{"title":"Multiple Organ Involvement in a Boy with Lipopolysaccharide-Responsive Beige-like Anchor Protein Deficiency Detected by F-18 Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography.","authors":"Ummuhan Abdulrezzak, Emre Temizer, Alper Ozcan","doi":"10.1177/2151321X251368265","DOIUrl":"10.1177/2151321X251368265","url":null,"abstract":"<p><p>Patients with pathogenic variants of <i>lipopolysaccharide-responsive beige-like anchor protein</i> (<i>LRBA</i>) are known to present with autoimmune diseases, inflammatory bowel disease, lymphoproliferative disorders, allergies, immunodeficiency, and malignancies. This condition, characterized by widespread infections that impact multiple systems, has various radiological findings reported in the literature. These include computed tomography (CT) findings indicating lung involvement and magnetic resonance imaging (MRI) findings showing neurological system involvement. However, F-18 fluorodeoxyglucose positron emission tomography-CT (FDG PET-CT) imaging findings in <i>LRBA</i> deficiency have not yet been described in the literature. This report presents multiple organ involvements detected by F-18 FDG PET-CT in a case with an <i>LRBA</i> gene variant. FDG PET-CT findings for diagnostic and primary focus evaluation were reviewed in a 17-year-old male patient with a pathogenic <i>LRBA</i> variant, prompted by multiple hypoechoic nodular appearances identified on abdominal ultrasonography. In this patient, who carried a pathogenic <i>LRBA</i> variant (c.3396-3397delAC, p.D975Yfs*15) and was treated with abatacept for liver involvement, FDG PET-CT revealed a wide range of system involvements. These included the lungs, liver, intestines, bone, bone marrow, and lymph nodes, as well as multiple joints and tendons. In immunodeficiency diseases with such extensive multisystem involvement, whole-body imaging techniques like F-18 FDG PET-CT can serve as valuable tools for physicians to comprehensively assess the disease and formulate effective treatment strategies.</p>","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":" ","pages":"144-148"},"PeriodicalIF":0.8,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144876870","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Navigating Measles Treatment in 2025: A Review on Vaccines, Medications, and Supplements.","authors":"Misty M Oldham, Sandra Benavides, Paul M Boylan","doi":"10.1177/2151321X251397927","DOIUrl":"10.1177/2151321X251397927","url":null,"abstract":"<p><p>Measles is a highly contagious, vaccine-preventable infectious disease. The incidence of measles has been rising due to a confluence of factors, including international travel and vaccine hesitancy. The purpose of this Pharmacotherapy Update was to examine and appraise preventive measures and treatment options, both pharmacotherapy and supplements, used in measles management. Topics included ribavirin, measles-mumps-rubella vaccine post-exposure prophylaxis, immune globulin, antibiotics, vitamin A, and cod liver oil supplements. Parents, caregivers, and policymakers considering these interventions are recommended to consult with health care providers and seek guidance from professional organizations regarding the effectiveness and safety of these treatments. Vaccination with the measles-mumps-rubella vaccine remains the most effective intervention to prevent measles, and efforts are urgently needed to achieve the requisite 95% vaccination rate to improve public health, confer herd immunity, and eradicate measles.</p>","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":" ","pages":"113-118"},"PeriodicalIF":0.8,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145543486","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Expression Profiles of Ferroptosis-Related Genes and Their Diagnostic Value in Infants with Bronchopulmonary Dysplasia.","authors":"Honghua Jiang, Yuan Lv, Weiwei Hou, Yanlin Liu","doi":"10.1177/2151321X251395599","DOIUrl":"10.1177/2151321X251395599","url":null,"abstract":"<p><p><b><i>Background:</i></b> Bronchopulmonary dysplasia (BPD), a common pulmonary condition in infants causing neonatal death, has a complicated pathogenic mechanism. As the new iron-dependent cell death type, ferroptosis can result from lipid peroxidation and exert a critical effect on the pathogenic mechanism of BPD. This study aimed to investigate ferroptosis-related genes with regard to their expression patterns and functional roles in BPD. <b><i>Methods:</i></b> Clinical and gene expression data were obtained based on the Gene Expression Omnibus (GEO) database, and the web-based analysis approach GEO2R was used for selecting differentially expressed genes (DEGs). For