Pediatric Pulmonology最新文献

Long-Term Pulmonary Sequelae 5-14 Years After Protracted Bacterial Bronchitis in Early Childhood. 儿童早期慢性细菌性支气管炎后5-14年的长期肺部后遗症。

IF 2.7 3区医学

Pediatric Pulmonology Pub Date : 2025-05-01 DOI: 10.1002/ppul.71111

Jan Hermann, Karen Brückner, Cordula Koerner-Rettberg, Stefanie Dillenhöfer, Folke Brinkmann, Christoph Maier, Christoph M Heyer, Anne Schlegtendal

{"title":"Long-Term Pulmonary Sequelae 5-14 Years After Protracted Bacterial Bronchitis in Early Childhood.","authors":"Jan Hermann, Karen Brückner, Cordula Koerner-Rettberg, Stefanie Dillenhöfer, Folke Brinkmann, Christoph Maier, Christoph M Heyer, Anne Schlegtendal","doi":"10.1002/ppul.71111","DOIUrl":"https://doi.org/10.1002/ppul.71111","url":null,"abstract":"Background: There is little information about long-term changes in pulmonary function tests (PFTs) many years after protracted bacterial bronchitis (PBB), the most common cause of chronic wet cough in early childhood.Methods: Of 200 consecutively recruited children with a previously proven diagnosis of PBB 62 (12.2 years, female 48%) were interviewed after 7.7 (5.4-14.7) years about their previous and current symptoms and pulmonary function tests (PFTs: spirometry, body plethysmography, nitrogen multi-breath washout, exhaled nitric oxide and nasal nitric oxide) were performed. Children with persistent symptoms were offered lung imaging.Results: 11 (17.7%) patients suffered from chronic or recurring wet cough years after their first PBB episode. 15 (24.19%) had at least one abnormal spirometry parameter. FEV1 was abnormal in eight of 62 (12.9%), LCI 2.5% in seven of 56 (12.5%), FVC in 12 of 62 (19.35%) and FEV1/FVC in five of 62 (8.06%) cases. PFT did not differ between children with and without wet cough. Lung MRI/CT demonstrate in four of nine cases abnormalities of the bronchial walls, including one with incipient bronchiectasis.Conclusion: After PBB in early childhood, a significant proportion of children suffer from respiratory symptoms many years later, some have an objectively reduced lung function and structural changes of the bronchial wall despite adequate initial therapy. Wet cough alone seems not to be a sensitive clinical predictor. Due to the retrospective study design, we cannot proof any causal relationship. However, to detect late bronchopulmonary sequelae, continuous follow-up of these children should become mandatory.","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 5","pages":"e71111"},"PeriodicalIF":2.7,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12053102/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144010613","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

The Association of Neonatal Respiratory Distress With Ciliary Ultrastructure and Genotype in Primary Ciliary Dyskinesia. 原发性纤毛运动障碍新生儿呼吸窘迫与纤毛超微结构及基因型的关系。

IF 2.7 3区医学

Pediatric Pulmonology Pub Date : 2025-05-01 DOI: 10.1002/ppul.71091

Andrew T Barber, Stephanie D Davis, Thomas W Ferkol, Adam J Shapiro, Jeff Atkinson, Scott D Sagel, Sharon D Dell, Kenneth Olivier, Carlos Milla, Margaret Rosenfeld, Lang Li, Feng-Chang Lin, Kelli M Sullivan, Nicole A Capps, Maimoona A Zariwala, Michael R Knowles, Margaret W Leigh

