Although an increase in the incidence of childhood anaphylaxis has been reported, it remains underdiagnosed. Foods are the most common triggers for anaphylaxis, particularly cow's milk, hen's egg, ...and nuts. Other common causes of anaphylaxis in children and adolescents include venom and drugs. The skin is the most commonly affected organ, but approximately 10% of patients with anaphylaxis may present without skin symptoms, which can lead to misdiagnosis. Recognition of anaphylaxis is a great challenge in children, possibly due to a lack of vigilance among patients, caregivers, and healthcare professionals, but also in part due to discrepancies in the clinical definition of anaphylaxis. In addition, anaphylaxis in infants often poses a distinct challenge because the wide spectrum of clinical manifestations and the inability of infants to describe their symptoms may hinder prompt diagnosis and treatment. Given the rapid onset of anaphylaxis and its unpredictable severity, rapid assessment and appropriate treatment are critical. Although the morbidity and mortality associated with anaphylaxis are potentially preventable with the timely administration of life-saving epinephrine, anaphylaxis is still undertreated worldwide. Long-term management of pediatric anaphylaxis is a patientcentered, multidimensional approach that focuses on the recognition of anaphylaxis, the use of epinephrine auto- injectors, and prevention of recurrences. Therefore, close communication and collaboration between the child, caregivers, healthcare professionals, and schools are the cornerstone of long-term care. This paper is designed to provide a comprehensive overview of current perspectives and concepts related to anaphylaxis in the pediatric population in light of recent guidelines and literature.
Hypersensitivity to nonsteroidal anti-inflammatory drugs (NSAIDs) are frequently encountered in daily clinical practice. The aim of this study was to determine the confirmation rates, risk factors of ...NSAID hypersensitivity in children and to try to classify them with a standardized diagnostic protocol.
All patients with a suspicion of NSAID-induced hypersensitivity were evaluated with European Network for drug Allergy (ENDA) recommendations. The children were classified as selective responders (SRs) or cross-intolerant (CI) depending on the drug provocation test (DPT) results.
We evaluated 106 children with a suspicion of NSAID hypersensitivity. NSAID hypersensitivity was confirmed with tests in 31 patients; 4 (12.9%) were diagnosed by skin tests and 27 (87.1%) by DPTs and two patients with a history of anaphylaxis by medical records. Eleven patients (33.3%) were classified as SRs, whereas twenty-two (66.6%) children as CIs. SRs and CIs were further classified as NSAID-induced urticaria/angioedema (n = 8), NSAID-exacerbated cutaneous disease (n = 6) and NSAID-exacerbated respiratory disease (n = 1) and single NSAID-induced urticaria/angioedema and/or anaphylaxis (n = 11). Eight (24.2%) patients could not be categorized according to ENDA/GA2LEN classification; one CI patient could not be classified based on pathomechanisms, seven CIs could not be categorized based on the underlying disease and clinical manifestations. A reaction within an hour of drug intake (aOR:3.0, 95% confidence interval: 1.18–7.67, p = 0.021), a history with multiple NSAIDs hypersensitivity (aOR:2.9, 95% confidence interval: 1.16–7.60, p = 0.022), and family history of atopy (aOR:4.0, 95% confidence interval: 1.50–10.82, p = 0.006) were found as the independent risk factors related to confirmed NSAID hypersensitivity.
This study suggests the presence of different phenotypes which do not fit into the current classifications in children with NSAID hypersensitivity.
Transcobalamin II deficiency is a rare autosomal recessive disease characterized by decreased cobalamin availability, which in turn causes accumulation of homocysteine and methylmalonic acid. The ...presenting clinical features are failure to thrive, diarrhea, megaloblastic anemia, pancytopenia, neurologic abnormalities, and also recurrent infections due to immune abnormalities in early infancy.
Here, we report the clinical and laboratory features of six children with transcobalamin II deficiency who were all molecularly confirmed.