significant ferroptosis-related DEGs (FDEGs), their bioinformatic functions and molecular interactions were explored using the WEB-based Gene Set Analysis Toolkit (WebGestalt) and Metascape, protein-protein interaction network analysis, and Kyoto Encyclopedia of Genes and Genomes enrichment. In addition, hub FDEG expression levels in BPD were verified through quantitative reverse transcription polymerase chain reaction (RT-qPCR). <b><i>Results:</i></b> There were totally 3,673 DEGs detected in BPD infants compared with controls, including 36 FDEGs with upregulation, whereas 13 with downregulation. Functional enrichment analysis revealed the significant activation of biological processes in response to stress and ferroptosis. Through RT-qPCR validation, five hub FDEGs were identified, including mitogen-activated protein kinase 14 (<i>MAPK14</i>), tumor antigen p53 (<i>TP53</i>), signal transducer and activator of transcription 3 (<i>STAT3</i>), toll-like receptor 4 (<i>TLR4</i>), and dual-specificity protein phosphatase 1 (<i>DUSP1</i>). Based on the outcomes of receiver operating characteristic curve analysis, the area under the curve values of these genes were >0.7, revealing that they might be used to identify BPD. <b><i>Conclusions:</i></b> The results in this study shed more insights on the diagnosis and mechanism of ferroptosis in BPD. Further research should be carried out to assess its clinical utility.</p>","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":" ","pages":"123-131"},"PeriodicalIF":0.8,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145543411","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Hiromi Onuma-Zamayoa, Claudia Santamaría-Arza, Leonardo Rivera-Rodríguez, Sarbelio Moreno-Espinosa, Marco Antonio Yamazaki-Nakashimada
{"title":"MIS-C Today: Diagnostic Challenges in a Changing Epidemiological Landscape.","authors":"Hiromi Onuma-Zamayoa, Claudia Santamaría-Arza, Leonardo Rivera-Rodríguez, Sarbelio Moreno-Espinosa, Marco Antonio Yamazaki-Nakashimada","doi":"10.1177/2151321X251395479","DOIUrl":"10.1177/2151321X251395479","url":null,"abstract":"","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":" ","pages":"119-122"},"PeriodicalIF":0.8,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145566159","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Rose Hawkins, Sara Weathers, Ronald Morton, Adrian O'Hagan, Erica Stevens, Michael Orangias, Scott Bickel
{"title":"Improved Lung Function with Home Oscillation and Lung Expansion Therapy in Children: A Case Series.","authors":"Rose Hawkins, Sara Weathers, Ronald Morton, Adrian O'Hagan, Erica Stevens, Michael Orangias, Scott Bickel","doi":"10.1177/2151321X251394848","DOIUrl":"10.1177/2151321X251394848","url":null,"abstract":"<p><p><b><i>Background:</i></b> Oscillation and lung expansion (OLE) therapy delivers both continuous high-frequency oscillation and continuous positive expiratory pressure. This therapy aids in both mobilizing secretions and lung expansion. Objective data about the chronic use of this therapy in the outpatient setting in pediatric patients are limited. <b><i>Case Presentation:</i></b> We identified 3 patients in our pediatric pulmonology clinic, each with different underlying conditions, who had used home OLE therapy for at least 1 year and were able to perform spirometry. We compared forced expiratory volume in 1 second (FEV<sub>1</sub>) in the year prior to therapy with the following year after starting therapy. Patients A, B, and C had a mean FEV<sub>1</sub> percent predicted improvement in the year after starting therapy of 19.3%, 13.6%, and 30.5%, respectively. <b><i>Conclusion:</i></b> Substantial and sustained improvements in lung function were observed with the addition of OLE to standard-of-care therapies. Larger studies are needed to confirm these findings.</p>","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":" ","pages":"149-152"},"PeriodicalIF":0.8,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145446541","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sevgi Sipahi Cimen, Gizem Kara Elitok, Muhammed Fatih Erbay, Lida Bulbul, Deniz Ozceker, Pınar Yılmazbas, Ugurcan Sayili, Zeynep Ulker Altinel, Ali Bulbul