{"title":"The Association of Neonatal Respiratory Distress With Ciliary Ultrastructure and Genotype in Primary Ciliary Dyskinesia.","authors":"Andrew T Barber, Stephanie D Davis, Thomas W Ferkol, Adam J Shapiro, Jeff Atkinson, Scott D Sagel, Sharon D Dell, Kenneth Olivier, Carlos Milla, Margaret Rosenfeld, Lang Li, Feng-Chang Lin, Kelli M Sullivan, Nicole A Capps, Maimoona A Zariwala, Michael R Knowles, Margaret W Leigh","doi":"10.1002/ppul.71091","DOIUrl":"https://doi.org/10.1002/ppul.71091","url":null,"abstract":"Objective: To evaluate the relationship between ciliary ultrastructure/genotype and prevalence of neonatal respiratory distress (NRD) in primary ciliary dyskinesia (PCD).Study design: This was a retrospective analysis from a multicenter, prospective study of children and adults with PCD. Participants were classified by ultrastructural defect associated with their diagnostic genetic variants: 1) outer dynein arm defect alone (ODA), 2) outer plus inner dynein arm defect (ODA/IDA), 3) inner dynein arm defect with microtubular disorganization (IDA/MTD), 4) DNAH11 (encodes ODA protein but has normal ultrastructure), and 5) normal/near-normal/other. The likelihood of NRD between ultrastructure groups or genotypes was evaluated by multivariate analysis using logistic regression, controlled for age, gender, race, and variant type. Similar analysis was performed within individual genotypes to assess association of NRD with the presence of 2 loss-of-function variants.Results: Of the 455 participants analyzed, 305 (67.0%) reported NRD. The odds ratio for NRD in the DNAH11 group was significantly lower (OR: 0.35, 95% CI: 0.16-0.76) compared to NRD in the ODA group. Within the DNAH5 group, those with two loss-of-function variants were more likely to have NRD compared to those with possible residual function variants (OR: 3.06, 95% CI: 1.33-7).Conclusion: NRD is less common in those with DNAH11 variants, thus a high index of suspicion should remain for PCD in the absence of NRD. Variant type (loss-of-function vs. residual function) may explain phenotypic variability within individual PCD genes.","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 5","pages":"e71091"},"PeriodicalIF":2.7,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12063519/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144035085","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

Prospective Evaluation of Cardiorespiratory Fitness After Hematopoietic Stem Cell Transplantation in Children. 儿童造血干细胞移植后心肺健康的前瞻性评价。

IF 2.7 3区医学

Pediatric Pulmonology Pub Date : 2025-05-01 DOI: 10.1002/ppul.70994

Véronique Houdouin, Hala Feghali, Sophie Boileau, Michel Guinot, Philippe Reix, Justine Pages, Sophie Guilmin Crepon, Christophe Delclaux

{"title":"Prospective Evaluation of Cardiorespiratory Fitness After Hematopoietic Stem Cell Transplantation in Children.","authors":"Véronique Houdouin, Hala Feghali, Sophie Boileau, Michel Guinot, Philippe Reix, Justine Pages, Sophie Guilmin Crepon, Christophe Delclaux","doi":"10.1002/ppul.70994","DOIUrl":"https://doi.org/10.1002/ppul.70994","url":null,"abstract":"Allogeneic hematopoietic stem cell transplantation (HSCT) is associated with long-term sequelae such as reduced exercise capacity, but whether some degree of spontaneous recovery occurs during childhood is yet to be determined. This prospective multicenter study aimed to evaluate exercise capacity at 1 and 3 years after HSCT. Forty-four (30 males, median age [interquartile] 13.0 years [10.5; 15.4] at first cardiopulmonary exercise test [CPET]) of the 292 children included in the RESPPEDHEM cohort had a CPET at both time points. Their median z-score of peak V'O2 did not significantly improve between the two CPETs: -2.7 (-4.0; -1.5) at 1 year versus -2.2 (-3.1; -0.6) at 3 years, p = 0.09. It was associated with a low z-score of peak oxygen pulse: -2.2 (-3.6; -0.9) versus -1.9 (-2.8; -0.9), p = 0.14, suggesting low muscular fitness. Female sex was associated with a higher risk of low muscular fitness, whereas the Play-Performance Scale (PPS) was unable to differentiate between adolescents with or without low fitness (37/39 adolescents had a PPS score evaluated by their parents of 100 and were judged as fully active and normal, contrasting with the results of their CPET). In conclusion, impaired exercise capacity is present one1 year after HSCT, does not improve after two subsequent years in adolescents, and is under-evaluated by their parents. CLINICALTRIALS.GOV IDENTIFIER: NCT02032381.","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 5","pages":"e70994"},"PeriodicalIF":2.7,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12079716/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144079381","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