The patients were admitted between 1 and 7 months of age with anemia or pancytopenia. Unexpectedly, one patient had a serum vitamin B12 level lower than the normal range and another one had nonsignificantly elevated serum homocysteine levels. Four patients had lymphopenia, four had neutropenia and three also had hypogammaglobulinemia. Suggesting the consideration of transcobalamin II deficiency in the differential diagnosis of immune deficiency. Hemophagocytic lymphohistiocytosis was also detected in one patient. Furthermore, two patients had vacuolization in the myeloid lineage in bone marrow aspiration, which may be an additional finding of transcobalamin II deficiency. The hematological abnormalities in all patients resolved after parenteral cobalamin treatment. In follow-up, two patients showed neurological impairments such as impaired speech and walking. Among our six patients who were all molecularly confirmed, two had the mutation that was reported in transcobalamin II-deficient patients of Turkish ancestry. Also, a novel
gene mutation was detected in one of the remaining patients.
Transcobalamin II deficiency should be considered in the differential diagnosis of infants with immunological abnormalities as well as cytopenia and neurological dysfunction. Early recognition of this rare condition and initiation of adequate treatment is critical for control of the disease and better prognosis.
: Data on characteristics of asthma in children with sickle cell disease (SCD) is conflicting. Recently, the L-arginine pathway has gained attention in the pathogenesis of asthma and SCD. This study ...aimed to determine the distinctive clinical and laboratory features and the role of arginine metabolism in asthmatic children with SCD.
: A total of 52 children and adolescents with SCD, including 24 with asthma (SCD-A) and 28 without asthma (SCD-NA), and 40 healthy controls were included. A questionnaire, atopy tests, fractional exhaled nitric oxide (FeNO), and lung function tests were employed. Serum metabolites of the arginine pathway were measured. The results of the three groups were compared.
: The demographic characteristics and atopy markers of the three groups were similar. FEV1%, FEV1/FVC, MMEF%, and total lung capacity (TLC%) values of SCD-A patients were not significantly different from the SCD-NA group, but they were significantly lower than the values measured in the controls. FeNO values greater than 35 ppb were present only in the SCD-A group. In impulse oscillometry, median resistance values at 5 Hz (R5)% were higher in both SCD subgroups than in healthy controls (
= 0.001). The (R5-20/R5)% values were higher in the SCD-A group (
= 0.028). Serum arginine levels and arginine bioavailability indices were significantly lower in the SCD-A group than in the SCD-NA group and healthy controls (
= 0.003 and
< 0.001).
: Asthma in children with SCD was not associated with atopy or low FEV1/FVC levels. However, lower arginine bioavailability and higher FeNO levels differentiated asthma in patients with SCD. High R5% and (R5-20/R5)% values indicated increased airway resistance in SCD, with a predominance of small airway disease in asthmatics.
Background
Parent or self-reported drug allergy claims frequently overestimate the real incidence of hypersensitivity reactions. A detailed and algorithmic diagnostic evaluation of drug reactions may ...allow a proper diagnosis.
Objective
The aim of this study was to determine the confirmation rates and risk factors for confirmed allergic drug reactions in children.
Setting
Mersin University Hospital in Turkey.
Method
The study consisted of children between ages of 8 months and 18 years with the history of suspected drug allergy as reported by the clinician or the patients. Parents were interviewed by a clinician to complete questionnaires that included questions about demographic data and characteristics of index drug reaction. Immediate reactions (IRs) were assessed with immediate-reading skin prick (SPT) and intradermal tests (IDT). Nonimmediate reactions (NIRs) were assessed with SPT, both early and delayed reading of IDT and patch tests. In case of negative skin tests, drug provocation tests were performed. The possible risk factors for confirmed drug allergy in univariate analysis (
p
< 0.1) were entered into the multivariate logistic regression analysis to determine independent predictors.
Main outcome measure
(1) Confirmation rates of drug allergy (2) Risk factors related to confirmed drug allergy in children.
Results
We evaluated a total of 180 suspected drug allergy reactions in 97 children, mainly to antibiotics, non-steroidal anti-inflammatory drugs (NSAIDs) and anticonvulsants. Among all suspected allergic drug reactions, 97 (53.9 %) were immediate type and 83 (46.1 %) were non-immediate type. The average time interval between the reaction and allergologic work-up was 5 months. Drug allergy confirmation rates were 30.1 % for beta-lactams, 27.2 % for non-betalactams, 21.1 % for NSAIDs and 30 % for anticonvulsants. Eight of 54 confirmed NIRs showed positivity on immediate skin tests. Regulatory T cells, TGF-β and IL-10 levels were not different between groups with and without confirmed drug allergy. A strong family and personal history of drug allergy were found to be significantly related to the confirmed allergic drug reactions.
