Intravenous lipid emulsions (ILE) containing soy protein may cause reactions in allergic patients. We present a case report of atopic dermatitis (AD) and soy allergy triggered by intravenous lipid emulsion containing soy in an infant with short bowel syndrome (SBS). A 9-month-old female infant with SBS who had been fed total parenteral nutrition (PN) since birth was consulted for severe AD. Laboratory tests revealed eosinophilia, elevated total IgE, and soy-specific IgE was found positive at 3.56 KU/L. Lipid emulsion containing 20% soybean-oil in PN was replaced with soy-free. The patient's AD lesions resolved rapidly. The food provocation test with re-administration of a soy-containing lipid emulsion confirmed the diagnosis of soy allergy by causing recurrence of eczematous lesions. This case demonstrates that ILE can cause soy sensitization and trigger AD without oral exposure. Food allergies should also not be forgotten in patients who receive only PN.

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. 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). 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 (), tumor antigen p53 (), signal transducer and activator of transcription 3 (), toll-like receptor 4 (), and dual-specificity protein phosphatase 1 (). 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. 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.

Patients with pathogenic variants of () 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 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 gene variant. FDG PET-CT findings for diagnostic and primary focus evaluation were reviewed in a 17-year-old male patient with a pathogenic variant, prompted by multiple hypoechoic nodular appearances identified on abdominal ultrasonography. In this patient, who carried a pathogenic 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.

Children with intestinal failure who are receiving parenteral nutrition through a central venous catheter are at risk of developing catheter-related bloodstream infections. For many years, a prophylactic lock with taurolidine has been used to decrease the incidence of these infections and is considered safe in children. A 1-month-old boy with jejunal atresia was receiving parenteral nutrition through a central venous catheter. Shortly after administration of taurolidine (TauroSept®), he developed circulatory insufficiency and angioedema. His serum tryptase increased, which was suggestive of anaphylaxis (baseline tryptase 6.3 µg/L; 2 h after start of the reaction: 21 µg/L; cutoff criterion for clinically relevant increase: 1.2 × baseline tryptase level + 2). To the best of our knowledge, this is the first case of anaphylaxis in response to TauroSept® in an infant worldwide. Clinicians should be aware of this possible but very rare side effect.

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. 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) in the year prior to therapy with the following year after starting therapy. Patients A, B, and C had a mean FEV percent predicted improvement in the year after starting therapy of 19.3%, 13.6%, and 30.5%, respectively. 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.

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. 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. 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. 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.

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.

Pollen-Food Allergy Syndrome (PFAS) typically presents as mild oral symptoms but can rarely progress to systemic reactions. Pathogenesis-related proteins group 10 (PR-10) are proteins usually associated with oral allergy syndrome due to their thermolabile and gastrolabile properties. We report a 12-year-old boy with birch pollen allergy who developed anaphylaxis after raw carrot consumption without identified cofactors. Previously, he experienced only oral allergy syndrome with raw carrot and fennel. Molecular diagnostics confirmed isolated PR-10 sensitization. The reaction occurred during birch pollen season, and the patient tolerated cooked carrot, consistent with PR-10's thermal instability. This case highlights how PFAS, typically characterized by mild and localized symptoms, can occasionally manifest with systemic reactions. High levels of specific IgE for Bet v 1 and pollen season may represent risk factors for systemic reactions in PFAS patients. Clinicians must be aware that allergies due to pollen cross-reactive allergens can be associated with systemic reactions.

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. 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. There was a statistically significant increase in rate of oxygen use among residents (5.67; 95% CI 3.44-7.8; < 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; < 0.001). HTS use decreased during the study period, with a change in the prior trend to downward (-3.43; -5.89-9.68; = 0.007). There was no significant change in albuterol or pulmonary hygiene treatment. This study demonstrates that patterns of oxygen and HTS use in a pLTCF changed during the COVID-19 pandemic.

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. 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 -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. 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, < 0.0001). A strong correlation was observed between the FEV1/FVC ratio and FEF50/0.5FVC (r = 0.88, < 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. 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.

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. 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. 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.

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.

Interferon regulatory factor-2 binding protein 2 () is an important new transcriptional cofactor that interacts with IFN regulatory factor 2 (IRF-2) and an IRF-2-dependent transcriptional repressor. plays a role in different cellular functions, including apoptosis, survival, and cell differentiation. In this study, we report a case with common variable immunodeficiency (CVID), which has a heterozygous variant in the gene. A 13-year-old girl was evaluated for immunodeficiency due to recurrent sinusitis and tonsillitis in the previous year. She had been suffering from chronic cough for 3 months. She was hospitalized with lobar pneumonia and bronchiectasis. She was the second child of consanguineous parents. On physical examination, there was no growth and development retardation. Immunological screening of the patient demonstrated panhypogammaglobulinemia with low total memory B and class-switching memory B cells. Specific antibody responses to rubella and hepatitis B were negative. T- and B-lymphocyte counts and T-cell responses to phytohemagglutinin (PHA) were normal. Exome sequencing identified a heterozygous variant in (c.112C>Tp.Arg38Cys). On follow-up, she has maintained a good infection control with antibiotic prophylaxis and immunoglobulin replacement therapy. To the best of our knowledge, this case is the youngest CVID who was diagnosed with in the literature. The low percentage of total memory and switched memory B cells in the proband suggested that might have had a role in the development or survival of memory B cells. Functional studies are needed about the critical role of protein in B-cell maturation and humoral immune responses.

