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Introduction: Poikiloderma with neutropenia (PN) is a rare autosomal recessive hereditary disease caused by biallelic mutations of the USB1 gene. It is characterized by poikiloderma, chronic noncyclic neutropenia, and recurrent sinopulmonary infections with bronchiectasis. Here we report a case with homozygous c.531delA mutation in USB1 gene. Case: An 15-month-old boy was admitted to our clinic with skin hyperpigmentation, growth retardation, and recurrent lower respiratory tract infections. The medical history revealed that he was hospitalized 6 times due to pneumonia since the age of 3 months. His physical examination showed facial dysmorphism with triangular face, depressed nasal bridge, and frontal bossing. He also had poikiloderma in the whole body. Skin biopsy was performed and showed only hyperkeratosis. His weight and height were below the 3 percentile. He is the first child of his consangenius parents. In the laboratuary findings; he has mild neutropenia (1,100/mm 3 ), hypogammaglobulinemia (serum IgG: 351 mg/dL, IgA: 17 mg/dL, IgM: 20 mg/dL) and, peripheral lymphocyte subset analysis was normal. Neutropenia was also observed in previous examinations (980-560-840/mm 3 ). Immunoglobulin replacement therapy and antibiotic prophylaxis were started. Exome sequence analysis showed the presence of known homozygous variant (c.351delA) in USB1 gene. Conclusion: Poikiloderma with neutropenia mainly affects the myeloid lineage. Unlike other patients in the literature, we observed hypogammaglobulinemia in addition to neutropenia in our patient. This case illustrated that it is important to monitor serum immunoglobulin levels in symptomatic patients with recurrent infections.

Purpose of Review: The rate of inborn errors of immunity (IEI) in the Middle East and North Africa (MENA) region is generally higher than in other parts of the world. IEI patients in MENA exhibit more severe disease phenotypes. One of the most important reasons for this is delayed diagnosis. In this review, we examine issues pertinent to primary, secondary, and tertiary physicians in diagnosing IEI in children and discuss the key points for pediatricians according to the MENA guideline., Recent Findings: Protocols and stepwise approaches designed by a panel of clinical immunologists included in the MENA-IEI registry network can help physicians facilitate the diagnosis of patients with IEI by providing recommendations. These recommendations for diagnostic approaches improve the care of patients within the MENA region and can also be applied to IEI patients in other parts of the world other regions., Summary: Physicians in the MENA region should be aware of IEI, obtain a detailed family history, request tests that can be ordered in primary care when IEI is suspected, and refer patients to clinical immunologists without delay. Primary and secondary care physicians should be aware that patients with IEI may present with noninfectious manifestations and increased infection frequency, severity, and atypical infections., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Although the gold standard for diagnosing beta-lactam antibiotic (BLA) allergy is the drug provocation test (DPT), there is no standardized protocol for children. Objective: We aimed to evaluate the clinical features and DPT results of children with a history of low-risk non-immediate reactions (NIR) to BLA who underwent initial direct single therapeutic dose challenge with a 5-day prolonged DPT. Methods: We retrospectively evaluated children ages 0-18 years with a history of low-risk NIRs to BLAs. On the first day of provocation, a single-dose DPT protocol without any skin test was administered at the clinic. The therapeutic dose was adjusted to not exceed the maximum single-unit dose (MSUD) for age and weight. The DPT protocol was administered with 100% of MSUD. To identify children with delayed reactions, the parents or caregivers were told to continue giving the medication at home for 5 days. Results: One hundred and nine children were included in this study. The median (interquartile range) age of the children was 62.5 months (26.5-94 months). Of the suspected drugs, the main culprit drug was amoxicillin-clavulanic acid for 89 children (81.7%). The most common clinical manifestation was maculopapular exanthema, which occurred in 85 children (78%), and 8 (7.3%) had a positive DPT result. Three children (2.8%) developed a reaction after the first DPT dose. The remaining children continued to use the suspected BLA at home. Five children (4.7%) developed a reaction while using the drug at home. All the children with positive DPT results developed mild cutaneous signs and presented with a reaction to amoxicillin-clavulanic acid. None had a systemic or severe cutaneous reaction. Conclusion: Initial direct single therapeutic dose challenge with a 5-day prolonged DPT is a useful and safe way to assess low-risk NIRs to BLAs in children.

Aim: Primary immunodeficiencies (PIDs) are a heterogeneous disorder group characterized by an impaired immune system, leading to an increased susceptibility to infections and a wide range of clinical manifestations, including gastrointestinal (GI) complications. This study aimed to assess the GI manifestations of PID patients and highlight the significance of atypical gastrointestinal symptoms in the early diagnosis of these patients., Methods: A retrospective analysis was conducted on pediatric patients diagnosed with PIDs at Selcuk University Medical Faculty from 2011 to 2021. The study focused on demographic data, clinical presentation, genetic mutations, and GI manifestations, including endoscopic evaluation. Patients were categorized according to the International Union of Immunological Societies (IUIS) PID classifications. Statistical analyses were performed to identify significant associations between PID types and GI manifestations., Results: The cohort comprised 101 patients, with 46% presenting with GI symptoms, including malnutrition and chronic diarrhea, as the most common findings. Primary antibody deficiency (PAD) emerged as the most prevalent PID with GI involvement, followed by combined immunodeficiencies (CID) with associated or syndromic features. Endoscopic evaluations revealed inflammatory bowel disease (IBD)-like colitis in a significant subgroup of patients. The analysis showed that some GI symptoms were more common in specific PID categories, highlighting the importance of early gastroenterological assessment in PID patients., Conclusion: Recognition of common GI symptoms in pediatric patients with PIDs may facilitate early diagnosis and prompt multidisciplinary management, potentially improving patient outcomes. The study highlights the necessity of considering PIDs in diagnosing persistent or severe GI symptoms in children.

