Allergic and autoimmune disorders in families with selective IgA deficiency.

BACKGROUND/AIM: IgA deficiency is the most common human primary immunodeficiency. The prevalence of allergic disorders and autoimmunity is thought to be increased in selective IgA deficiency (sIgAD). However, it is currently unclear if these disorders coincide within these families. We aimed to evaluate the frequency of allergic and autoimmune disorders in children with sIgAD and their first-degree relatives (FDRs). MATERIALS AND METHODS: The study included 81 children diagnosed with sIgAD and 274 of their FDRs. The presence of allergic and autoimmune disorders was evaluated and serum antithyroglobulin and antithyroid peroxidase levels were measured in both patients and their first-degree relatives. RESULTS: The mean age of the patients was 9.9 ± 3.9 years. Among the patients with sIgAD, 45.7% of them had at least one allergic disorder and 17.3% of them had at least one autoimmune disorder. The frequencies of asthma, allergic rhinitis, and eczema in the FDRs of sIgAD patients were 10.9%, 9.1%, and 7.7%, respectively. Among their FDRs, 14.6% had autoimmunity, compared to an estimate of 5% in the general population. CONCLUSION: Increased frequency of allergic and autoimmune disorders in patients with sIgAD and their FDRs suggests a possible common predisposing genetic component for sIgAD and autoimmunity in these families.

The Etiology and Clinical Features of Anaphylaxis in a developing country: A nationwide survey in Turkey.

BACKGROUND: Despite the increasing frequency of anaphylaxis, there is inadequate information on the etiology and clinical features in various countries, regions and age groups, especially in developing countries. OBJECTIVE: Our aim is to assess the etiology and clinical findings of anaphylaxis in Turkey. Gathering reliable data about the etiology and clinical findings of anaphylaxis in the general population will decrease the related morbidity and mortality. METHOD: We obtained the names and phone numbers of individuals who had been prescribed an epinephrine auto-injector with a diagnosis of anaphylaxis from ministry of health. Demographic data, clinical history of the first episode of anaphylaxis including the triggering agent, clinical findings, course of hospitalization, and the management of anaphylaxis were obtained by phone survey. RESULTS: A total of 843 patients with a mean age of 21.4±17.3 years were evaluated. There was a significant male predominance among children younger than 10 years of age but a female predominance in older subjects. The most common causes of anaphylaxis were foods(40.1%) in children and bee venom(60.8%) in adults. The biphasic reaction rate was 4.3% and the median length of stay at an emergency department was 4.0 hours. Almost 60% of the patients had recurrent anaphylaxis episodes. Only 10.7% of the cases were prescribed an epinephrine auto-injector at their first anaphylaxis episode and only 59.2% of the patients were referred to an allergist during discharge from the emergency department. CONCLUSIONS: In Turkey, bee venom was the most common cause of anaphylaxis, followed by food and drug. While more than a half of patients reported recurrent attacks; only 10% had been prescribed epinephrine auto-injector kit after their first episode. Strategies to improve the anaphyalxis management are therefore urgently required.

Bronchial hyperreactivity in children with antibody deficiencies

BACKGROUND: Antibody deficiency comprises a heterogeneous group of disorders characterised by the body's inability to mount an effective antibody response to pathogens. Although it has been reported that asthma and allergic disease are frequent in antibody deficiencies, there are no data that evaluate and compare bronchial hyperreactivity (BHR) in all groups of antibody deficiencies. In this study, we aimed to evaluate and compare the frequency of BHR in patients with different antibody deficiencies. METHODS: The study was carried out on 113 patients between ages 5 and 18 diagnosed with antibody deficiencies. The patients and their families were questioned on their history of asthma and allergic diseases. Allergic skin prick tests and non-specific bronchial provocation test with methacholine was done for all patients. Complete blood count and serum total IgE levels were measured. RESULTS: The mean age of the patients was 10.8 ± 3.8 years and 66.4% were male. Within the study group 41.6% of the patients had selective IgA deficiency, 24.8% had IgG subclass deficiency,14.2% had partial IgA deficiency, 10.6% had common variable immunodeficiency, 6.2% had transient hypogammaglobulinaemia and 2.7% X-linked agammaglobulinaemia. In total group, 42.5% had bronchial hyperreactivity with methacholine challenge test. BHR was more significant in both patients with selective IgA deficiency and partial IgA deficiency compared to those with IgG subclass deficiency (P = 0.041 and P = 0.038, respectively). CONCLUSION: BHR was high in antibody deficiencies, especially selective IgA deficiency compared to IgG subclass deficiency

