Pediatric Pulmonology最新文献_第5页

Congenital Diaphragmatic Hernia Patch Infection Presenting With Digital Clubbing. 先天性膈疝补片感染表现为指征。

IF 2.3 3区医学

Pediatric Pulmonology Pub Date : 2025-09-01 DOI: 10.1002/ppul.71263

Alexander I Gipsman, Mark N Blanke, Edward R Oliver, Holly L Hedrick, Lisa M Herkert, Howard B Panitch

引用次数: 0

An Optimal Initial Ventilation Mode in Congenital Diaphragmatic Hernia: A Prospective Cohort Study. 先天性膈疝最佳初始通气模式：一项前瞻性队列研究。

IF 2.3 3区医学

Pediatric Pulmonology Pub Date : 2025-09-01 DOI: 10.1002/ppul.71316

Miharu Ito, Satoko Ohfuji, Keita Terui, Kouji Nagata, Noriaki Usui, Yoshiaki Sato, Shoichiro Amari, Hidehiko Maruyama, Kouji Masumoto, Yuichiro Miyake, Tadaharu Okazaki, Yunosuke Kawaguchi, Katsuaki Toyoshima, Masaya Yamoto, Kiyokazu Kim, Noboru Inamura, Yuhki Koike, Akiko Yokoi, Hiroomi Okuyama

{"title":"An Optimal Initial Ventilation Mode in Congenital Diaphragmatic Hernia: A Prospective Cohort Study.","authors":"Miharu Ito, Satoko Ohfuji, Keita Terui, Kouji Nagata, Noriaki Usui, Yoshiaki Sato, Shoichiro Amari, Hidehiko Maruyama, Kouji Masumoto, Yuichiro Miyake, Tadaharu Okazaki, Yunosuke Kawaguchi, Katsuaki Toyoshima, Masaya Yamoto, Kiyokazu Kim, Noboru Inamura, Yuhki Koike, Akiko Yokoi, Hiroomi Okuyama","doi":"10.1002/ppul.71316","DOIUrl":"10.1002/ppul.71316","url":null,"abstract":"Objectives: Respiratory management is crucial for patients with congenital diaphragmatic hernia (CDH). This prospective study aimed to assess the optimal initial ventilatory mode for CDH by comparing conventional mechanical ventilation (CMV) and high-frequency oscillatory ventilation (HFO).Methods: Fifteen participating institutes used CMV or HFO as the initial ventilation mode for CDH, we thus divided them into two groups (CMV and HFO). Clinical data were prospectively collected from patients with isolated left CDH born at a gestational age ≥ 34 weeks between 2017 and 2021. The primary outcome was mortality to discharge, with non-inferiority analysis. We also performed analyses stratified by disease severity.Results: Of the 247 patients, 124 received CMV, and 123 received HFO. The mortality rates for CMV and HFO were 11.3% and 15.4%, respectively, which met the definition of non-inferiority. We observed no significant differences in BPD (CMV, 41% vs. HFO, 42%) or the combined outcome of mortality or BPD (48% vs. 51%). Among patients with mild and moderate disease, CMV was associated with less frequent switching of the ventilation mode, shorter mechanical ventilation time, lower use of inhaled nitric oxide (iNO) therapy, and a shorter duration of iNO. In severe cases, HFO was associated with less frequent switching of the ventilation mode and a reduced need for ECMO.Conclusions: The initial ventilation mode in CDH patients does not influence mortality or BPD outcomes. However, this study suggests that CMV may be more beneficial for mild-to-moderate cases, while HFO may be more advantageous for severe cases.","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 9","pages":"e71316"},"PeriodicalIF":2.3,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145186477","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

Genetic Characterization of Primary Ciliary Dyskinesia in a Consanguineous Population: Insights From the Largest Middle Eastern Cohort. 近亲人群中原发性纤毛运动障碍的遗传特征：来自最大中东队列的见解。

IF 2.3 3区医学

Pediatric Pulmonology Pub Date : 2025-09-01 DOI: 10.1002/ppul.71297

Kawther Al Adawi, Aliya Al Ansari, Nawal Al Shamli, Khoula Al Shidhani, Ahmed Qoura, Saif Al Mubaihsi, Jamal Al-Aghbari, Taher Baomar, Hussein Al Kindi, Almundher Al-Maawali

