Is there any difference regarding atopy between children with familial Mediterranean fever and healthy controls?

Introduction: There are only a few studies regarding the prevalence of atopy in Familial Mediterranean fever (FMF) patients, and their results are conflicting. Methods: In this study children with the diagnosis of FMF were evaluated for the presence of atopy by comparing with controls. One hundred and eighteen children diagnosed as FMF and 50 healthy age and sex matched controls were enrolled. They were evaluated for the presence of rhinitis, atopic dermatitis, urticaria and asthma. Laboratory assessment was done by measuring IgA, IgM, IgG, IgE levels, total eosinophil count and by performing skin prick test (SPT) panels for common allergens to children with FMF and healthy controls. Results: One hundred and eighteen children (61girls and 57 boys) diagnosed as FMF with a median age of 120 ± 47 months (range 36-204 months) were compared with 50 healthy controls (31 girls and 19 boys) having a median age of 126±37 (range 48-192 months). The mean percentage of total eosinophil count of patients was similar to that of the control group. The mean level of IgE was significantly higher in children with FMF than controls (136 ± 268, 87 ± 201, respectively; p values < 0.05). The percentage of skin prick test positivity was similar for both patients and controls (13% and 8.2%, respectively; p > 0.05). The prevalences of atopic dermatitis, allergic rhinitis, and asthma in the patient group were 5.08%, 28.8%, and 15.25%, respectively, while the control group had the prevalences of 0%, 36%, and 14% respectively. Conclusion: Children with FMF did not show an increase of atopic dermatitis, allergic rhinitis and asthma with respect to controls (AU)

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Is there any difference regarding atopy between children with familial Mediterranean fever and healthy controls?

INTRODUCTION: There are only a few studies regarding the prevalence of atopy in Familial Mediterranean fever (FMF) patients, and their results are conflicting. METHODS: In this study children with the diagnosis of FMF were evaluated for the presence of atopy by comparing with controls. One hundred and eighteen children diagnosed as FMF and 50 healthy age and sex matched controls were enrolled. They were evaluated for the presence of rhinitis, atopic dermatitis, urticaria and asthma. Laboratory assessment was done by measuring IgA, IgM, IgG, IgE levels, total eosinophil count and by performing skin prick test (SPT) panels for common allergens to children with FMF and healthy controls. RESULTS: One hundred and eighteen children (61girls and 57 boys) diagnosed as FMF with a median age of 120±47 months (range 36-204 months) were compared with 50 healthy controls (31 girls and 19 boys) having a median age of 126±37 (range 48-192 months). The mean percentage of total eosinophil count of patients was similar to that of the control group. The mean level of IgE was significantly higher in children with FMF than controls (136±268, 87±201, respectively; p values <0.05). The percentage of skin prick test positivity was similar for both patients and controls (13% and 8.2%, respectively; p>0.05). The prevalences of atopic dermatitis, allergic rhinitis, and asthma in the patient group were 5.08%, 28.8%, and 15.25%, respectively, while the control group had the prevalences of 0%, 36%, and 14% respectively. CONCLUSION: Children with FMF did not show an increase of atopic dermatitis, allergic rhinitis and asthma with respect to controls.

Immediate adverse reactions to intravenous immunoglobulin in children: a single center experience.

Intravenous immunoglobulin (IVIG) is commonly used in primary and secondary immunodeficiency diseases as well as autoimmune conditions as immunomodulatator treatment. Immediate adverse events which are generally mild and occur during infusion are seen in 6 hours. Reported immediate adverse events are in a wide range from 1%-40% in pediatric patients. 115 patients who received IVIG (except newborns) were included into this crosssectional study. IVIG was given to patients for primary immunodeficiencies (n=8), ITP (n=65), Kawasaki disease (n=11), secondary immunosupression (n=28), and passive immunization (n=3). 5%, 10% IVIG preparations and pentaglobin were used. Headache, fever, chills, nausea, rash, arthralgia, myalgia and back pain were accepted as mild immediate events. There were 62 (54%) boys and 53 (46%) girls aged 1 month-18 years. Mean age of the group was 7.4±4.6 years. Immediate adverse events due to IVIG infusions were seen in 29 (25.2%) of all patients. Gender and types of the disease were not different in significance regarding the presence of adverse events. The rate of adverse events did not change with receiving pre-medication. The most common reaction was fever/chills. Immediate reactions were seen in first 6 hours in 7 patients and during infusion in the remaining. They were treated with slowing of the infusion rate and infusion was stopped in 3 patients because of moderate events. Because of the increasingly use of IVIG therapy, it is important to know the side effects. High doses, high infusion rates, accompanying infection may worsen the adverse effects especially in primary immunodeficiency diseases.

Are skin prick tests really safe? A case of anaphylaxis caused by skin prick testing with inhalant allergens

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B-cell subsets in patients with transient hypogammaglobulinemia of infancy, partial IgA deficiency, and selective IgM deficiency.