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Bronchial hyperreactivity in children with antibody deficiencies.

BACKGROUND: Antibody deficiency comprises a heterogeneous group of disorders characterised by the body's inability to mount an effective antibody response to pathogens. Although it has been reported that asthma and allergic disease are frequent in antibody deficiencies, there are no data that evaluate and compare bronchial hyperreactivity (BHR) in all groups of antibody deficiencies. In this study, we aimed to evaluate and compare the frequency of BHR in patients with different antibody deficiencies. METHODS: The study was carried out on 113 patients between ages 5 and 18 diagnosed with antibody deficiencies. The patients and their families were questioned on their history of asthma and allergic diseases. Allergic skin prick tests and non-specific bronchial provocation test with methacholine was done for all patients. Complete blood count and serum total IgE levels were measured. RESULTS: The mean age of the patients was 10.8 ± 3.8 years and 66.4% were male. Within the study group 41.6% of the patients had selective IgA deficiency, 24.8% had IgG subclass deficiency, 14.2% had partial IgA deficiency, 10.6% had common variable immunodeficiency, 6.2% had transient hypogammaglobulinaemia and 2.7% X-linked agammaglobulinaemia. In total group, 42.5% had bronchial hyperreactivity with methacholine challenge test. BHR was more significant in both patients with selective IgA deficiency and partial IgA deficiency compared to those with IgG subclass deficiency (P=0.041 and P=0.038, respectively). CONCLUSION: BHR was high in antibody deficiencies, especially selective IgA deficiency compared to IgG subclass deficiency.

Risk factors of severe atopic dermatitis in childhood: single-center experience.

The aim of this study was to evaluate the risk factors of disease severity to facilitate better management of children with severe atopic dermatitis (AD). All the patients were diagnosed using Hanifin-Rajka criteria. After medical and family histories were obtained and a detailed physical examination was performed, disease severity was determined with the objective SCORing Atopic Dermatitis (SCORAD) index. Skin prick tests were performed, and percent of peripheral blood eosinophils, total serum IgE and specific IgE were measured. The median age of the 501 patients was 15 months (interquartile range [IQR]: 6-40 months), and 62.9% (315) were male. Sensitization to at least one allergen and foods was observed in 40.3% (202) and 30.9% (155) of all patients, respectively. Of the study group, 17.6% (88) had severe disease. When logistic regression analyses were performed, with adjustments, the risk factors for severe AD were determined to be eosinophilia (odds ratio [OR] 1.137, 95% confidence interval [CI] 1.062-1.217; p=0.003) and food allergen sensitization (OR: 1.937, 95%CI: 1.217-3.084; p=0.005). The patients with severe AD had sensitization to common allergens, food allergens and eosinophilia more frequently than those with mild-moderate disease (p=0.001, p=0.001 and p=0.005, respectively). Eosinophilia may predict severe disease and allergic sensitization. Further large-scale follow-up studies are needed to improve the reliability and relevance of this relation.

Allergic diseases in children with primary immunodeficiencies.