{"title":"Genetic Characterization of Primary Ciliary Dyskinesia in a Consanguineous Population: Insights From the Largest Middle Eastern Cohort.","authors":"Kawther Al Adawi, Aliya Al Ansari, Nawal Al Shamli, Khoula Al Shidhani, Ahmed Qoura, Saif Al Mubaihsi, Jamal Al-Aghbari, Taher Baomar, Hussein Al Kindi, Almundher Al-Maawali","doi":"10.1002/ppul.71297","DOIUrl":"10.1002/ppul.71297","url":null,"abstract":"Background: Primary Ciliary Dyskinesia (PCD) is a Mendelian disorder most commonly inherited in an autosomal recessive pattern. It impairs the mucociliary clearance in the respiratory system. As genetic variants linked to this disorder continue to be identified, genetic testing becomes more accessible. This study aimed to characterize genetic variants associated with PCD in a consanguineous population.Methods: Medical records of 327 affected individuals from 242 families, all strongly suspected of having PCD, were reviewed. All patients exhibited at least two of the four main clinical symptoms of PCD: unexplained neonatal respiratory distress in term infants, year-round daily cough since infancy, year-round nasal congestion since infancy, or organ laterality defects.Results: Out of the cohort, 130 affected individuals from 117 families underwent genetic testing, which included targeted Sanger sequencing, exome sequencing, or panel sequencing. The genetic diagnostic rate was 33%, with 43 of these 130 patients from 24 families receiving a molecular diagnosis. Electron microscopy (EM) revealed ciliary ultrastructural defects in 13 of the 43 patients, which correlated with their genetic variants.Conclusions: This represents the largest cohort of PCD patients from the Middle East. The findings highlight the genetic diversity of PCD and underscore the importance of genetic testing for diagnosis. We report novel variants and contribute to the growing understanding of the genetic causes of PCD. Early genetic diagnosis can significantly influence timely management, helping to prevent lung damage and other complications associated with this challenging disease.Clinical trial number: Not applicable.","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 9","pages":"e71297"},"PeriodicalIF":2.3,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145070094","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

Detecting Impaired Lung Growth Using the Conditional Change Score of FEV₁ in an Open Cohort of Children With Asthma. 在哮喘儿童开放队列中使用FEV1条件变化评分检测肺生长受损

IF 2.3 3区医学

Pediatric Pulmonology Pub Date : 2025-09-01 DOI: 10.1002/ppul.71295

Bruno Mahut, Plamen Bokov, Nicole Beydon, Christophe Delclaux

{"title":"Detecting Impaired Lung Growth Using the Conditional Change Score of FEV1 in an Open Cohort of Children With Asthma.","authors":"Bruno Mahut, Plamen Bokov, Nicole Beydon, Christophe Delclaux","doi":"10.1002/ppul.71295","DOIUrl":"10.1002/ppul.71295","url":null,"abstract":"Background: It has been suggested that a quarter of children with asthma show impaired lung growth, with better initial function as a risk factor. A conditional change score (CCS) for FEV1 that is independent of the initial FEV1 and that takes into account the time interval between the measurements has been proposed, a value < -1.96 being predictive of impaired growth.Objective: To evaluate whether the use of the CCS allows the identification of a subgroup of children with asthma that has an impaired lung growth, and to identify the risk factors of impaired growth.Methods: We reanalyzed the data of 295 children with confirmed asthma (199 boys) who had undergone at least 10 spirometry tests from the age of 8 who were selected from a single-center open cohort. The annualized rate of change (slope) for prebronchodilator FEV1 (percent predicted) was estimated for each participant. Using the first and last visit of follow-up, the CCS was calculated.Results: In total, 46 children (16%, 95% confidence interval: 12-20) followed up for a median duration of 6.5 years [interquartile: 5.7-7.3] exhibited an impaired lung growth. The CCS correlated with the standard deviation of individual slopes (FEV1 variability criterion: R = 0.21; p < 0.001), the z-score of FEV1 at first visit (R = -0.22; p < 0.001), and the body mass index at final visit (R = 0.16; p = 0.005), which remained independently associated with the CCS.Conclusion: We confirm that some children with asthma (16%) show impaired lung growth with better initial lung function and decreased FEV1 variability as risk factors.","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 9","pages":"e71295"},"PeriodicalIF":2.3,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12439321/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145070157","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

Persistence of Viable But Non-Culturable Stenotrophomonas maltophilia in a Cystic Fibrosis Patient. 囊性纤维化患者存活但不可培养的嗜麦芽寡养单胞菌的持续存在。