Introduction: There are limited data regarding the characteristics and management of drug hypersensitivity reactions (DHR) in hospitalized children. This study aims to determine the prevalence, clinical features, and management of DHRs in pediatric inpatients., Methods: Children who had pediatric allergy consultation for suspected DHR during hospitalization in Ankara Bilkent City Hospital between August 1, 2020, and July 30, 2021, were included. Patient and reaction characteristics, culprit drugs, and management strategies were recorded. When possible, diagnostic tests (skin or provocation tests) were performed after discharge., Results: Among the 14,090 hospitalized children, 165 (72% male, median age: 106 months) underwent consultation for 192 suspected DHRs with 246 drugs. Cutaneous eruptions were the most common (94.3%). There was anaphylaxis in 40 patients and severe cutaneous adverse drug reaction in 4 patients (drug rash with eosinophilia and systemic symptoms in 3, acute generalized exanthematous pustulosis in 1). Antimicrobials were the leading cause (78.4%, n = 193/246). In 48 reactions, 60 (24%) culprit drugs could be readministered with close follow-up or desensitization (n = 12). In total, 186 suspected drugs were discontinued, and 115 were replaced with alternative drugs. After discharge, 38 provocation tests (2 positives) and 36 skin tests (1 positive prick test, 1 positive intradermal test, and 1 positive patch test) were performed., Discussion/conclusions: The incidence of suspected DHR among pediatric inpatients was approximately 1.1%. Skin symptoms were the most common manifestation. Twenty-four percent of suspected drugs could be continued during hospitalization. Patients with DHR during hospitalization should be evaluated with a drug allergy work-up unless there are contraindications to testing., (© 2024 The Authors.)

Background: Seeds are widely consumed as a traditional snack and have rich contents beneficial to health. With an increase in consumption rates, allergic reactions occur more frequently. We focus on multiple seed consumption related to recurrent anaphylaxis in this case. Case Presentation: We evaluated an 11-year-old boy with recurrent anaphylaxis. According to his medical records, he had been hospitalized several times, diagnosed with anaphylaxis, and treated. The family noticed direct (eating) or indirect contact with pumpkin seeds. In addition, the family mentioned another anaphylactic episode after watermelon seed and poppy seed bread consumption. We conducted skin prick-to-prick tests, examined total immunoglobulin E levels, and prescribed the treatment with an adrenalin autoinjector and preventive dietary recommendations. Conclusion: Anaphylaxis, particularly recurrent ones, should be evaluated with detailed anamnesis and supported with laboratory tests. Although seeds are beneficial and highly nutritious, it is necessary to consider them a source of allergens.

Background: The drug provocation test (DPT) and the oral food challenge (OFC) are considered as the criterion standard for the diagnosis of drug hypersensitivity reactions and food allergy. Severe allergic reactions may develop during these tests. Objective: To evaluate the frequency and features of anaphylaxis in pediatric patients undergoing OFCs and DPTs. Method: OFCs and DPTs performed in an open method in the pediatric allergy clinic of our institution between January 2014 and January 2021 were reviewed retrospectively. The characteristics of anaphylaxis that developed during these tests were evaluated. Results: A total of 3631 OFCs and/or DPTs were performed on 2588 pediatric patients. Reactions were recorded in 317 challenges (8.7%), including 42 (1.2%) in the form of anaphylaxis. Of the patients with anaphylaxis, 31 developed anaphylaxis during OFC and 11 during DPT. Anaphylaxis during OFCs was mostly triggered by yogurt (n = 8), hen's egg (n = 6), baked milk (n = 5), and baked egg (n = 4). Cases with anaphylaxis during DPT were recorded mostly with ibuprofen (54.5% [n = 6]). All patients who developed anaphylaxis during OFC had cutaneous manifestations, and 90.3% had respiratory symptoms. Gastrointestinal involvement was present in 32.3% of the patients. During DPT, cutaneous manifestations were observed in 90.9% in the patients who developed anaphylaxis and the respiratory tract was involved in 81.8%. In terms of concomitant allergic diseases, 51.6% of the patients who developed anaphylaxis during OFC had atopic dermatitis and 38.7% had asthma. All the patients with anaphylaxis triggered by nonsteroidal anti-inflammatory drug DPT had asthma. Of the anaphylaxis, 54.8% were mild, 35.7% were moderate, and 9.5% were severe. Severe anaphylaxis was recorded with baked milk (n = 2), baked egg and trimethoprim-sulfamethoxazole (n = 1, each). The patients did not require intensive care, and no death occurred. Conclusion: Anaphylaxis may develop during OFCs and DPTs. These tests should be carried out by experienced allergists in an appropriate setting where emergency equipment and medications, including epinephrine, are readily available.

Background: Drug provocation tests (DPTs) are the gold standard for the diagnosis of drug hypersensitivity reaction (DHR). To the best of our knowledge, there is no previous study reporting DPT-related anxiety levels in children and their parents. This study aimed to determine the difference in pre- and post-DPT anxiety levels of parents and children who were informed of the possibility of another DHR during the DPT, and to evaluate the relationship between parental psychological distress and anxiety levels. Methods: The study included children who underwent DPT in our clinic between July 1, 2019, and February 29, 2020, and accompanying parents who consented to participate. Age-appropriate State-Trait Anxiety Inventory scales were used to assess levels of state and trait anxiety in the patients and parents. The Symptom Checklist-90-Revised (SCL-90-R) was used to screen for psychological symptoms in parents. Results: Data were collected from the parents of 69 children who underwent DPTs. The patients' median age was 7.28 (interquartile range: 4.52-10.06) and their parents' mean age was 35.28 ± 5.38 years. Anxiety-related data were collected from 21 pediatric patients. The children and parents had higher state anxiety scores before DPT compared to after DPT. There was a positive correlation between the parents' trait anxiety and pre-DPT state anxiety scores. In addition, parental pre-DPT state anxiety scores were positively correlated with SCL-90-R general severity index, somatization, anxiety, obsessive-compulsive, and depression subscale scores. Conclusion: The risk of allergic reaction in DPT may cause anxiety. A high level of parental anxiety before DPT, which gradually decreased after negative test results, was associated with history of drug-induced anaphylaxis in their children and high trait anxiety. Appropriate evaluation of patients and parents before DPT and providing detailed information may be important to reduce this anxiety.

Background: Food allergies are known to resolve over time, but there is little information on the natural history of food-induced anaphylaxis (FIA). Objective: This study aimed to evaluate the natural history of FIA in children and determine the factors that affect prognosis. Methods: Children with FIA who were followed up for at least 3 years, between 2010 and 2020, were included. Patients' families were contacted by telephone to question their child's tolerance status and invite them for reevaluation if uncertain. The patients were grouped as tolerant or persistent according to parent reports or reevaluation results. Logistic regression analysis was performed to determine the factors that affected persistence. Results: The study included 185 patients (62.2% boys) with 243 anaphylactic reactions to various foods. Fifty-eight patients (31%) gained tolerance within a 3-year follow-up period. Tolerance rates were higher in patients with FIA to milk (40%) and egg (43.9%) compared with to tree nuts (18.8%), legumes (5.6%), and/or seafood (11.1%) (p < 0.001). In a multivariate analysis, risk factors for persistent FIA were multiple food anaphylaxis (odds ratio [OR] 3.755 [95% confidence interval {CI}, 1.134-12.431]; p = 0.030), total IgE > 100 kU/L (OR 5.786 [95% CI, 2.065-16.207]; p = 0.001), and skin-prick test wheal size > 10 mm (OR 4.569 [95% CI, 1.395-14.964]; p = 0 .012) at presentation. Conclusion: Approximately a third of the patients with FIA developed tolerance within 3 years. Clinicians should remember that children with food allergies, even anaphylaxis, may develop tolerance over time. Regular follow up and reevaluation of tolerance status are necessary to avoid unnecessary elimination.