The aim of this study was to evaluate and compare the frequency of atopy and allergic disease in all groups of primary immunodeficiency (PID) patients. The study was done on 318 patients with PID between the ages of 6 months and 18 years. The patients and their parents were questioned regarding their histories of asthma and allergic disease. Within the study group, 82.4% of the patients had antibody deficiency, 10.4% combined immunodeficiency, 6.6% phagocyte number or function defect, and 0.6% complement deficiency. Patients with selective immunoglobulin (Ig)A deficiency had a more significant history of ever wheezing compared to those with IgG subclass deficiency (p=0.022). The frequency of current wheezing was higher in patients with antibody deficiency than in patients with combined immunodeficiency (p=0.049). In conclusion, patients with antibody deficiency, especially those with selective IgA deficiency, should be evaluated regarding asthma and allergic diseases if recurring respiratory symptoms are present.

The evaluation of drug provocation tests in pediatric allergy clinic: a single center experience.

Drug provocation tests (DPTs) are gold standard to diagnose drug allergy. Our goal was to evaluate the results and safety of diagnostic methods including DPTs during childhood. Between January 2010 and February 2013 DPTs were performed and evaluated, prospectively, in children who attended our pediatric allergy clinic with a suspected drug hypersensitivity reaction. One hundred ninety-eight suspected drug reactions in 175 patients (88 boys and 87 girls) were evaluated. The median age of the subjects at the time of the suspected drug-induced hypersensitivity reaction and at the time of the study was 56 (interquartile range [IQR] = 24-120 months) months and 76 (IQR = 35-149 months) months, respectively. Suspected drugs were beta-lactam antibiotics in 108 cases (54.5%), non-beta-lactam antibiotics in 22 cases (11.1%), and nonsteroid anti-inflammatory drugs in 52 cases (26.3%). The history was compatible with immediate-type reactions in 69 cases (34.8%). Skin-prick tests were not positive in any of the cases. Intradermal tests were positive in three cases (4%). DPTs were positive in 13 (6.8%) of 191 provocation cases, which were performed with culprit drugs. Our results suggest that a positive clinical history is not enough to make a diagnosis of drug allergy, which highlights the significance of undertaking further diagnostic evaluation especially for DPTs.

TRACK as a complementary tool to GINA and NAEPP guidelines for assessing asthma control in pre-school children.

BACKGROUND: In this study, our goal is to evaluate the consistency between TRACK and the asthma control levels assessed according to the GINA and NAEPP guidelines in children younger than 5 years of age. METHODS: Patients under 5 years old, who were followed up for recurrent wheezing for at least 1 year have been included. Parents were given the TRACK questionnaire and the control level of asthma according to GINA and NAEPP guidelines were determined by a pediatric allergist blinded to TRACK scores. Patients were classified into two groups regarding the compatibility of the control level between TRACK and both GINA and NAEPP guidelines. RESULTS: A total of 365 questionnaires were evaluated. The TRACK cut-off point of 80 provided the most consistent balance between sensitivity and specificity for the compatibility with both GINA and NAEPP (for GINA 0.763 and 0.663, kappa = 0.487, p < 0.001 and for NAEPP 0.761 and 0.769, kappa = 0.524, p < 0.001, respectively). When 80 was taken as the cut-off value for TRACK, the compatibility rate of asthma control levels between TRACK and GINA and TRACK and NAEPP was 71.0 and 76.4%, respectively. About 70.1% of the patients who had TRACK scores over 80 and had mild asthma were grouped as controlled according to GINA and 50.0% of patients who had TRACK scores over 80 and had moderate to severe asthma was grouped as uncontrolled according to GINA (p = 0.019). CONCLUSION: TRACK is compatible with NAEPP and GINA in majority of asthmatic children under 5 years of age. Nevertheless, there is a discrepancy between guidelines and TRACK scores; therefore, it should be used in conjunction with a detailed clinical examination in order to make a better decision for assessing the control levels and management plan.

Reactive thrombocytosis in children.

