern. For BH, the median FPD 1-5 µm was highest during adult breathing. OD and EC showed higher overall DD and higher doses in the 1-5 µm range with paediatric breathing profiles compared to adult inhalation. The median DD and FPD 1-5 µm were significantly lower with BH compared to any other VHCs during tidal breathing. Compared to EC, the FPD of the other VHCs were skewed towards <2 µm particles.Conclusion: Fluticasone delivery is markedly affected by breathing pattern and VHC model. The observed differences in throat deposition and FPD delivered may have significant clinical implications for side effects and controlling airway inflammation. All VHCs intended for paediatric use should undergo testing using internationally recognised standardised methods incorporating clinically relevant paediatric breathing patterns.","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":" ","pages":"e27300"},"PeriodicalIF":2.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11715152/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142351752","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

Successful Management of Pediatric Endobronchial Tuberculosis With Endoscopic Cryotherapy: A Case Series. 内镜冷冻治疗成功治疗小儿支气管结核：一个病例系列。

IF 2.7 3区医学

Pediatric Pulmonology Pub Date : 2025-01-01 Epub Date: 2024-12-26 DOI: 10.1002/ppul.27444

Enrique G Villarreal, Anita Nagy, Ray Lam, Valerie Waters, Ian Kitai, Fiona Kritzinger

引用次数: 0

Facilitators and Barriers to Increasing Equity in Cystic Fibrosis Newborn Screening Algorithms. 提高囊性纤维化新生儿筛查算法公平性的促进因素和障碍。

IF 2.7 3区医学

Pediatric Pulmonology Pub Date : 2025-01-01 Epub Date: 2024-12-20 DOI: 10.1002/ppul.27449

Kellyn Madden, Rebecca Mueller, Camille Brown, Kathleen D Valverde, Elinor Langfelder-Schwind

{"title":"Facilitators and Barriers to Increasing Equity in Cystic Fibrosis Newborn Screening Algorithms.","authors":"Kellyn Madden, Rebecca Mueller, Camille Brown, Kathleen D Valverde, Elinor Langfelder-Schwind","doi":"10.1002/ppul.27449","DOIUrl":"10.1002/ppul.27449","url":null,"abstract":"Background: Newborn screening (NBS) for cystic fibrosis (CF) was universally implemented in the United States in 2010 to improve disease outcomes. Despite universal screening, disparities in outcomes currently exist between people with CF (PwCF) with Black/African, Asian, Indigenous, and Latino/Hispanic ancestry in comparison to PwCF of European ancestry. This is in part because CFTR panels used for newborn screening are often based on variants common in European ancestries leading to higher rates of false negatives for PwCF from minoritized racial and ethnic groups.Methods: This study investigated how states evaluate and update their CFNBS algorithms through semi-structured interviews with professionals from four states with ethnically diverse populations and one national consultant. Interviews were transcribed verbatim and analyzed through inductive thematic analysis.Results: Five themes were identified encompassing facilitators, barriers, and motivations for evaluating and updating CF NBS algorithms. Facilitators of effective evaluation and updating of algorithms included effective communication with CF clinical centers and extensive support for CF as compared to other conditions. Although participants stated that their respective NBS programs were aware of the disparate impact of their CF panels on PwCF from minoritized racial and ethnic groups, motivations to decrease this disparity were hampered by a range of funding and logistical barriers, such as limited information about false negative cases and difficulties incorporating next generation sequencing technology.Conclusions: This study shed light on the experiences of states considering alterations to their CFNBS panels, revealing several key barriers and facilitators to implementing equitable CFNBS algorithms.","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":" ","pages":"e27449"},"PeriodicalIF":2.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142864994","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