Introduction: Anaphylaxis is a severe, potentially fatal systemic hypersensitivity reaction with an acute onset. Etiology, clinical presentation, risk factors, comorbidities of pediatric anaphylaxis may vary depending on the age of the child., Objective: The aim of this study was to investigate the etiology, clinical features, management of anaphylaxis in infants, preschoolers, school-age children, and adolescents., Methods: The patients presenting with anaphylaxis between January 2015 and December 2018 in a single pediatric tertiary hospital were evaluated retrospectively. Demographic data, the triggers, sign-symptoms, severity, and the management of anaphylaxis were recorded., Results: 239 patients were included in the study, 62.3% of whom were boys. The median age was 6.7 (IQR 2.33-12.83) years. 23.8% of the patients were infants, 15.5% were preschoolers, 33.5% were school-age children, and 27.2% were adolescents. Anaphylaxis mostly occurred at home. The most common causative agents were foods (39.3%), drugs (30.1%), and venoms (15.9%) of all cases. Main food allergens were cow's milk and hen's eggs in infants, cow's milk and tree nuts in preschoolers, and tree nuts and legumes in school-age children. Cases of drug-induced anaphylaxis (DIA) were recorded mostly with antibiotics (40.3%), followed by NSAIDs (23.6%). The primary trigger of anaphylaxis was foods in infants and preschoolers and drugs in school-age children and adolescents. There was no difference between age groups in terms of the system involved and severity. Severe anaphylaxis was more common with DIA. Adrenaline was used in 69.8% of all cases with no significant difference between age groups., Conclusion: Etiology and symptoms of anaphylaxis may differ between age groups. Raising awareness, educating patients and their parents on anaphylaxis and its management is essential., (© 2022 S. Karger AG, Basel.)

Allergic transfusion reactions (ATRs)are a common form of acute transfusion reaction. It was aimed to determine the clinical characteristics and frequency of ATRs in children. This study included children who were transfused with red cell concentrate (RCC), fresh-frozen plasma (FFP), platelet concentrates(PC), apheresis granulocyte, and cryoprecipitate.The patients' sociodemographic characteristics, the blood product that caused the reaction, the type and timing of the reaction, the patient's age at time of reaction and their diagnosis, follow-up period, and clinical data were recorded. A total of 89703 bags of blood products were transfused to 4193 children.Two hundred eleven acute transfusion-related reactions occurred in 157 (3.74%) patients.Of these, 125 reactions (59%) were allergic. ATR occurred in 125 of 89703 infusions (0.14%).The median age of patients was 9.99 years (IQR:4.67-14.38) and ATRs occurred at a median of 30 minutes into the transfusion. Eighteen (18%) of the patients also had a history of drug reaction.When the blood products that caused ATRs were examined, 43(34.5%) occurred with apheresis and single-donor PC, 37(29.6%) with FFP, 32 (25.6%) with RCC, 10(8%) with pooled PC, 2(1.6%) with cryoprecipitate, 1(0.8%) with apheresis granulocyte.Ninety-nine(79%) of the reactions were minor allergic reactions and 26(21%) were anaphylaxis.Compared to minor allergic reactions, the proportion of PCs was statistically higher in anaphylaxis(p=0.02). Patients receiving PC should be monitored more carefully during the first half hour of transfusion. In addition, approximately one-fifth of the patients who developed ATR also had a history of drug reaction. Patients with previous reactions to drugs may be more likely to have ATR., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Introduction: Limited data are available on recurrent anaphylaxis in childhood. Delayed adrenaline administration is the major cause of deaths due to anaphylaxis. As well as prescribing the adrenaline autoinjector (AAI), it is important to make sure that the patient carries the device at all times and uses it correctly for the appropriate indication. Objective: The aim of our study was to evaluate the recurrence of anaphylaxis and AAI use in childhood. Methods: Pediatric patients who were evaluated for anaphylaxis and prescribed AAI between January 2015 and December 2018, in the pediatric allergy and immunology clinic of our hospital were screened retrospectively. A telephone-based survey was conducted with the parents of the patients to investigate the recurrence of anaphylaxis in patients and the use of AAI by their parents for the management of anaphylaxis. Results: A total of 148 patients (64.9% boys) were prescribed an AAI for anaphylaxis. The telephone survey could be conducted with 111 parents (75%) with an AAI prescription. Of these patients, 23.4% (n = 26) of the parents reported that their children experienced recurrent episodes of anaphylaxis. In the recurrent anaphylaxis cases, the triggers were foods in 77%, venoms in 11.5%, drugs in 3.8%, and idiopathic anaphylaxis in 7.7% of the patients. AAI use at the time of anaphylaxis was reported by 42.3% of the parents. The reasons cited by the parents for not using AAI during an episode of anaphylaxis included the preference to have adrenaline administered at a health care facility because of its proximity (60%), fear of using the device (13.3%), hesitation (6.7%), not having the device with them (13.3%), and unavailability of the device (6.7%). Conclusion: Educating the patients and families about the importance of using AAI is crucial, and training on how to use the device should be repeated at each clinic visit and every opportunity.