The aim of this study was to evaluate the causes of thrombocytosis, which was defined as a platelet count greater than 500 x 10(9)/L, and to compare the groups with mild and severe thrombocytosis. A total of 484 patients were evaluated for the etiology of thrombocytosis. Patients with a platelet count between 500-800 x 10(9)/L were considered to have mild thrombocytosis, while those with a count of ≥800 x 10(9)/L were considered as having severe thrombocytosis. Of 484 patients included, 63% had thrombocytosis due to an infectious disease, 11.4% had a chronic inflammatory condition, 8.5% had anemia, and 5.2% had tissue injury. The frequency of chronic inflammation was higher in the severe thrombocytosis group compared to the mild thrombocytosis group (p=0.006). In conclusion, severe infections and chronic inflammatory conditions should be considered in the differential diagnosis of a patient with severe thrombocytosis.

The effect of obesity on the level of fractional exhaled nitric oxide in children with asthma.

BACKGROUND: Several studies have demonstrated a relationship between asthma and obesity. However, the results have been conflicting with regard to the relationship between fractional exhaled nitric oxide (FeNO), used as a marker of airway inflammation in asthmatic patients, and obesity. We aimed to evaluate the association of FeNO with obesity and obesity-related metabolic complications in asthmatic and nonasthmatic children. METHODS: The study population included children aged between 6 and 17 years and consisted of 4 groups: obese asthmatics (n = 52), normal-weight asthmatics (n = 49), obese nonasthmatics (n = 51) and normal-weight nonasthmatics (n = 42). FeNO measurement and spirometry were performed for all patients. To evaluate the metabolic complications, serum lipids, glucose and insulin levels were measured. Insulin resistance (IR) was estimated by the homeostasis model assessment, HOMA-IR. All participants were evaluated for the presence of metabolic syndrome (MS). RESULTS: The mean age for the 194 subjects participating in the study was 11.6 ± 2.5 years. The FeNO level of asthma patients with MS was not different from those without MS (14.5 ± 8.0 and 16.7 ± 8.7, respectively, p = 0.449). In the nonasthmatic group, subjects with MS had a higher FeNO level than subjects without MS (12.5 ± 5.1 and 17.3 ± 8.3, respectively, p = 0.014). Spearman's rank correlation coefficients revealed a positive correlation between FeNO and body mass index (BMI; p = 0.049, r(2): 0.204) in the nonasthmatic group and after multivariate regression analysis, BMI still persisted as an independent risk factor for FeNO. CONCLUSION: We found a positive correlation between BMI and FeNO level which suggests a link between obesity and increased airway inflammation in nonasthmatic children.

Characteristics of anaphylaxis in children referred to a tertiary care center.

Anaphylaxis is a potentially life-threatening condition. There are limited data about the etiology and the clinical characteristics in developing countries. This study aimed to investigate the clinical characteristics of anaphylaxis patients attending our pediatric allergy clinic. We conducted a prospective analysis of patients who were admitted to our allergy clinic for anaphylaxis from 2010 to 2012. Ninety-six patients were evaluated during the study period. The mean age was 7.4 ± 5.2 years. Venom, food, and drugs were the most common causative agents responsible for 31 (32.3%), 30 (31.3%), and 26 (27.1%) of the cases, respectively. Foods implicated most frequently were peanuts and nuts (n = 9; 30.0%), cow's milk (n = 7; 23.3%), and egg white (n = 6; 20.0%). The clinical manifestations during anaphylaxis in order of frequency were cutaneous (97.9%), respiratory (86.5%), gastrointestinal (42.7%), neurological (37.5%), and cardiovascular symptoms (30.2%). A biphasic course was noticed in five cases (5.2%). Of the 91 patients, 79 (86.8%) received H1-antihistamines, 73 (80.2%) received corticosteroids, 40 (44.4%) received adrenaline, 38 (41.8%) received fluid replacement therapy, 18 (19.8%) received ß2-mimetics, and 8 (8.8%) received H2-antihistamines. According to severity, 7.3% of patients had mild, 59.4% had moderate, and 33.3% had severe anaphylaxis. Food and bee venom allergy were the most common etiologies. Adrenaline, the first-line treatment of anaphylaxis, was administered in only 44.4% of our cases.

Prevalence of confirmed immediate type drug hypersensitivity reactions among school children.