Radial EBUS to Identify Tuberculosis Mediastinal Lymph Nodes for Biopsy in Young Children. 放射状EBUS对幼儿结核纵隔淋巴结活检的鉴别。

IF 2.7 3区医学

Pediatric Pulmonology Pub Date : 2025-05-01 DOI: 10.1002/ppul.71120

Pierre Goussard, Ernst Eber, Shyam Venkatakrishna, Savvas Andronikou, Andre Gie, Delano Rhode, Carmen Jacobs, Lars Ebert, Janette Verster, Jacques Janson, Pawel Schubert, Celeste Burger

引用次数: 0

Multicystic Interstitial Lung Disease Due to a Novel Biallelic C-C Chemokine Receptor Type 2 Variant. 由一种新的双等位基因C-C趋化因子受体2型变异引起的多囊间质性肺病。

IF 2.7 3区医学

Pediatric Pulmonology Pub Date : 2025-05-01 DOI: 10.1002/ppul.71135

Moritz Herkner, Christina Rapp, Simon Y Graeber, Charlotte Marx, Carlotta Rambuscheck, Simone Reu-Hofer, Nagehan Emiralioglu, Nural Kiper, Alexandru I Gilea, Ilenia Notaroberto, Enrico Baruffini, Bettina Temmesfeld-Wollbrück, Christoph Klein, Han Wen, Mirjam Stahl, Matthias Griese, Florian Gothe

{"title":"Multicystic Interstitial Lung Disease Due to a Novel Biallelic C-C Chemokine Receptor Type 2 Variant.","authors":"Moritz Herkner, Christina Rapp, Simon Y Graeber, Charlotte Marx, Carlotta Rambuscheck, Simone Reu-Hofer, Nagehan Emiralioglu, Nural Kiper, Alexandru I Gilea, Ilenia Notaroberto, Enrico Baruffini, Bettina Temmesfeld-Wollbrück, Christoph Klein, Han Wen, Mirjam Stahl, Matthias Griese, Florian Gothe","doi":"10.1002/ppul.71135","DOIUrl":"10.1002/ppul.71135","url":null,"abstract":"Objective: We are presenting two individuals with biallelic C-C chemokine receptor type 2 (CCR2) deficiency carrying the novel c.644C>T p.L215P variant, who presented with chronic respiratory symptoms during infancy and developed multiple diffuse cystic lesions during childhood.Methods: The patients were diagnosed by means of whole exome sequencing and functional validation of the variant was performed in primary patient cells.Results: While size and extent of the cysts were stable over years, progressive lung function decline was noted in adolescence and adulthood respectively. The CCR2 p.L215P variant was found to be loss-of-expression and patient monocytes displayed a migration defect upon stimulation with the CCR2 ligand C-C motif ligand 2 (CCL2).Conclusion: With a follow-up of up to 25 years, this report expands our understanding of lung disease in CCR2 deficiency and offers another monogenic cause of cystic lung disease. Early genetic diagnosis of affected individuals might allow potentially curative treatment by haematopoietic stem cell transplantation.","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 5","pages":"e71135"},"PeriodicalIF":2.7,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12117283/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144161035","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

Use of Asthma Medications in African American Children With Sickle Cell Disease: A Single Center Experience. 在患有镰状细胞病的非裔美国儿童中使用哮喘药物：单中心经验