Background: Currently, there are no reliable clinical tools available for predicting asthma in pre-school-aged children with recurrent wheezing. The aim of this study was to evaluate the usefulness of serum periostin, YKL-40, and osteopontin biomarkers in wheezy pre-school-aged children for predicting the development of asthma in school ages., Methods: The study was prospectively conducted between 2011 and 2017. The clinical features of the pre-school-aged children with recurrent wheezing and the levels of serum periostin, YKL-40, and osteopontin were measured. The same participants were reevaluated in school-age period, and participants with asthma were identified. Relative risk (RR) for the development of asthma was analyzed., Results: Of the 197 pre-school-aged children with recurrent wheezing who were reevaluated in school-age years, 32% of them had asthma. Serum periostin, YKL-40, and osteopontin levels at admission could not predict participants who would have asthma symptoms in school-age years. The RR for continuing of asthma symptoms was higher in participants who had their first wheezing episode before 1 year of age, preterm birth, cesarean section delivery, prenatal smoking exposure, multi-trigger wheezing, parental asthma, modified asthma predictive index positivity, prophylactic vitamin D intake ≤ 12 months, breastfeeding time ≤ 12 month, and aeroallergen sensitivity [RR (95% CI) and P value: 2.813 (1.299-6.091), 0.002; 1.972 (1.274-3.052), 0.009; 1.929 (1.195-3.114), 0.004; 2.232 (1.463-3.406), <0.001; 3.152 (1.949-5.097), <0.001; 1.730 (1.144-2.615), 0.016; 2.427 (1.559-3.777), <0.001; 2.955 (1.558-5.604), <0.001; 1.767 (1.084-2.881), 0.016; 0.765 (0.556-1.053), 0.016; respectively]., Conclusion: Results have shown that clinical features were more valuable than biomarkers in predicting having asthma in school-age years in participants who had recurrent wheezing in pre-school-age period., (© 2020 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.)

Introduction: Beta-lactams (BLs) are one of the most frequent causes of drug hypersensitivity reactions (HRs), and cephalosporins are a widely used subclass of BLs, especially in children. The aim of this study was to evaluate the clinical features and diagnostic test results of pediatric patients evaluated for suspected cephalosporin allergy., Methods: This study included patients who presented to our pediatric allergy clinic with a history of reactions attributed to cephalosporins between January 1, 2011, and December 31, 2019, and whose diagnostic tests were completed for the diagnosis., Results: This study included 120 pediatric patients and 69 (57.5%) of them were girls. The median age was 38.63 (interquartile range 10.5-85.7) months. Reactions occurring within 1 h of drug intake were reported in 33 patients (27.5%). Reactions were maculopapular rash in 55 (45.8%) patients, urticaria and/or angioedema in 49 (40.8%), anaphylaxis in 11 (9.2%), severe cutaneous drug reaction in 4 (3.3%), and fixed drug reaction in 1 patient (0.83%). The most frequently suspected agent was cefixime in 41 patients (34.2%). In total, 30 (25%) patients were diagnosed as having cephalosporin hypersensitivity. Confirmation of HRs was also significantly more frequent among patients who were older (p: 0.000), who had taken the drug parenterally (p: 0.000) and with immediate reactions (p: 0.000)., Conclusion: Cephalosporin allergy has been confirmed in approximately one-fourth of the patients evaluated for suspected cephalosporin allergy. Confirmation of HRs was significantly more common among patients who were older, had immediate reactions, and had taken the drug parenterally., (© 2021 S. Karger AG, Basel.)

Background: The first-line method in the diagnosis of patients who describe an immediate reaction after penicillin intake is a skin test (ST) with penicillin reagents. Objectives: We aimed to determine the safety and diagnostic value of penicillin STs in the diagnosis of immediate reactions to penicillins in pediatric patients. Methods: The study included pediatric patients with suspected immediate reaction to penicillin who were subjected to STs by using a standard penicillin test kit as well as suspected penicillin and the drug provocation tests (DPT) with the suspected penicillin at our clinic. Results: A total of 191 patients (53.9% boys) with a median age of 6.83 years (interquartile range, 4.2-12 years) were included in the study. The time from drug intake to the onset of reaction was ≤1 hour in 138 patients (72.3%) and 1 to 6 hours in 53 patients (27.7%). Penicillin allergy (PA) was confirmed by diagnostic tests in 36 of the 191 patients (18.8%). In multivariate logistic regression analysis, the history of both urticaria and angioedema (odds ratio [OR] 27.683 [95% confidence interval {CI}, 3.143-243.837]; p = 0.003) and anaphylaxis (OR 56.246 [95% CI, 6.598-479.489]; p < 0.001) were the main predictors of a PA diagnosis. Although ST results were positive in 23 patients (63.8%), 13 patients (26.2%) had positive DPT results despite negative ST results. The negative predictive value (NPV) of STs was calculated 92.2% (155/168). None of our patients experienced immediate or delayed systemic and/or local reactions in relation to the STs. Conclusion: A history of urticaria with angioedema and anaphylaxis were the main predictors of true PA in children with suspected immediate reactions. STs with penicillin reagents are safe for use in children. Although STs have a high NPV, DPT is the gold standard for diagnosis. DPTs should be performed as the final step of the diagnostic evaluation of PA in patients with negative ST results.