{"title":"Allergic Adverse Reactions Following Measles-Mumps-Rubella Vaccination in Children with Food Allergy: A Prospective Multicenter Study.","authors":"Sevgi Sipahi Cimen, Gizem Kara Elitok, Muhammed Fatih Erbay, Lida Bulbul, Deniz Ozceker, Pınar Yılmazbas, Ugurcan Sayili, Zeynep Ulker Altinel, Ali Bulbul","doi":"10.1177/2151321X251393016","DOIUrl":"10.1177/2151321X251393016","url":null,"abstract":"<p><p><b><i>Introduction:</i></b> The aim of this study was to determine the frequency and risk factors of allergic adverse reaction following measles-mumps-rubella (MMR) vaccination in children with egg and/or cow's milk allergies. <b><i>Methods:</i></b> Children with cow's milk and/or egg allergy were included. Patients with IgE-mediated cow's milk allergy were subjected to skin tests with MMR vaccines before vaccination. For patients with a positive skin test, administration of an alternative vaccine not containing the suspected excipient was planned. In case an alternative vaccine is not available, the MMR vaccine is planned to be administered with a gradual desensitization protocol. <b><i>Results:</i></b> Two hundred two patients (133 male and 69 female) with a mean age of 14.9 ± 11.9 months were evaluated. Of the patients, 126 (62.4%) received Tresivac®, while 76 (37.6%) received Priorix®. Before vaccination, 84 patients underwent skin testing with the vaccine, and the test was positive in 7 patients. Allergic reactions were observed in 12 patients (urticaria in 10 patients, angioedema in 1 patient, and anaphylaxis in 1 patient). Egg white-specific IgE (spIgE) levels were found to be higher in patients with egg-allergy who developed allergic reactions to MMR vaccines. The receiver operating characteristic (ROC) analysis identified a cut-off value of 6.5 kU/L for egg white-spIgE. In patients who developed allergic reactions following alpha-lactalbumin-containing vaccine, egg white-spIgE and cow's milk-spIgE levels were significantly higher compared with those without reactions. In the ROC analysis for predicting allergic reactions following Tresivac® vaccination, the cut-off values were as follows: egg white-spIgE: 6 kU/L, cow's milk-spIgE: 12.5 kU/L. <b><i>Conclusion:</i></b> It was determined that the cut-off values for cow's milk and egg white spIgE were found to be significant in identifying children at risk for allergic reactions following MMR vaccination. Children with food allergies who had a family history of atopy were found to have a higher risk of allergic reactions.</p>","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":" ","pages":"132-140"},"PeriodicalIF":0.8,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145483855","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Rose Hawkins, Corrie Harris, Heather Huxol, Scott Bickel, Mary Elise McClanahan, Theresa Kluthe, Ronald Morton
{"title":"Changes in Respiratory Care Use During the COVID-19 Pandemic in a Pediatric Long-Term Care Facility.","authors":"Rose Hawkins, Corrie Harris, Heather Huxol, Scott Bickel, Mary Elise McClanahan, Theresa Kluthe, Ronald Morton","doi":"10.1089/ped.2024.0125","DOIUrl":"10.1089/ped.2024.0125","url":null,"abstract":"<p><p><b><i>Background:</i></b> The COVID-19 pandemic impacted multiple aspects of care at pediatric long-term care facilities (pLTCFs). This study evaluated the effect of COVID-19 infection control policies on pLTCF resident's respiratory health. <b><i>Methods:</i></b> A retrospective study of 76 residents' oxygen, albuterol, hypertonic saline (HTS), and pulmonary hygiene treatment use from January 2019 to December 2022 was completed using de-identified billing data and chart review. <b><i>Results:</i></b> There was a statistically significant increase in rate of oxygen use among residents (5.67; 95% CI 3.44-7.8; <i>P</i> < 0.001) during the study period, but at a slower rate than expected by the pre-pandemic trend (-4.89; 95% CI -7.20-2.59; <i>P</i> < 0.001). HTS use decreased during the study period, with a change in the prior trend to downward (-3.43; -5.89-9.68; <i>P</i> = 0.007). There was no significant change in albuterol or pulmonary hygiene treatment. <b><i>Conclusions:</i></b> This study demonstrates that patterns of oxygen and HTS use in a pLTCF changed during the COVID-19 pandemic.</p>","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":" ","pages":"88-92"},"PeriodicalIF":0.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144477894","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A Rare Clinical Course in Drug Reaction with Eosinophilia and Systemic Symptoms Syndrome: Lung Involvement.","authors":"Deniz Yılmaz, Zeynep Şengül Emeksiz, Gülsüm İclal, Işıl Bilgiç, Emine Dibek Mısırlıoğlu","doi":"10.1177/2151321X251372016","DOIUrl":"10.1177/2151321X251372016","url":null,"abstract":"<p><p><b><i>Background:</i></b> Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) syndrome is a rare, potentially life-threatening hypersensitivity reaction characterized by skin rash, fever, eosinophilia, and multi-organ involvement. Although pulmonary complications are uncommon, they can significantly impact prognosis. <b><i>Case Presentation:</i></b> Here, we present a 6-year-old male with antibiotic-induced DRESS syndrome complicated by pleural effusion and pneumonitis. The patient was treated successfully with systemic corticosteroids and intravenous immunoglobulin following multidisciplinary evaluation. <b><i>Conclusion:</i></b> Pulmonary involvement in DRESS is frequently mistaken for pneumonia. This case highlights the importance of recognizing pulmonary involvement in DRESS syndrome and differentiating it from bacterial pneumonia, as misdiagnosis may lead to delayed corticosteroid treatment and unnecessary antibiotic use.