Aquagenic urticaria (AU) is a rare form of physical urticaria, characterized by pruritic skin eruptions triggered by water exposure, irrespective of the water's temperature. Fewer than 100 cases have been documented. The wet towel provocation test is the diagnostic standard for AU. A 12-year-old female patient presented with urticarial eruptions induced by water exposure and was diagnosed with AU following the wet towel provocation test. Initially, she tolerated brief water exposure; however, over time, eruptions occurred even with exposure to her sweat. Partial symptom control was achieved through preventive measures and antihistamine therapy. AU is a rare chronic condition that necessitates prompt diagnosis and tailored management strategies to improve the patient's quality of life.

Butter and beef are often prejudicially forbidden for children affected by IgE-mediated cow's milk allergy (IgE-CMA). Children with IgE-CMA underwent prick-by-prick (PbP) test with clarified butter and normal butter, raw and cooked beef. In case of positivity of the PbP, an oral food challenge (OFC) with that food was proposed. Thirty children with IgE-CMA were enrolled. Twenty-five (83%) introduced clarified butter into their diet; the remaining 5 did not want to do so. Fifteen patients (50%) were authorized to introduce normal butter into their diet; 1 patient (3%) was allergic to normal butter; for 14 patients (47%), allergy to normal butter remained uncertain. All 30 patients enrolled ate cooked beef without any problem. No one introduced raw or undercooked beef in his own diet for reasons of hygiene and taste. It is advisable to perform a PbP and eventually an OFC with normal butter, clarified butter, and cooked beef in any child affected by IgE-CMA.

Although the forced oscillation technique has been used for many years in children, there is still inconclusive results about its efficiency in cystic fibrosis (CF). Moreover, no studies have been conducted on impulse oscillometry (IOS) in children with primary ciliary dyskinesia (PCD). Age, sex, weight, height, body mass index, and oscillometric parameters were compared in 3-6-year-old children with CF and PCD and healthy children. This prospective study included 27 children with CF, 21 with PCD, and 27 healthy children, with mean ages of 4.11 ± 1.08, 4.33 ± 1.23, and 4.41 ± 0.79 years, respectively. No significant differences were revealed in the comparison of the z-scores of the parameters of the CF group with those of the healthy group. However, in the PCD group, z-scores of R5 and Z5 were significantly higher than those in the healthy group ( = 0.018 and = 0.008, respectively). In addition, z-scores of X10, X15, and X20 were significantly lower in children with PCD compared with the healthy group ( = 0.013, = 0.033, and = 0.029, respectively). This first study simultaneously reporting IOS results in preschool children with CF or PCD showed a significant difference of resistance and reactance of airways between PCD and healthy children. This study is also very significant in showing that IOS can be performed in young children who are unable to cooperate with spirometry. In contrast, no such differences were noted between CF and healthy controls, possibly due to thick mucus affecting sound wave transmission through the airways in CF. In addition, IOS may be less effective in detecting early pulmonary disease, as in some studies it failed to identify abnormalities in young children with CF even when spirometry is abnormal.

CD71+ erythroid cells (CECs) play a complex immunoregulatory role. Flow cytometry was used to assess CECs in 84 individuals, from preterm infants to adults, divided into 5 age groups, 12 from infants under 6 months, 16 from young children (6 months-5 years), 24 from children (6-10 years), 22 from adolescents (11-19 years), and 10 from adults (≥20 years). CECs are universally present at very low frequencies, except in individuals younger than 6 months ( < 0.001). Although preterm infants tended to have a higher median CECs than full-term infants, this difference was not statistically significant. Among individuals over 6 months, CECs showed significant negative correlations with hemoglobin (r = -0.448, < 0.001) and hematocrit (r = -0.416, < 0.001), but no such correlations were found in younger infants. CECs are present across all age groups, with higher levels in early infancy.

The atopic march refers to the progression of allergic disorders from atopic dermatitis (AD) in early childhood to food allergies, eosinophilic esophagitis (EOE), and later respiratory allergies such as asthma and allergic rhinitis. Mutations in the filaggrin gene, which compromise skin barrier function, are strongly associated with this progression and contribute to allergic sensitization. We introduce a 7-year-old boy with severe AD, food anaphylaxis, EOE, allergic rhinitis, and asthma associated with a filaggrin mutation (c.5152C>T). His clinical course illustrates the atopic march, with initial skin involvement progressing to gastrointestinal and respiratory allergic manifestations. Understanding the genetic factors that drive this progression may help identify targets for early intervention to prevent the further development of allergic disease.

The assessment of breathing reserve (BR) is essential to determine if ventilatory function is limited during exercise. Few studies reported the values of BR in healthy children and adolescents of both sexes. This study aimed to analyze the effects of age and sex on changes in BR in healthy untrained children and adolescents during growth. A study was conducted in 186 healthy untrained children and adolescents (10-16 years old). Maximal ventilation () and maximal oxygen uptake (O) were measured during a maximal graded test. Forced vital capacity and forced expiratory volume in 1 s (FEV) were also measured. BR was expressed as a percentage of theoretical maximal voluntary ventilation (MVV) which was obtained by multiplying FEV by 35. The data were assessed by one-way analysis of variance (ANOVA) and two-way ANOVA supplement a Newman-Keuls test when was significant ( < 0.05). Height, weight, and lean body mass increased between 11 and 16 years old. For boys,O, , FEV, and MVV increased ( < 0.05) between 12 and 16 years. For girls, O increased ( < 0.05) only between 11 and 12 and 14 and 15 years. Increases in , FEV, and MVV were observed between 10 and 12 years and stabilized afterward. The BR in girls was higher than in boys ( < 0.05). There were no significant differences in BR at the same age correlated with sex during growth. No significant changes were observed in BR as a function of age. BR changes during growth seem to be independent of changes in anthropometric characteristics.