IF 2.3 3区医学

Pediatric Pulmonology Pub Date : 2025-09-01 DOI: 10.1002/ppul.71300

Gianmarco Mangiaterra, Valentina Schiavoni, Nicholas Cedraro, Barbara Citterio, Carla Vignaroli, Rosaria Gesuita, Benedetta Fabrizzi, Francesca Biavasco, Natalia Cirilli

引用次数: 0

Airway Disease Progression on Chest Computed Tomography in Children With Primary Ciliary Dyskinesia. 原发性纤毛运动障碍儿童胸部计算机断层扫描显示气道疾病进展。

IF 2.3 3区医学

Pediatric Pulmonology Pub Date : 2025-09-01 DOI: 10.1002/ppul.71287

BreAnna Kinghorn, Federico Mollica, Daan Caudri, Stephanie D Davis, Sharon Dell, Thomas W Ferkol, Robert Andrew Gardner, Michael R Knowles, Carlos Milla, Jessica E Pittman, Margaret Rosenfeld, Scott D Sagel, Adam J Shapiro, Erin Sullivan, Kelli M Sullivan, Harm A W M Tiddens, Maimoona A Zariwala, Margaret W Leigh

{"title":"Airway Disease Progression on Chest Computed Tomography in Children With Primary Ciliary Dyskinesia.","authors":"BreAnna Kinghorn, Federico Mollica, Daan Caudri, Stephanie D Davis, Sharon Dell, Thomas W Ferkol, Robert Andrew Gardner, Michael R Knowles, Carlos Milla, Jessica E Pittman, Margaret Rosenfeld, Scott D Sagel, Adam J Shapiro, Erin Sullivan, Kelli M Sullivan, Harm A W M Tiddens, Maimoona A Zariwala, Margaret W Leigh","doi":"10.1002/ppul.71287","DOIUrl":"10.1002/ppul.71287","url":null,"abstract":"Rationale: The longitudinal trajectory of airway disease on chest computed tomography (CT) and risk factors for progression in primary ciliary dyskinesia (PCD) is largely unknown.Methods: Longitudinal analysis of structural airway disease among children with PCD enrolled in a prospective, multicenter observational study, stratified by ultrastructural defect group. CTs were scored utilizing the Melbourne-Rotterdam Annotated Grid Morphometric Analysis for PCD, evaluating airway abnormalities in a hierarchical order: atelectasis, bronchiectasis (%BE), airway wall thickening, and mucus plugging/tree in bud opacities (%MP). Volume fraction of each component was expressed as % of total lung volume. %Disease (%DIS) is the sum of all components. Mixed effects regression analyses were utilized to describe the association between clinical predictors and CT scores adjusted for relevant covariates.Results: Chest CTs (N = 424) were obtained from 142 children over a mean (SD) of 4.9 (3.7) years. Mean (SD) age at first CT was 8.5 (4.6) years. Mean %DIS increased with age for the cohort (0.09% per year, 95% CI: 0.02-0.15, p < 0.01), driven by increased %BE (0.06 higher per year, 95% CI: 0.04-0.08, p = 0.0001) and increased odds of MP (1.14 higher odds per year, 95% CI: 1.08-1.21, p < 0.0001). Children with inner dynein arm and microtubular defects had increased mean %DIS and increased odds of MP compared to other ciliary abnormalities (1.73% higher, 95% CI: 0.71-2.74, p = 0.001; 3.21 higher odds per year, 95% CI: 1.73-5.95, p < 0.001). Annual increase in CT scores did not differ between defect groups.Conclusions: Children with PCD experience progressive bronchiectasis and worsening mucus plugging with age.","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 9","pages":"e71287"},"PeriodicalIF":2.3,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145081317","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

Beyond the Usual Suspects: Neonatal Fever and Desaturation. 超出通常的怀疑：新生儿发烧和去饱和。

IF 2.3 3区医学

Pediatric Pulmonology Pub Date : 2025-09-01 DOI: 10.1002/ppul.71291

Karolína Doležalová, Jana Kodetová, Eva Klabusayová, Jana Svobodová, Karel Karmazín, Pavel Tkadlec, Emília Kopecká, Jan Helešic