Background: Intravenous recombinant enzyme replacement therapy (ERT) is currently available for 8 lysosomal diseases. Hypersensitivity reactions (HSRs) may be observed during this long-term treatment., Objective: To evaluate the frequency and clinical treatment features of ERT HSRs and the management of desensitizations in children., Methods: Medical records were reviewed retrospectively for patients who received ERT. Those who had experienced HSRs to ERT were included in the study. The demographic characteristics of the patients, culprit enzyme, signs and symptoms, diagnostic tests, management of the reaction, and the protocol employed for the maintenance of ERT were recorded., Results: During the study period, 54 patients received ERT in our institution. A total of 11 patients (20.4%) experienced HSR to ERT. All reactions were of immediate type. The most common symptoms were cutaneous manifestations. A total of 9 patients experienced urticaria, and 2 had anaphylaxis as initial reaction. Patients who had isolated cutaneous symptoms continued their treatments with antihistamines, corticosteroid premedication, slower infusion rate or both. Patients who had recurrent urticaria with these modalities or those who had anaphylaxis continued their ERT with desensitization (n = 8). A total of 3 patients required revisions in desensitization protocols because of recurrent anaphylaxis., Conclusion: The reactions that develop during this long-term treatment may be treated by premedication-prolonged infusion, but in some patients, desensitization protocols are necessary for the continuation of therapy. Revisions in desensitization protocols may be required., (Copyright © 2020 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Background: Nonsteroidal anti-inflammatory drugs (NSAIDs) are commonly used in children and can frequently cause hypersensitivity reactions. Rates of confirmed NSAID hypersensitivity (NSAID-H) in children are low., Objective: To evaluate the results of drug provocation tests (DPTs) with NSAIDs and to evaluate the difficulties encountered in the classification of NSAID-H in children., Methods: The study included patients with suspected NSAID-H who were examined in our clinic between January 1, 2015, and December 31, 2018. Oral provocation tests with NSAIDs were performed and reactions were classified according to the European Academy of Allergy and Clinical Immunology position paper on NSAID-H., Results: A total of 243 patients (57.2% male patients) presented with suspected NSAID-H during the study period. Of these, 168 patients (69.1%) had a history of reaction to ibuprofen. Isolated skin involvement was the most frequent symptom (86%). A total of 238 DPTs were performed with the suspected agents and 34 had positive results. The families of 12 patients refused provocation testing with the suspected agent or aspirin and these patients could not be diagnosed. Of the 231 patients, 47 patients (20.3%) received a diagnosis of NSAID-H. Twenty patients with NSAID-H could not be classified because their guardians did not consent to further testing with aspirin., Conclusion: Performing diagnostic tests is important in patients with no contraindications. Characterizing these reactions in children can be difficult because of the coexistence of indistinguishable symptoms in their history and DPTs, as well as the need for multiple provocation tests. Therefore, further research is needed on this subject., (Copyright © 2020 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Background: Extending the drug provocation test (DPT) period is recommended for patients with suspected nonimmediate beta-lactam antibiotic (BLA) allergy and negative DPT. No consensus has been reached regarding the duration of prolonged provocation., Objective: We aimed to determine the negative predictive value (NPV) of the 5-day extended DPT., Methods: Parents of patients with suspected nonimmediate mild cutaneous reactions with BLAs who had been subjected to 5-day DPT with culprit drugs were questioned by telephone interview about reexposure to the tested drug. Patients with reported reaction during reexposure were reevaluated. Skin tests and serum-specific immunoglobulin E (IgE) analysis were not performed before first DPT., Results: A total of 355 patients had negative results in 5-day DPT. The median age at DPT was 4.2 years, and 52.9% were male. The families of 255 patients (72%) could be contacted. Of these 255 patients, 179 (70%) had used the same drug, and reactions were reported for 6 (3.4%) of those patients, who were subsequently reevaluated. Five of the 6 patients had DPT with amoxicillin-clavulanate and 1 with cefixime. When detailed history was taken, 2 of the 5 patients with amoxicillin-clavulanate reaction were found to have used the drug unintentionally after their reaction to reexposure and did not have any symptoms. One of the patients underwent allergy workup and tested negative, and the other 2 refused the test. The patient with reported cefixime reaction underwent repeated allergy workup and tested negative. Therefore, the NPV of 5-day prolonged DPT was 98.9%., Conclusion: The 5-day prolonged DPT has high NPV and seems appropriate in duration for children with suspected nonimmediate-BLA allergy., (Copyright © 2020 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Objective: Continued progress in our understanding of the food protein-induced allergic proctocolitis (FPIAP) will provide the development of diagnostic tests and treatments. We aimed to identify precisely the clinical features and natural course of the disease in a large group of patients. Also, we investigated the predicting risk factors for persistent course since influencing parameters has not yet been established., Methods: Infants who were admitted with rectal bleeding and had a diagnosis of food protein-induced allergic proctocolitis in 5 different allergy or gastroenterology outpatient clinics were enrolled. Clinical features, laboratory tests, and prognosis were evaluated. Risk factors for persistent course were determined by logistic regression analyses., Results: Among the 257 infants, 50.2% (n = 129) were girls and cow's milk (99.2%) was the most common trigger. Twenty-four percent of the patients had multiple food allergies and had more common antibiotic use (41.9% vs 11.8%), atopic dermatitis (21% vs 10.2%), wheezing (11.3% vs 1.5%), colic (33.8% vs 11.2%), and IgE sensitization (50% vs 13.5%) compared to the single-food allergic group (P < 0.001, P = 0.025, P = 0.003, P < 0.001, respectively). In multivariate logistic regression analysis, presence of colic (odds ratio [OR]: 5.128, 95% confidence interval [CI]: 1.926-13.655, P = 0.001), IgE sensitization (OR: 3.964, 95% CI: 1.424-11.034, P = 0.008), and having allergy to multiple foods (OR: 3.679, 95% CI: 1.278-10.593, P = 0.001] were found to be risk factors for continuing disease after 1 year of age., Conclusion: Although most children achieve tolerance at 1 year of age, IgE sensitization, allergy to multiple foods, and presence of colic were risk factors for persistent course and late tolerance. In this context, these children may require more close and extended follow-up.

Introduction : Drug allergy is an important public health problem that causes 0.4-10.3% of hospital admissions in children. The epidemiology, clinical spectrum, diagnosis, and management of drug hypersensitivity reactions (DHRs) differ in pediatric and adult patients. However, the same algorithms used in adults have often been applied in the pediatric population due to a lack of evidence in the literature. Areas covered : In this review, we aimed to discuss recent developments in the area of pediatric DHRs based on the growing body of literature advancing our understanding of the epidemiology, clinical aspects, and diagnostic approaches to DHRs in children. Expert opinion : Misdiagnosis, under-diagnosis, and self-diagnosis are common problems related to drug allergies in children. Viral infections are particularly frequent in children and make diagnosis difficult. Identifying true DHR is a key step in the management of drug allergy in children. Therefore, a complete allergy work-up with standardized drug allergy tests is necessary. In order to eliminate non-standardized tests, future studies including larger numbers of children should be conducted to determine more accurate standardized tests for diagnosing DHRs in the pediatric population.

Objectives: In the study, the effect of valproic acid on serum free/acylcarnitine levels and left ventricular systolic function in pediatric patients with idiopathic epilepsy receiving valproic acid was investigated., Patients and Methods: Patients receiving valproic acid treatment for six months between January 2012 and December 2012 were evaluated. Blood samples were obtained from the participants twice (pretreatment and the sixth month of treatment) and serum-free and acylcarnitine levels (from C2 to C18:1-OH) were measured using tandem mass spectrometry. Cardiac functions (ejection fraction, shortening fraction, cardiac output, left ventricular systolic and diastolic diameters, left atrial diameter, aortic diameter, cardiac output, and myocardial performance index) were evaluated by echocardiography simultaneously., Results: A total of fourty patients, 23 female (57.5%) and 17 male (42.5%), with the diagnosis of idiopathic epilepsy and receiving valproic acid monotherapy were studied. Comparison of serum-free and acylcarnitine levels measured pretreatment and sixth month of treatment revealed a decrease in average C0 and C5:1 (respectively p < 0.001, p = 0.013) and an increase in C2, C3, C5-OH, C8:1 and C4-DC levels (respectively p < 0.001, p < 0.001, p = 0.019, p = 0.013, p < 0.001). Other serum acylcarnitine levels did not change significantly (p > 0.05). No difference was observed in concurrent echocardiographic measurements of left ventricular systolic function (p > 0.05)., Conclusion: The study demonstrated that valproic acid treatment results in low levels of free carnitine and changes in some acylcarnitine subgroups but has no influence on left ventricular systolic function., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Copyright of Firat Tip Dergisi is the property of Firat University, Faculty of Medicine and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

