Pierre Goussard, Ernst Eber, Shyam Venkatakrishna, Savvas Andronikou, Andre Gie, Delano Rhode, Carmen Jacobs, Lars Ebert, Janette Verster, Jacques Janson, Pawel Schubert, Celeste Burger
{"title":"Radial EBUS to Identify Tuberculosis Mediastinal Lymph Nodes for Biopsy in Young Children.","authors":"Pierre Goussard, Ernst Eber, Shyam Venkatakrishna, Savvas Andronikou, Andre Gie, Delano Rhode, Carmen Jacobs, Lars Ebert, Janette Verster, Jacques Janson, Pawel Schubert, Celeste Burger","doi":"10.1002/ppul.71120","DOIUrl":"https://doi.org/10.1002/ppul.71120","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 5","pages":"e71120"},"PeriodicalIF":2.7,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143977286","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}
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":"<p><strong>Objective: </strong>To investigate use of asthma controller medications and their effect on lung function in pediatric patients with sickle cell disease (SCD).</p><p><strong>Methods: </strong>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 (FEV<sub>1</sub>, FVC, FEV<sub>1</sub>/FVC, FEF<sub>25%-75%)</sub>) were examined (Fisher exact, Wilcoxon rank sum) in SCD patients overall, and by physician-diagnosed asthma.</p><p><strong>Results: </strong>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 FEV<sub>1</sub> (12.3 vs -3.6; p < 0.0001), FVC (10.5 vs -1.3; p < 0.0001), and FEF<sub>25%-75%</sub>, (20.6 vs -8.8; p < 0.0001), overall and in both asthmatics and non-asthmatics.</p><p><strong>Conclusion: </strong>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.</p>","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}
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":"<p><strong>Objective: </strong>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.</p><p><strong>Methods: </strong>The patients were diagnosed by means of whole exome sequencing and functional validation of the variant was performed in primary patient cells.</p><p><strong>Results: </strong>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).</p><p><strong>Conclusion: </strong>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.</p>","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}
{"title":"Allergic Broncho-Pulmonary Aspergillosis (ABPA) as an Initial Manifestation of Cystic Fibrosis in a Young Child.","authors":"Nikhil Rajvanshi, Taruna Yadav, Jagdish Prasad Goyal, Prawin Kumar","doi":"10.1002/ppul.71140","DOIUrl":"https://doi.org/10.1002/ppul.71140","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 5","pages":"e71140"},"PeriodicalIF":2.7,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144111562","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}
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":"<p><strong>Objective: </strong>To investigate the clinical efficacy of gua sha therapy combined with acupoint application on pediatric chronic cough.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>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.</p>","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}
{"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":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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).</p><p><strong>Conclusion: </strong>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.</p>","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}
Benjamin Stoecklin, Zeena Al-Obaidi, Jenny Svedenkrans, Raffaele Dellacà, J Jane Pillow
{"title":"Diaphragm Function in Very Preterm Infants at 36 Weeks' Postmenstrual Age.","authors":"Benjamin Stoecklin, Zeena Al-Obaidi, Jenny Svedenkrans, Raffaele Dellacà, J Jane Pillow","doi":"10.1002/ppul.71121","DOIUrl":"10.1002/ppul.71121","url":null,"abstract":"<p><strong>Objectives: </strong>Understand how bronchopulmonary dysplasia (BPD) and antenatal and postnatal factors influence diaphragmatic functional effectiveness in very preterm infants.</p><p><strong>Working hypothesis: </strong>Diaphragmatic functional effectiveness during spontaneous breathing is impaired in infants with BPD. Moreover, diaphragmatic functional effectiveness is influenced by adverse antenatal and postnatal factors.</p><p><strong>Methodology: </strong>Diaphragmatic functional effectiveness was assessed in a single-centre, prospective observational study in preterm infants. Transdiaphragmatic pressure (Pdi) and respiratory flow were measured during quiet sleep at 36 weeks' postmenstrual age (PMA). Pdi was normalized to tidal volume (Pdi/V<sub>T</sub>). Diaphragmatic work of breathing per minute was calculated from the inspiratory pressure time integral (PTIdi) and respiratory rate. Factors predictive for each outcome were identified from multivariable linear regression.</p><p><strong>Results: </strong>Very preterm infants (n = 182) were measured at a median (IQR) 35.6 (1.3) weeks' PMA. Infants with BPD had a lower Pdi/V<sub>T</sub> (p = 0.007) and lower PTIdi·min<sup>-1</sup> (p = 0.022) but higher minute ventilation (p = 0.032) and similar respiratory rates (p = 0.419) compared to infants without BPD. Birthweight Z score (R<sup>2</sup> = 0.08, p < 0.001) and BPD (R<sup>2</sup> = 0.04, p = 0.022) were independent negative predictors for Pdi/V<sub>T</sub> while gestational age (R<sup>2</sup> = 0.04, p = 0.01) and average early postnatal energy intake (R<sup>2</sup> = 0.03, p = 0.026) were independent positive predictors for PTIdi·min<sup>-1</sup> on multivariable analysis. Chorioamnionitis and duration of mechanical ventilation did not contribute to the final model.</p><p><strong>Conclusions: </strong>Contrary to our hypothesis, diaphragm functional effectiveness appears improved in infants with BPD. We speculate this finding may reflect an adaptive process, or alternatively indicate an increased recruitment of accessory muscles to achieve required ventilation in BPD infants. Adverse antenatal and postnatal factors only explain a small proportion of variance in diaphragm effectiveness.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"60 5","pages":"e71121"},"PeriodicalIF":2.7,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12068034/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144006142","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}