BACKGROUND: The pathogenesis of some primary humoral immunodeficiencies, such as transient hypogammaglobulinemia of infancy (THI) and immunoglobulin (Ig) A deficiency, remains unknown and can render diagnosis problematic. OBJECTIVE: In the present study, we used flow cytometry to analyze peripheral blood B-cell subsets in patients with THI and unclassified hypogammaglobulinemia (UCH), partial IgA deficiency, and selective IgM deficiency. METHODS: The study population comprised 41 patients with hypogammaglobulinemia (THI, 18; UCH, 23), 16 patients with partial IgA deficiency, and 16 patients with selective IgM deficiency who were admitted to Ankara University Department of Pediatric Immunology-Allergy between January 2010 and April 2011, as well as 29 healthy controls. B-cell subsets were examined according to the EUROclass classification. RESULTS: Age at diagnosis in the hypogammaglobulinemia group ranged between-14 months and 13 years (median, 26 months). Naive B-cell percentages were significantly higher and activated B-cell values lower in the THI patients than in the UCH patients and age-matched healthy controls. Nonswitched (IgM+CD27+IgD+) memory B-cell values were found to be significantly lower in patients with selective IgM deficiency than in healthy controls. No significant differences in B-cell subsets were found in patients with partial IgA deficiency. CONCLUSIONS: Previous reports show that reduced class-switched memory B cell values are associated with CVID, THI, and selective IgA deficiency. Our findings did not support these reports. Furthermore, we observed that naive B cell values were higher in patients with THI. A maturation defect could play a role in the pathogenesis of THI.

B-Cell Subsets in Patients With Transient Hypogammaglobulinemia of Infancy, Partial IgA Deficiency, and Selective IgM Deficiency

Antecedentes: La patogénesis de algunas inmunodeficiencias primarias tales como la hipogammaglobulinemia transitoria de la infancia (THI), el déficit de IgA permanece desconocida y a veces es motivo de un problema en su diagnóstico. Objetivos: El motivo de este estudio fue analizar mediante citometría de flujo, las subclases de células B en sangre periférica en pacientes con THI junto con hipogammaglobulinemias no clasificadas (UCH), deficiencias parciales de IgA y deficiencias selectivas de IgM. Métodos: Se incluyeron 41 pacientes con hipogammaglobulinemia (THI: 18 y UCH: 23 pacientes), 16 con déficit parcial de IgA, 16 con deficiencia selectiva de IgM y 29 controles sanos admitidos en Ankara University, Departamento de Pediatría e Inmunología -Alergia. Se examinaron las subclases de células B de acuerdo con la clasificación Euroclass. Los pacientes fueron vistos entre enero de 2010 y abril de 2011. Resultados: En el grupo de hipogammaglobulinemia, la edad en el diagnóstico se encontraba en un rango entre 14 meses y 13 años (Med: 26 meses). Las células B naive se encontraban significativamente elevadas y las células B activadas disminuidas en el grupo THI respecto al grupo UCH y los controles sanos. Las células B memoria (IgM+ CD27+ IgD+) se encontraban significativamente disminuidas en pacientes con diagnóstico de déficit selectivo de IgM. En la deficiencia parcial de IgA no se encontraron diferencias en las subclases de células B. Conclusiones: Nuestros resultados no confirman resultados previos de una reducción de células B memoria relacionada con CVID, THI y selectiva deficiencia de IgA. Encontramos un aumento de células B naive en pacientes con hipogammaglobulinemia transitoria, sugiriendo un defecto de maduración que puede jugar un papel en la patogénesis de esta enfermedad (AU)

Background: The pathogenesis of some primary humoral immunodeficiencies, such as transient hypogammaglobulinemia of infancy (THI) and immunoglobulin (Ig) A deficiency, remains unknown and can render diagnosis problematic. Objective: In the present study, we used flow cytometry to analyze peripheral blood B-cell subsets in patients with THI and unclassified hypogammaglobulinemia (UCH), partial IgA deficiency, and selective IgM deficiency. Methods: The study population comprised 41 patients with hypogammaglobulinemia (THI, 18; UCH, 23), 16 patients with partial IgA deficiency, and 16 patients with selective IgM deficiency who were admitted to Ankara University Department of Pediatric Immunology- Allergy between January 2010 and April 2011, as well as 29 healthy controls. B-cell subsets were examined according to the EUROclass classification. Results: Age at diagnosis in the hypogammaglobulinemia group ranged between 14 months and 13 years (median, 26 months). Naïve B-cell percentages were significantly higher and activated B-cell values lower in the THI patients than in the UCH patients and age-matched healthy controls. Nonswitched (IgM+CD27+IgD+) memory B-cell values were found to be significantly lower in patients with selective IgM deficiency than in healthy controls. No significant differences in B-cell subsets were found in patients with partial IgA deficiency. Conclusions: Previous reports show that reduced class-switched memory B cell values are associated with CVID, THI, and selective IgA deficiency. Our findings did not support these reports. Furthermore, we observed that naive B cell values were higher in patients with THI. A maturation defect could play a role in the pathogenesis of THI (AU)