IF 2.7 3区医学

Pediatric Pulmonology Pub Date : 2025-05-01 DOI: 10.1002/ppul.71147

Allison Jue, Lucia Mirea, Alyssa McGary, Sophia Williams

{"title":"Use of Asthma Medications in African American Children With Sickle Cell Disease: A Single Center Experience.","authors":"Allison Jue, Lucia Mirea, Alyssa McGary, Sophia Williams","doi":"10.1002/ppul.71147","DOIUrl":"10.1002/ppul.71147","url":null,"abstract":"Objective: To investigate use of asthma controller medications and their effect on lung function in pediatric patients with sickle cell disease (SCD).Methods: Retrospective study in pediatric patients who self-identified as African American with SCD treated at Phoenix Children's between 2014 and 2021. Associations of asthma controller medications with changes in lung symptoms (cough, wheeze, chest pain, shortness of breath with exercise, sleep disturbance), Acute Chest Syndrome (ACS), and percent predicted probabilities (FEV1, FVC, FEV1/FVC, FEF25%-75%)) were examined (Fisher exact, Wilcoxon rank sum) in SCD patients overall, and by physician-diagnosed asthma.Results: Of the total 98 SCD patients, 28 (29%) had an asthma diagnosis and 76 (78%) were treatment naïve. During study follow-up, asthma controller medications were used by 57 (58%) patients (35 new prescriptions, 13 continued prescriptions and 9 with prescription escalation), with 41 patients remaining treatment naïve. Medication use vs non-use during follow-up improved cough (33% vs 7%, p = 0.002), chest pain (12% vs 5%, p = 0.03) and shortness of breath with exercise (32% vs 10%, p = 0.01) among all SCD patients. Medications also improved the mean relative percent change FEV1 (12.3 vs -3.6; p < 0.0001), FVC (10.5 vs -1.3; p < 0.0001), and FEF25%-75%, (20.6 vs -8.8; p < 0.0001), overall and in both asthmatics and non-asthmatics.Conclusion: These findings demonstrate improved lung function and respiratory symptoms with asthma controller medications in pediatric patients with SCD, irrespective of an asthma diagnosis, and hold great promise for this undertreated population.","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 5","pages":"e71147"},"PeriodicalIF":2.7,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144161051","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

Feeding the Need: A Study on Food Security Among People With Cystic Fibrosis in Turkey. 满足需求：土耳其囊性纤维化患者的粮食安全研究。

IF 2.7 3区医学

Pediatric Pulmonology Pub Date : 2025-05-01 DOI: 10.1002/ppul.71101

Damla Kocaman, Ceren Ayça Yıldız, Neval Metin Çakar, Burcu Uzunoğlu, Gamze Taştan, Mine Yüksel Kalyoncu, Merve Selçuk Balcı, Şeyda Karabulut, Pınar Ergenekon, Yasemin Gökdemir, Ela Erdem Eralp, Fazilet Karakoç, Bülent Karadağ

{"title":"Feeding the Need: A Study on Food Security Among People With Cystic Fibrosis in Turkey.","authors":"Damla Kocaman, Ceren Ayça Yıldız, Neval Metin Çakar, Burcu Uzunoğlu, Gamze Taştan, Mine Yüksel Kalyoncu, Merve Selçuk Balcı, Şeyda Karabulut, Pınar Ergenekon, Yasemin Gökdemir, Ela Erdem Eralp, Fazilet Karakoç, Bülent Karadağ","doi":"10.1002/ppul.71101","DOIUrl":"https://doi.org/10.1002/ppul.71101","url":null,"abstract":"Background: Cystic fibrosis (CF) is a genetic disorder that necessitates high-calorie, protein-rich diets, leading to nutritional deficiencies. Food insecurity (FI) poses a significant challenge for people with CF (pwCF), impacting their ability to maintain the necessary dietary intake. This study aims to explore FI and dietary patterns among pwCF in Turkey.Methods: A cross-sectional study involving 290 pwCF from the Marmara University Selim Çöremen Cystic Fibrosis Center was conducted between April 2023 and February 2024. The \"US Household Food Security Survey Module\" and the \"Your Current Life Situation\" survey were used to assess FI and socioeconomic status among the participants. Nutritional data, including BMI, FEV1 values, and dietary intake, were recorded.Results: Among the participants, 52.7% were female, with a mean age of 13.3 ± 8.1 years. FI was detected in 46.8% of pwCF, with 18% facing very low food security. Higher income levels were associated with better food security (p = 0.008). Nutritional inadequacies were observed even among food-secure individuals, particularly in the consumption of legumes, nuts, and fish. BMI and BMI percentile values were significantly lower in the very low FS group compared to the high FS group (p = 0.03 and p = 0.02, respectively).Conclusion: Ensuring adequate nutrition and calorie intake is crucial for pwCF. Our study highlights significant FI among pwCF in Turkey, with income levels influencing food security status. Nutritional inadequacies persist even among those classified as food secure. Based on these findings, targeted nutritional support will be provided to those in need to improve overall health and well-being.","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 5","pages":"e71101"},"PeriodicalIF":2.7,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12053234/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143973747","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