A proper allergy work-up, based on the gold standard drug provocation test (DPT), usually rules out suspected drug hypersensitivity in children. These tests are generally open, given their high efficiency compared with double-blind placebo-controlled DPTs. Although their negative predictive value is excellent, no studies have calculated their positive predictive value, highly dependent on the prevalence of the disease. Most studies have found a rate of <5%-10% of true beta-lactam hypersensitivity in children. Given this low prevalence (pre-test probability), a few false-positive results can significantly reduce the estimated positive predictive value. False positives may arise from the nocebo effect during the test, including nocebo by proxy, or from observer bias, which depends on professional expertise and organizational circumstances. Some studies have found a high rate of tolerance on a second DPT in children who failed the first, but these results may be affected by the interval between the two tests, of a year or more in most cases, reflecting a loss of hypersensitivity over time. Taking into account the low rate of positive DPTs, with commonly mild reactions, we suggest confirming nonsevere positive DPTs with a second provocation performed soon after the first, especially in the case of beta-lactam antibiotics, in order to improve the diagnostic accuracy, de-label more patients, and achieve a better estimation of true drug hypersensitivity prevalence. In case of mild immediate reactions, the potential benefits of a second DPT should be carefully weighed against the risk of anaphylaxis., (© 2023 European Academy of Allergy and Clinical Immunology and John Wiley & Sons Ltd.)

Background/aim: The transfusion of blood products is a life-saving clinical practice in patients with bleeding, hemoglobinopathy, and cancer. It was aimed herein to analyze the frequency and types of blood component-related acute transfusion reactions (ATRs) in pediatric patients. Materials and methods: This retrospective study was conducted at a tertiary care academic pediatric hospital. Results: During the study period, 30,811 transfusions were administered to 25,448 patients. There were 103 ATRs detected in 81 patients (0.33%; 3.34 reactions per 1000 transfusions, mean age 8.3 ± 5.98 years, 36 females and 45 males). All the reactions were observed within an average of 4 h after the transfusion began. The most common ATRs were allergic reactions (79; 76.6%) and febrile nonhemolytic transfusion reactions (12; 11.6%). All the allergic transfusion reactions occurred within the first hour after the start of the transfusion. Granulocyte concentrates were the blood component associated with the highest ATR rate (2.1%). Conclusion: Within our hospital, pediatric hematology-oncology wards and the stem cell transplantation unit had the most frequent ATR reports; therefore, when transfusions are carried out, increased attention should be given to these units. Educating health staff about the adverse effects of transfusion therapy should increase the awareness and reporting of ATRs in children. [ABSTRACT FROM AUTHOR]

Background: Ibuprofen is one of the most commonly used analgesic and antipyretic drugs in children. However, its potential causal role in childhood asthma pathogenesis remains uncertain. In this systematic review, we assessed the association between ibuprofen administration in children and the risk of developing or exacerbating asthma. Methods: We searched MEDLINE, Embase, Cochrane Library, CINAHL, Web of Science, and Scopus from inception to May 2022, with no language limits; searched relevant reviews; and performed citation searching. We included studies of any design that were primary empirical peer-reviewed publications, where ibuprofen use in children 0–18 years was reported. Screening was performed in duplicate by blinded review. In total, 24 studies met our criteria. Data were extracted according to PRISMA guidelines, and the risk of bias was assessed using RoB2 and NOS tools. Quantitative data were pooled using fixed effect models, and qualitative data were pooled using narrative synthesis. Primary outcomes were asthma or asthma-like symptoms. The results were grouped according to population (general, asthmatic, and ibuprofen-hypersensitive), comparator type (active and non-active) and follow-up duration (short- and long-term). Results: Comparing ibuprofen with active comparators, there was no evidence of a higher risk associated with ibuprofen over both the short and long term in either the general or asthmatic population. Comparing ibuprofen use with no active alternative over a short-term follow-up, ibuprofen may provide protection against asthma-like symptoms in the general population when used to ease symptoms of fever or bronchiolitis. In contrast, it may cause asthma exacerbation for those with pre-existing asthma. However, in both populations, there were no clear long-term follow-up effects. Conclusions: Ibuprofen use in children had no elevated risk relative to active comparators. However, use in children with asthma may lead to asthma exacerbation. The results are driven by a very small number of influential studies, and research in several key clinical contexts is limited to single studies. Both clinical trials and observational studies are needed to understand the potential role of ibuprofen in childhood asthma pathogenesis. [ABSTRACT FROM AUTHOR]

Purine nucleoside phosphorylase (PNP) deficiency is a rare immunodeficiency syndrome generally characterized by profound T cell deficiency and variable B cell function. More than half of PNP-deficient patients present with neurological dysfunction, with manifestations such as mental and motor retardation, spasticity, hypertonia, ataxia, and behavioral disturbances. Here, we report two siblings diagnosed with PNP deficiency in early infancy. Our patients had developmental delays, and their immunological findings indicated T-B+NK+ leaky/atypical severe combined immune deficiency. The patients are being treated with regular intravenous immunoglobulin replacement, as well as trimethoprim-sulfomethoxazole and fluconazole, for prophylaxis in preparation for transplantation. These cases draw attention to the possibility of primary immune deficiency in patients with recurrent infections and lymphopenia. In addition, PNP deficiency should be kept in mind in the presence of developmental delay, low uric acid levels, and lymphopenia. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Pediatric Disease / Türkiye Çocuk Hastalıkları Dergisi is the property of Turkish Journal of Pediatric Disease and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: Arylpropionic acid derivatives (APs) are the main triggers of nonsteroidal anti-inflammatory drug (NSAID) hypersensitivity. Data on clinical patterns and risk factors for AP hypersensitivity in children are quite limited., Aim: To assess the clinical characteristics and potential risk factors for proven AP hypersensitivity in children., Method: Patients with a history of AP hypersensitivity were retrospectively assessed using a standardized diagnostic algorithm. Children with confirmed hypersensitivity were defined as selective responders or cross-intolerants based on the result of drug provocation tests and further categorized according to the EAACI/ENDA classification. A multivariable logistic regression analysis was performed to analyze the potential risk factors for proven AP hypersensitivity., Results: A total of 166 patients (51.2% male, median age of six years) with a history of AP hypersensitivity were included. Ibuprofen (89.2%) was the most frequently reported AP in the patients' histories. The reported hypersensitivity of 40 (22.4%) patients was confirmed by diagnostic testing: eight (13.6%) patients with a history of reaction only to APs and 32 (29.9%) patients with a history of reactions to multiple NSAIDs, including chemically unrelated NSAIDs in addition to APs. Five (12.5%) patients were classified as selective responders and 35 (87.5%) were cross-intolerants. Overall, five (12.5%) of the confirmed cases could not be categorized according to the EAACI/ENDA classification. Older age (aOR: 1.11, 95% CI 1.02-1.21, p = 0.015), chronic urticaria as an underlying disease (aOR: 2.87, 95% CI 1.09-7.54, p = 0.033) and a history of anaphylaxis (aOR: 7.84, 95% CI 1.86-33.04, p = 0.005) were related to confirmed AP hypersensitivity., Conclusion: Almost a quarter of children and adolescents were confirmed to have AP hypersensitivity. Older age, the presence of chronic urticaria and a history of anaphylaxis were potential risk factors for proven AP hypersensitivity., Competing Interests: Conflicts of interest The authors declare that they have no conflict of interest., (© 2024. The Author(s), under exclusive licence to Springer Nature Switzerland AG.)