BACKGROUND: Despite drug-related hypersensitivity reactions are an important health problem, epidemiologic data on drug allergy and hypersensitivity are limited, and studies including diagnostic work-up are scarce. The aim of this study was to determine the actual frequency of immediate type drug hypersensitivity using diagnostic tests in school children with parent-reported drug allergies. METHODS: This study involved three phases. The first phase is a survey of children with a mean age of 12.9 yrs attending grades 6-8 of primary schools with a questionnaire asking drug-related symptoms within 2 h of ingestion. The total population of sixth to eight grade school children was 210,000, and a sample size of 9096 was deemed to be representative of Ankara [(p) = 1.0%, α < 0.05, ß = 0.8, (d) = 0.2.] During the second phase, a detailed clinical history was obtained by phone from the parents of children who had positive parent-reported drug allergy. The final stage of the study consisted of a detailed diagnostic work-up of children with a clinical history consistent with immediate type drug hypersensitivity reaction. RESULTS: Overall, 11,233 questionnaires were distributed, 10,096 of which were retrieved after completion by parents. The rate of parent-reported immediate type drug hypersensitivity was 7.87% (792 children). However, phone survey revealed a clinical history suggestive of drug allergy in only 117 children (1.16%). After further diagnostic work-up, the true frequency of immediate type drug hypersensitivity was 0.11%. CONCLUSION: Our results suggest that a positive clinical history is not enough to make a diagnosis of drug allergy, which highlights the significance of undertaking further diagnostic evaluation.

Vitamin D level in children is correlated with severity of atopic dermatitis but only in patients with allergic sensitizations.

Vitamin D is believed to affect the progression and severity of atopic dermatitis (AD). Allergic sensitization may cause this effect to vary. Individuals who fulfilled the Hanifin and Rajka criteria for AD underwent epidermal prick tests and blood tests for specific immunoglobulin E(IgE), serum total IgE, 25-hydroxy vitamin D, and peripheral blood eosinophil count and percentage. Disease severity was determined according to the Scoring Atopic Dermatitis (SCORAD) index. Patients were grouped according to allergic sensitization. Seventy-three children with AD (median age 33.0 mos, interquartile range 19.0-61.5 mos) were enrolled in the study; 33 (45.2%) were found to have allergic sensitization. In this group there was a negative correlation between SCORAD score and serum vitamin D level (p = 0.047, correlation coefficient [r] = -0.349), whereas there was no correlation in the group without sensitization (p = 0.30, r = -0.168). Vitamin D was not correlated with total IgE and eosinophil percentage in either AD group (p = 0.77, r = 0.054 and p = 0.73, r = -0.062, respectively). Vitamin D may affect the severity of AD, especially in children with allergic sensitization.

Evaluation of respiratory viral pathogens in acute asthma exacerbations during childhood.

OBJECTIVE: Common upper respiratory tract viruses are the most frequent and important causes of asthma exacerbations in both children and adults. Prospective epidemiologic studies report that up to 80% of childhood exacerbations are associated with viral upper respiratory tract infections. MATERIALS AND METHODS: The study group consisted of 104 children with asthma aged 3-17 years who received treatment for asthma exacerbations in our clinic between September 2009 and 2010. Nasopharyngeal and nasal swabs were obtained from all patients during an acute attack, and from the control group (31 subjects). These specimens were investigated for the presence of viral respiratory pathogens using a real-time multiplex PCR method. The patients were compared for the presence of respiratory pathogens and factors related to the severity of the asthma exacerbation. RESULTS: A pathogenic respiratory virus was detected in 53.8% of patients in the acute exacerbation group. The most commonly encountered viral agent was Rhinovirus (35.6%). Patients who had an acute exacerbation with or without a detectable viral pathogen were compared according to the severity of the exacerbation, the need for systemic steroids, and hospitalization rates. No statistically significant difference was found. CONCLUSION: Although viral upper respiratory tract infections are the most common cause of asthma exacerbations, the severity level of the exacerbation seems to be independent of whether a respiratory virus has been detected.