Clinical Efficacy of Gua Sha Therapy Combined With Acupoint Application on Chronic Cough in Pediatrics. 瓜沙疗法配合穴位贴敷治疗小儿慢性咳嗽临床疗效观察。

IF 2.7 3区医学

Pediatric Pulmonology Pub Date : 2025-05-01 DOI: 10.1002/ppul.71065

Danxia Ni, Liyun Zhong, Xinping Liu, Wenjing Lai, Jingyi Mai, Weiyi Dai, Meifeng Xie, Lifang Lei

{"title":"Clinical Efficacy of Gua Sha Therapy Combined With Acupoint Application on Chronic Cough in Pediatrics.","authors":"Danxia Ni, Liyun Zhong, Xinping Liu, Wenjing Lai, Jingyi Mai, Weiyi Dai, Meifeng Xie, Lifang Lei","doi":"10.1002/ppul.71065","DOIUrl":"https://doi.org/10.1002/ppul.71065","url":null,"abstract":"Objective: To investigate the clinical efficacy of gua sha therapy combined with acupoint application on pediatric chronic cough.Methods: The convenience sampling method was used to enroll 200 children with chronic cough from a hospital in Guangzhou between April 2023 and October 2024 in this study. These children were divided into a conventional control group, an acupoint patch group, a gua sha group, and a gua sha therapy combined with an acupoint patch treatment group (combined group). The intervention measures included conventional medication treatment, gua sha, and acupoint application. There were 50 patients in each group, and cough symptom scores, life quality questionnaire (Leicester Cough Questionnaire), and simulated visual pain scores (visual analog scale [VAS]) were collected for comparison.Results: Compared with the pretreatment period, daytime and nighttime scores in all four groups were reduced (p < 0.05). After treatment, the pairwise comparisons showed the following descending order: control group > acupoint application group = gua sha group > combined group (p < 0.05). Quality of life scores increased, whereas VAS scores decreased in all four groups compared with pretreatment (p < 0.05). After treatment, the pairwise comparisons revealed that the quality of life scores, in descending order, were as follows: combined group > acupoint application group = gua sha group > control group (p < 0.05), and the VAS scores, in descending order, were as follows: control group > acupoint application group = gua sha group > combined group (p < 0.05). No significant adverse reactions occurred during the treatment period in all four groups.Conclusion: Gua sha therapy and acupoint application have good clinical efficacy on pediatric chronic cough, and the combination of the two can further improve the efficacy.","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 5","pages":"e71065"},"PeriodicalIF":2.7,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144143353","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

Allergic Broncho-Pulmonary Aspergillosis (ABPA) as an Initial Manifestation of Cystic Fibrosis in a Young Child. 过敏性支气管-肺曲霉病（ABPA）是幼儿囊性纤维化的初始表现。

IF 2.7 3区医学

Pediatric Pulmonology Pub Date : 2025-05-01 DOI: 10.1002/ppul.71140

Nikhil Rajvanshi, Taruna Yadav, Jagdish Prasad Goyal, Prawin Kumar

引用次数: 0

Pulmonary Embolization in Pediatric Patients: Single-Center Experience. 儿科患者肺栓塞：单中心经验。

IF 2.7 3区医学

Pediatric Pulmonology Pub Date : 2025-05-01 DOI: 10.1002/ppul.71131

Nilgun Kula, Tugba Sismanlar Eyuboglu, Mehmet Koray Akkan, Oznur Leman Boyunaga, Ahmet Baran Onal, Ayse Tana Aslan

引用次数: 0