</p>","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":"38 3","pages":"108-111"},"PeriodicalIF":1.3,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144978716","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Value of the FEF50/0.5FVC Ratio in the Identification of Obstructive Ventilatory Defects in Asthmatic Children.","authors":"Khouloud Kchaou, Chaima Briki, Soumaya Rebai, Soumaya Khaldi, Saloua Ben Khamsa Jameleddine","doi":"10.1177/2151321X251360549","DOIUrl":"10.1177/2151321X251360549","url":null,"abstract":"<p><p><b><i>Introduction:</i></b> Pediatric asthma is a common condition that requires early detection of obstructive ventilatory defects (OVDs) for optimal management. While the forced expiratory volume in 1 second to forced vital capacity (FEV1/FVC) ratio is widely used in spirometry, it may lack sensitivity in children, especially in detecting mild or early obstruction. This study aimed to assess the utility of the forced expiratory flow (FEF) at 50% of FVC to the half of FVC (FEF50/0.5FVC) ratio as a complementary tool for identifying OVD in asthmatic children. <b><i>Methods:</i></b> This was a retrospective study conducted on 165 asthmatic children (aged 5-15 years). Spirometric data including FEV1, FVC, FEV1/FVC, FEF50, FEF at 25%-75% of FVC (FEF25%-75%), and peak expiratory flow were analyzed. The FEF50/0.5FVC ratio was calculated for each child. OVD was defined as an FEV1/FVC ratio below the lower limit of normal, based on Z-scores. Statistical analysis included <i>t</i>-tests, Pearson's correlation, and receiver operating characteristic (ROC) curve analysis to compare the diagnostic efficiency of the FEF50/0.5FVC ratio with traditional spirometric measures. <b><i>Results:</i></b> OVD was identified in 25.5% of participants. The FEF50/0.5FVC ratio was significantly lower in the OVD group (1.23 ± 0.24) compared with the normal spirometry group (2.08 ± 0.5, <i>P</i> < 0.0001). A strong correlation was observed between the FEV1/FVC ratio and FEF50/0.5FVC (r = 0.88, <i>P</i> < 0.001). ROC analysis showed that the FEF50/0.5FVC ratio had superior diagnostic accuracy for OVD (area under the curve = 0.948, 95% confidence interval 0.899-0.996) compared with other spirometric indices, with a sensitivity of 91% and specificity of 90.2% at an optimal threshold of 1.56. <b><i>Conclusion:</i></b> FEF50/0.5FVC ratio is a promising tool for detecting OVD in asthmatic children, demonstrating high sensitivity and specificity. It may outperform traditional spirometric measures in identifying subtle airway obstructions, offering a valuable addition to routine asthma diagnostics, particularly in pediatric patients with early-stage or mild asthma.</p>","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":" ","pages":"93-99"},"PeriodicalIF":0.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144857039","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"What Are Alternatives to Oral Phenylephrine in Children?","authors":"Julia Young, Kimberly Novak, Paul M Boylan","doi":"10.1089/ped.2025.0031","DOIUrl":"10.1089/ped.2025.0031","url":null,"abstract":"<p><p>The United States Food and Drug Administration (FDA) proposed removing oral phenylephrine (PE) from over-the-counter single-agent and combination products because it is ineffective at FDA-approved doses to treat sinonasal congestion. Health care providers make thousands of recommendations per month for phenylephrine-containing over-the-counter products to treat adults and children with signs and symptoms of the common cold, specifically nasal congestion. Health care providers may feel compelled by parents to recommend over-the-counter medications to treat the common cold in children, despite resources suggesting those products may be mildly effective, ineffective, or possibly unsafe. The objective of this Pharmacotherapy Update is to suggest alternative treatments to PE in children with the common cold and nasal congestion.</p>","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":" ","pages":"83-87"},"PeriodicalIF":0.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144289759","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}