{"title":"Beyond the Usual Suspects: Neonatal Fever and Desaturation.","authors":"Karolína Doležalová, Jana Kodetová, Eva Klabusayová, Jana Svobodová, Karel Karmazín, Pavel Tkadlec, Emília Kopecká, Jan Helešic","doi":"10.1002/ppul.71291","DOIUrl":"10.1002/ppul.71291","url":null,"abstract":"Background: We report a 1-month-old infant admitted with fever, dyspnea, and desaturation after an unmonitored pregnancy in a migrant family. The patient rapidly required mechanical ventilation. Laboratory parameters were markedly elevated, suggesting an infectious etiology.Methods: The clinical course is described with emphasis on diagnostic challenges, including imaging, laboratory findings, and the stepwise process of differential diagnosis.Results: After common etiologies were excluded, rarer causes were considered. The final diagnosis was established through integration of clinical assessment, radiological imaging, laboratory data, and microbiological testing.Conclusion: In neonates with fever and respiratory distress, RSV bronchiolitis is usually the first consideration. If excluded, clinicians must remain alert to less frequent etiologies. This case underscores the importance of a broad differential diagnosis in infants with acute respiratory compromise.","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 9","pages":"e71291"},"PeriodicalIF":2.3,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12431716/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145054958","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 Dose of Hope: Successful Tobramycin Desensitization in Pediatric Cystic Fibrosis. 一剂希望：妥布霉素成功脱敏治疗儿童囊性纤维化。

IF 2.3 3区医学

Pediatric Pulmonology Pub Date : 2025-09-01 DOI: 10.1002/ppul.71276

Isabel G Wedig, Nicholas D Kayser, Brittany A Wright

引用次数: 0

Tracheal Compression in a Child With a Bovine Aortic Arch. 牛主动脉弓患儿气管受压1例。

IF 2.3 3区医学

Pediatric Pulmonology Pub Date : 2025-09-01 DOI: 10.1002/ppul.71282

Manoj Madhusudan, S Jeyakumar, Singaravelu Manikavasagam

引用次数: 0

Caregiver Social Needs and Outpatient Respiratory Outcomes in a Cohort of Children With Bronchopulmonary Dysplasia. 支气管肺发育不良儿童队列的护理人员社会需求和门诊呼吸结局。

IF 2.3 3区医学

Pediatric Pulmonology Pub Date : 2025-09-01 DOI: 10.1002/ppul.71286

Danielle Carlin, Amanda Martin, Camya VanCliff, Noah Baldwin, Brianna C Aoyama, Joseph M Collaco, Sharon A McGrath-Morrow

{"title":"Caregiver Social Needs and Outpatient Respiratory Outcomes in a Cohort of Children With Bronchopulmonary Dysplasia.","authors":"Danielle Carlin, Amanda Martin, Camya VanCliff, Noah Baldwin, Brianna C Aoyama, Joseph M Collaco, Sharon A McGrath-Morrow","doi":"10.1002/ppul.71286","DOIUrl":"https://doi.org/10.1002/ppul.71286","url":null,"abstract":"Objective: Bronchopulmonary dysplasia (BPD) is the most common cause of chronic lung disease in infancy. Caregivers often experience significant challenges in caring for these medically complex children. The purpose of this study was to determine feasibility of administering an electronic social determinants of health (SDoH) screening tool and to determine if caregiver social needs correlate with respiratory outcomes in children with BPD.Study design: An SDoH screening tool was completed by caregivers of children seen in BPD pulmonary clinics at the Children's Hospital of Philadelphia (2022-2025). Subject characteristics and respiratory health outcomes were obtained by chart review and questionnaires.Results: Approximately 1/3 of caregivers (34.7%) endorsed a social need, with difficulty paying utility bills the most expressed need. Children of caregivers who endorsed at least one social need were more likely to have lower birth weights, earlier gestational ages, and caregivers who self-report as Nonwhite. For every social need identified, there was a 32% increased risk of an emergency department visit and a 38% increased risk of rehospitalization (unadjusted). When analyzed by self-reported race, children of White but not Nonwhite caregivers, had twice the likelihood of an ED visit/rehospitalization with each social need endorsed.Conclusion: Social need endorsement is high in caregivers of children with BPD. An association between social need endorsement and ED visits/re-hospitalizations was found in children of self-reported White but not Nonwhite caregivers, suggesting that other systemic issues not identified by the SDoH tool account for the high acute care events in children of Nonwhite caregivers.","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 9","pages":"e71286"},"PeriodicalIF":2.3,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145023983","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