Pulmonary complications are one the main causes of morbidity and mortality in patients with common variable immunodeficiency (CVID). Although CVID pathogenesis is not completely understood, several genes have been identified to mainly regulate the process of terminal B-cell differentiation, antibody isotype maturation and long-life memory/plasma cell generation. The link between underlying genetic defects and the prognosis of developing different clinical complications like different pulmonary manifestations is still elusive. We provide an overview of recent advancements in the monogenic form of CVID which lead to dysregulation of B-cells at different levels of cytokine stimulations, intracellular signaling, transcriptionfactor activation, gene transcription and epigenetic controls and predispose patients to different pulmonary complications. The susceptibility to Coronavirus disease 2019 pulmonary complications was discussed in these patients. Monogenic forms of CVID have a distinct pattern in different infectious and non-infectious pulmonary manifestations including autoimmunity, atopy, lymphoproliferation, and cancer. [ABSTRACT FROM AUTHOR]

Drug-induced anaphylaxis in children is less common than in adults and primarily involves beta-lactams and nonsteroidal anti-inflammatory drugs. Epidemiological studies show variable prevalence, influenced by age, gender, and atopic diseases. The pathophysiology includes IgE-mediated reactions and non-IgE mechanisms, like cytokine release reactions. We address drug-induced anaphylaxis in children, focusing on antibiotics, nonsteroidal anti-inflammatory drugs, neuromuscular blocking agents, and monoclonal antibodies. Diagnosis combines clinical criteria with in vitro, in vivo, and drug provocation tests. The immediate management of acute anaphylaxis primarily involves the use of adrenaline, coupled with long-term strategies, such as allergen avoidance and patient education. Desensitization protocols are crucial for children allergic to essential medications, particularly antibiotics and chemotherapy agents. [ABSTRACT FROM AUTHOR]

Food-protein-induced allergic proctocolitis (FPIAP) is an increasingly reported transient and benign form of colitis that occurs commonly in the first weeks of life in healthy breastfed or formula-fed infants. Distal colon mucosal inflammation is caused by a non-IgE immune reaction to food allergens, more commonly to cow's milk protein. Rectal bleeding possibly associated with mucus and loose stools is the clinical hallmark of FPIAP. To date, no specific biomarker is available, and investigations are reserved for severe cases. Disappearance of blood in the stool may occur within days or weeks from starting the maternal or infant elimination diet, and tolerance to the food allergen is typically acquired before one year of life in most patients. In some infants, no relapse of bleeding occurs when the presumed offending food is reassumed after a few weeks of the elimination diet. Many guidelines and expert consensus on cow's milk allergy have recently been published. However, the role of diet is still debated, and recommendations on the appropriateness and duration of allergen elimination in FPIAP are heterogeneous. This review summarizes and compares the different proposed nutritional management of infants suffering from FPIAP, highlighting the pros and cons according to the most recent literature data. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Pediatric Disease / Türkiye Çocuk Hastalıkları Dergisi is the property of Turkish Journal of Pediatric Disease and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Gaucher disease (GD) is mainly caused by glucocerebrosidase (GCase) enzyme deficiency due to genetic variations in the GBA1 gene leading to the toxic accumulation of sphingolipids in various organs, which causes symptoms such as anemia, thrombocytopenia, hepatosplenomegaly, and neurological manifestations. GD is clinically classified into the non-neuronopathic type 1, and the acute and chronic neuronopathic forms, types 2 and 3, respectively. In addition to the current approved GD medications, the repurposing of Ambroxol (ABX) has emerged as a prospective enzyme enhancement therapy option showing its potential to enhance mutated GCase activity and reduce glucosylceramide accumulation in GD-affected tissues of different GBA1 genotypes. The variability in response to ABX varies across different variants, highlighting the diversity in patients' therapeutic outcomes. Its oral availability and safety profile make it an attractive option, particularly for patients with neurological manifestations. Clinical trials are essential to explore further ABX's potential as a therapeutic medication for GD to encourage pharmaceutical companies' investment in its development. This review highlights the potential of ABX as a pharmacological chaperone therapy for GD and stresses the importance of addressing response variability in clinical studies to improve the management of this rare and complex disorder. [ABSTRACT FROM AUTHOR]

Copyright of Paediatrics & Family Medicine / Pediatria i Medycyna Rodzinna is the property of Medical Communications Sp. z o.o. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: Accumulating evidence suggests that food‐induced anaphylaxis (FIA) may induce different psychological disorders (PDs). In this study, we aimed to further evaluate the effect of FIA, specifically when occurring in early life, on subsequent PDs development. Methods: We conducted a population‐based, retrospective, matched‐cohort study of pediatric patients (age ≤ 18 years) treated at the "Clalit" healthcare organization during the period 2001–2021. Children diagnosed with FIA were propensity score‐matched with patients without any allergies (controls) of similar demographic parameters. Associations between FIA and different PDs were examined by multivariable regression models. Results: The cohorts comprised 545 FIA patients and 4514 controls. Most patients were <3 years old [87.6% of controls (N = 3955) and 87.3% of the FIA cohort (N = 476)]. In this age group, the major food allergens were cow's milk (N = 258; 54.2%), eggs (N = 60; 12.6%), and peanuts (N = 20; 4.2%). The multivariable regression model identified an association between FIA and any PDs (p <.001), sleeping disorders (p <.001), and eating disorders (p =.050). Kaplan–Meier curves revealed that patients who experienced FIA before 3 years of age had an increased cumulative risk over the follow‐up time of developing any PDs, sleeping disorders, and eating disorders. Conclusion: FIA during the first 3 years of life increases the risk of later developing eating and sleeping disorders, which can last into adulthood. Further attention should be focused on accurately diagnosing these children. [ABSTRACT FROM AUTHOR]