Conclusion
Parent or self-reported drug allergy should be evaluated with a standardized diagnostic work-up before strict prohibitions are made. In addition, family and personal histories of drug allergy were significant risk factors related to allergic drug reactions in children.
Drug allergies pose a great deal of danger for the patients. It hinders effective treatment procedures in hospitalized patients. Moreover, it complicates the symptoms due to the allergic reactions of ...the immune system. Allergic reactions may arise against any medication including antibiotics and chemotherapeutics. Therefore, it is crucial to assess the sensitization pattern of the patients to culprit drug(s) before retreatment with the same or similar drug, or in order to confirm/exclude a suspected drug hypersensitivity reaction. In vivo and in vitro tests are performed in the evaluation of patients. Current methods of in vitro drug allergy evaluations rely on time consuming and expensive methods. Ficoll separation of peripheral blood mononuclear cells, their activation with stimulants in the presence of the drug of interest, CD69 or CD25 or BrdU or radioactive thymidine analysis of the cells after a couple of days of incubation is an excessively elaborate work and also uneconomical. Moreover, it requires a great deal of expertise to interpret the results. Here, we are reporting a new whole blood based lymphocyte transformation test method that does not require ficoll separation, CD69, CD25, BrdU and radioactive thymidine analysis. Thanks to the color change in the whole blood itself one can easily determine the allergic reaction to a certain drug. This new method is less time consuming, more economical and easy to apply.
•Current drug allergy tests are time consuming and expensive.•A new whole blood based test method is reported.•This method does not require ficoll separation.•It also does not require CD69, CD25, BrdU and radioactive analysis.•This new method is less time consuming, more economical and easy to apply.
Background
: Although it is the most common lower respiratory infection of infancy, the optimal treatment for acute bronchiolitis is still controversial. The aim of this study was to compare the ...early and late effects of nebulized l‐epinephrine (EPI) and intramuscular dexamethasone (DEX) combination therapy with nebulized salbutamol (SAL) and dexamethasone combination and bronchodilators alone in outpatients with acute bronchiolitis.
Methods
: A total of 69 infants aged 2−21 months who were admitted to the Pediatrics Department of the Faculty of Medicine, Mersin University, with acute bronchiolitis were included in a randomized, placebo‐controlled, prospective trial study. Patients were assigned to receive either nebulized l‐epinephrine (3 mg) or salbutamol (0.15 mg/kg) and 15 min later, either dexamethasone 0.6 mg/kg or placebo (PLA), intramuscularly, in a double‐blind randomized fashion. The study groups were: epinephrine + dexamethasone group (group 1, n = 23), salbutamol + dexamethasone group (group 2, n = 23), epinephrine + placebo group (group 3, n = 11), and salbutamol + placebo group (group 4, n = 12). The outcome measures were heart rate, respiratory rate and Respiratory Distress Assessment Instrument (RDAI) score determined at 30, 60, 90 and 120 min, 24 h, and 5 days after the first therapy. Patients were then followed‐up during the subsequent 2 months for the prevalance of respiratory complaints regarding bronchial hyperreactivity.
Results
: There were no significant differences between the outcome variables of the four groups within the first 120 min and at 24 hours, or between the rates of requirement of a second dose of the same bronchodilator. However, fifth day RDAI score values of both DEX groups were significantly lower than that of SAL + PLA group (P = 0.000 and P = 0.01, respectively). The fifth day score value of group 1 was also significantly better than that value of EPI + PLA group but not different from group 2.
Conclusions
: A single dose of intramuscular dexamethasone added to nebulized l‐epinephrine, or salbutamol therapies resulted in better outcome measures than bronchodilators alone in the late phase (fifth day) of mild to moderate degree bronchiolitis attack. However, effects of EPI + DEX combination was not different from SAL + DEX combination.
BACKGROUNDArylpropionic 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.AIMTo assess the clinical characteristics and potential risk factors for proven AP hypersensitivity in children.METHODPatients 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.RESULTSA 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.CONCLUSIONAlmost 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.