Pediatric Allergy Immunology and Pulmonology最新文献

Background: 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. Case Presentation: 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. Conclusion: 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.

Introduction: 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. Methods: 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 t-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. Results: 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, P < 0.0001). A strong correlation was observed between the FEV1/FVC ratio and FEF50/0.5FVC (r = 0.88, P < 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. Conclusion: 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.

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.

Background: Interferon regulatory factor-2 binding protein 2 (IRF2BP2) is an important new transcriptional cofactor that interacts with IFN regulatory factor 2 (IRF-2) and an IRF-2-dependent transcriptional repressor. IRF2BP2 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 IRF2BP2 gene. Case Presentation: 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 IRF2BP2 (c.112C>Tp.Arg38Cys). On follow-up, she has maintained a good infection control with antibiotic prophylaxis and immunoglobulin replacement therapy. Conclusion: To the best of our knowledge, this case is the youngest CVID who was diagnosed with IRF2BP2 in the literature. The low percentage of total memory and switched memory B cells in the proband suggested that IRF2BP2 might have had a role in the development or survival of memory B cells. Functional studies are needed about the critical role of IRF2BP2 protein in B-cell maturation and humoral immune responses.

Background: 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. Case Presentation: 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. Conclusions: 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.

Background: Chronic urticaria (CU) in children, persisting beyond six weeks, is less common than acute urticaria, with a prevalence of 0.1%-0.3%. CU is classified into chronic idiopathic urticaria (CIU) and chronic inducible urticaria (CIndU), with CIU having an often unknown etiology, whereas CIndU is triggered by physical stimuli. Our study aims to explore the clinical and demographic characteristics, laboratory results, and possible etiological factors in children diagnosed with CU, and to assess the necessity of aeroallergen skin prick tests (SPTs) in these patients. Methods: The study evaluated the medical records of 242 children with CU, treated at the Pediatric Allergy-Immunology Clinic of Prof. Dr. Cemil Taşcıoğlu City Hospital from January 2018 to January 2024. Data on age, gender, presence of angioedema, dermatographism, concomitant allergic diseases, family history of atopy, infection status, urticaria duration, SPT results, and laboratory tests were collected and analyzed. Results: Results showed that 48.3% of patients were females and 51.7% were males, with an average age of 12.8 years and an average onset age of 9.9 years. Angioedema was present in 15.7% of patients, dermatographism in 17.8%, concomitant allergic diseases in 24%, and a family history of atopy in 14.5%. Infections were documented in 10.8% of patients, with urinary tract infections, Helicobacter pylori (H. pylori) infections, and dental infections identified. Discussion: Our study found no significant differences in clinical features, treatment requirements, or response to treatments between patients with positive and negative SPT results. Laboratory parameters such as eosinophilia, total Immunoglobulin E (IgE), and thyroid function tests also showed no significant differences. These findings suggest that routine SPTs for the management of CU in children will only be useful if IgE-mediated allergic comorbidities are suspected but may not be necessary in other cases, prompting a reevaluation of their use in clinical practice to seek more cost-effective diagnostic methods.

Background: CD71+ erythroid cells (CECs) play a complex immunoregulatory role. Methods: 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). Results: CECs are universally present at very low frequencies, except in individuals younger than 6 months (P < 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, P < 0.001) and hematocrit (r = -0.416, P < 0.001), but no such correlations were found in younger infants. Conclusions: CECs are present across all age groups, with higher levels in early infancy.

Introduction: 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. Case Presentation: 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. Conclusion: Understanding the genetic factors that drive this progression may help identify targets for early intervention to prevent the further development of allergic disease.

Background: 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. Methods: A study was conducted in 186 healthy untrained children and adolescents (10-16 years old). Maximal ventilation ($\dot{V}$_Emax) and maximal oxygen uptake ($\dot{V}$O_2max) were measured during a maximal graded test. Forced vital capacity and forced expiratory volume in 1 s (FEV₁) were also measured. BR was expressed as a percentage of theoretical maximal voluntary ventilation (MVV) which was obtained by multiplying FEV₁ by 35. The data were assessed by one-way analysis of variance (ANOVA) and two-way ANOVA supplement a Newman-Keuls test when P was significant (P < 0.05). Results: Height, weight, and lean body mass increased between 11 and 16 years old. For boys,$\hspace{0.17em}\dot{V}$O_2max, $\dot{V}$_Emax, FEV₁, and MVV increased (P < 0.05) between 12 and 16 years. For girls, $\dot{V}$O_2max increased (P < 0.05) only between 11 and 12 and 14 and 15 years. Increases in $\dot{V}$_Emax, FEV₁, and MVV were observed between 10 and 12 years and stabilized afterward. The BR in girls was higher than in boys (P < 0.05). There were no significant differences in BR at the same age correlated with sex during growth. Discussion: 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.

Background: 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). Methods: 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. Results: 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 (P = 0.018 and P = 0.008, respectively). In addition, z-scores of X10, X15, and X20 were significantly lower in children with PCD compared with the healthy group (P = 0.013, P = 0.033, and P = 0.029, respectively). Conclusions: 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.