Objective: Allergic diseases, infections, parasitic infections, hematologic malignancies, chronic lung diseases, inflammatory diseases, cystic fibrosis, nephrotic syndrome and primary immunodeficiencies (PIDs) are the main disorders in the differential diagnosis of elevated serum IgE levels. The aim of this study was to evaluate the differential clinical diagnosis of patients whose serum IgE levels were found to be above 2000 IU/ml when measured at the pediatric allergy and immunology outpatient clinics of our hospital. Materials and Methods: This was a retrospective study of children aged between 2 months to 18 years referred to the Pediatric Allergy and Immunology clinics and evaluated at our hospital between October 2010 and March 2021. Results: In the 480 patients with serum total IgE levels exceeding 2000 IU/ml in this study, allergic diseases were observed in 313 (65.2%) patients, primary immunodeficiencies in 28 (5.8%), infectious diseases in 12 (2.4%), hematological diseases in 14 (2.8%), rheumatologic diseases in 5 (1%), and other diseases in 2 (0.4%), while 113 (23.5%) patients were undefined. Conclusion: This study revealed that allergic diseases were the most common cause of extremely elevated serum IgE levels, while 5.8% of the patients were diagnosed with primary immunodeficiencies. Our study indicates that primary immunodeficiencies, although rare, should be kept in mind in patients with a serum total IgE above 2000 IU/ml. [ABSTRACT FROM AUTHOR]

Objectives: Human recombinant enzyme replacement therapy, given to compensate for genetic enzyme deficiency in lysosomal storage diseases, delays the progression of the disease and improves the quality of life. However, enzyme replacement therapy may cause hypersensitivity reactions. Within the scope of this research, we aimed to elucidate the frequency and clinical features of hypersensitivity reactions against enzyme replacement therapy in children with lysosomal storage diseases and clarify the management of these reactions., Methods: Medical records of pediatric patients with lysosomal storage disease and receiving enzyme replacement therapy were retrospectively reviewed, and patients who experienced allergic reactions were included in the study. The demographic characteristics of the patients, their diagnosis, the responsible enzyme, the time at which the reaction started and at what dose, the signs and symptoms associated with the reaction, diagnostic tests, the management of the reaction, and the protocol applied for the maintenance of enzyme replacement therapy after the reaction were recorded., Results: Hypersensitivity reactions developed in 18 of 71 patients (25.3 %) who received enzyme replacement therapy. The most common cutaneous findings were observed. Anaphylaxis developed in 6 of 18 patients. Patients who experienced recurrent hypersensitivity reactions with premedication or a slower infusion rate, those with positive skin test results, and patients who developed anaphylaxis were given enzyme replacement therapy with desensitization., Conclusions: HSR may develop during enzyme replacement therapy, which are vital in lysosomal storage diseases, and discontinuation of enzyme replacement therapy is a significant loss for patients with metabolic disorders. These reactions can be treated with premedication and long-term infusions, but some patients may require desensitization protocols for continued treatment., (© 2024 Walter de Gruyter GmbH, Berlin/Boston.)

Background: Anaphylaxis is a severe systemic hypersensitivity reaction that usually has a rapid onset and can be fatal. Presentations of childhood anaphylaxis vary widely in accordance with the triggers and the patient's age, geographical region and dietary and lifestyle habits., Methods: The medical records of 177 paediatric patients diagnosed with anaphylaxis between January 2021 and January 2024, whose disease progression was monitored at a single tertiary care centre, were reviewed retrospectively., Results: The study included 177 patients diagnosed with anaphylaxis (107 males and 70 females with a median age of 48 months). The most common allergen responsible was food (53.7%). Egg allergy was the most common source of anaphylaxis, afflicting 35 patients (19.3%), while beta-lactam provoked the most common drug allergy, affecting 24 patients (13.6%). The most common organ involved was the skin (92.7%). When the patients were analysed by age group, there were more males in the infancy, preschool and school age groups, while there were more females in the adolescent group (p = 0.44). Food-induced anaphylaxis became less common with increasing age, whereas the rate of drug-induced anaphylaxis increased (p = 0.01 and p = 0.01, respectively). Cardiovascular system findings were observed more frequently in adolescents compared to other age groups (p = 0.003). Most cases stemming from a food allergy were mild, whereas most drug-induced cases were moderate or severe (p < 0.05). When severity was analysed by age group, mild cases in infants were more common than moderate to severe cases., Conclusion: The aetiological and clinical manifestations of childhood anaphylaxis vary among different age groups., Competing Interests: The authors declare no conflict of interest.

Copyright of Ümraniye Pediatri Dergisi is the property of KARE Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective: Risk of severe systemic reactions(SR) with subsequent hymenoptera stings for patients who had a large local reaction (LLR) or only cutaneous symptoms is considered to be negligible but recent studies report cases who have experienced more severe reactions. We aimed to evaluate the frequency of reactions that are more severe than previous ones after re-sting for children with hymenoptera venom hypersensitivity reactions. Materials and Methods: Children who had attended four pediatric allergy clinics for reactions due to hymenoptera sting were questioned for previous and subsequent stings by phone call. Number of stings, characteristics of reactions, type of bee were recorded. Whether they had a reaction more severe than the first reaction with subsequent stings was evaluated. Results: By phone call, 148 children who had LLR or SR were contacted. Of these 55 had a subsequent sting after LLR or SR and 49 had had reactions more than once. In 29 patients (59.1%), there was a more severe subsequent reaction. Twenty (52.6%) of 38 children with LLR had Grade II-III SR with subsequent stings. Age, gender, type of hymenoptera, living place, presence of concomitant allergic reaction in children and/or parents of patients who had or did not have a more severe reaction with subsequent stings were not different between two groups. Median number of hymenoptera stings and sequence of the subsequent sting causing the most severe reaction were higher among patients who had a more severe reaction (p<0.001, p<0.001 respectively). Conclusion: Our results show that we may be underestimating the risk of more severe reactions with subsequent stings for children. [ABSTRACT FROM AUTHOR]