RESUMEN
BACKGROUND: Common variable immune deficiency (CVID) is a heterogeneous syndrome with a wide variety of signs and symptoms. This study describes the phenotyping and survival of the CVID patients in the allergy and clinical immunology department of Rasol-E-Akram Hospital of Iran University of Medical Sciences in Tehran. METHOD: We retrospectively reviewed hospital files of CVID patients in our department until January 2014. All patients were diagnosed with standard diagnostic criteria of CVID, treated and visited monthly, during the follow-up period. We divided the patients into four phenotypes; infection only, cytopenia, polyclonal lymphocytic infiltration and unexplained enteropathy. The immunologic, demographic and clinical findings in different phenotypes were analysed. RESULTS: The study included 47 CVID patients with mean age at onset of symptoms and diagnosis of 11.2 and 20.2 years, respectively. Phenotyping of our patients was: only infection (62%), cytopenia (26%) and PLI (19%) and 94% of cases had only one phenotype. We did not find a significant relation between the clinical phenotypes and immunologic or demographic data. Rate of parental consanguinity in our cases was 47%. Parental consanguinity was related to lower age at onset, lower age at diagnosis and higher baseline IgG levels. Patients with malignancy and autoimmunity had significantly higher age at onset. Our patients were followed-up for 6.9 years and the mortality rate during this time was 6%. CONCLUSIONS: Parental consanguinity and age at onset of CVID symptoms may have important roles in CVID manifestations.
Asunto(s)
Inmunodeficiencia Variable Común/epidemiología , Inmunodeficiencia Variable Común/genética , Consanguinidad , Adolescente , Adulto , Edad de Inicio , Autoinmunidad , Niño , Preescolar , Inmunodeficiencia Variable Común/mortalidad , Femenino , Estudios de Seguimiento , Humanos , Irán/epidemiología , Masculino , Persona de Mediana Edad , Fenotipo , Estudios Retrospectivos , Adulto JovenRESUMEN
BACKGROUND: Vitamin D is hypothesized to have some roles in innate and adaptive immunity, inflammation reduction, and remodeling; therefore, it is supposed to affect the asthma phenotype, severity, and response to inhaled corticosteroid (ICS). OBJECTIVE: To explore the synergistic effects of vitamin D supplementation in addition to asthma controllers (ICS or ICS plus long-acting ß-agonist) on airway functions. METHODS: A randomized clinical trial was conducted in 130 individuals aged 10 to 50 years who lived in Tehran during a 24-week period. Data on age, sex, body mass index, stage of asthma, serum total IgE, history of allergic rhinitis, atopic dermatitis, food allergy, and urticaria were collected. Spirometric parameters (forced expiratory volume in 1 second [FEV1] and ratio of FEV1 to forced vital capacity) and serum vitamin D measurement were obtained before and 8 and 24 weeks after the intervention. Patients were divided in 2 groups randomly. Both groups received asthma controllers (budesonide or budesonide plus formoterol) according to their stage, but the intervention group received vitamin D supplementation (100,000-U bolus intramuscularly plus 50,000 U orally weekly) in addition to asthma controllers. RESULTS: FEV1 improved significantly in both groups after 8 weeks, but no significant difference was found between the 2 groups at baseline (P = .20) or after 8 weeks (P = .99); however, a significant improvement was seen in the intervention group in the last 16 weeks, and FEV1 was significantly better in the intervention group than the other group after 24 weeks (P < .001). CONCLUSION: Vitamin D supplementation associated with asthma controllers could significantly improve FEV1 in mild to moderate persistent asthma after 24 weeks. TRIAL REGISTRATION: irct.ir Identifier: IRCT201302079608N1.
Asunto(s)
Asma/tratamiento farmacológico , Budesonida/uso terapéutico , Suplementos Dietéticos , Etanolaminas/uso terapéutico , Vitamina D/uso terapéutico , Adolescente , Adulto , Antiinflamatorios/uso terapéutico , Broncodilatadores/uso terapéutico , Niño , Dermatitis Atópica/complicaciones , Sinergismo Farmacológico , Femenino , Hipersensibilidad a los Alimentos/complicaciones , Volumen Espiratorio Forzado , Fumarato de Formoterol , Humanos , Inmunoglobulina E/sangre , Irán , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Rinitis Alérgica/complicaciones , Urticaria/complicaciones , Capacidad Vital , Vitamina D/sangre , Adulto JovenRESUMEN
AIM: New approaches such as oral immunotherapy (OIT) may be useful in IgE-mediated anaphylaxis to wheat. PATIENTS & METHODS: 12 patients underwent OIT protocol that comprised of two phases: the first with semolina flour and the second with spaghetti. Total and specific wheat IgE were assayed by ELISA before and after OIT and 18 months later. Skin prick tests were also performed. RESULTS: Patients successfully tolerated 50 g of wheat. The median baseline total IgE was decreased after up-dosing phase and decreased after follow-up (p < 0.01). The median baseline wheat-specific IgE was increased after up-dosing and decreased after follow-up (p < 0.001). CONCLUSION: The efficiency and safety of our OIT protocol were shown on wheat allergic patients but further investigation is needed.
Asunto(s)
Anafilaxia/terapia , Desensibilización Inmunológica , Hipersensibilidad a los Alimentos/terapia , Triticum/efectos adversos , Anafilaxia/inmunología , Niño , Preescolar , Femenino , Hipersensibilidad a los Alimentos/inmunología , Humanos , Inmunoglobulina E/inmunología , MasculinoRESUMEN
Cow's milk allergy (CMA) is the most frequent food allergy in children and oral immunotherapy (OIT) is a promising approach for treatment of patients. The most challenging cases are anaphylactic with coexisting asthma and proposing safe protocols is crucial especially in high risk groups. Considering that CMA varies among patients, an individualized OIT protocol would be beneficial to achieve a safer and more efficient method of desensitization. 18 children more than 3 years of age with IgE-mediated CMA were enrolled. CMA was confirmed by positive skin prick test (SPT) and positive oral food challenge (OFC) and 60% of individuals had a convincing history of persistent asthma. SPT with milk extracts, whole fresh milk and serially diluted milk concentrations were performed. The dilution of milk that induced 3-5 mm of wheal in each individual was selected as the starting dilution for OIT. Desensitization began by 1 drop of the defined dilution and continued increasingly. Overall, 16 out of 18 children (88.8%) achieved the daily intake of 120 mL of milk. Four out of these 16 children accomplished the protocol without any adverse allergic reactions. 12 patients experienced mild to severe reactions. Wheal and erythema in SPT (p≤0.001), and sIgE (p≤0.003) to most milk allergens were significantly decreased following desensitization. We successfully desensitized 16 of 18 children with IgE-mediated CMA by individualized desensitization protocol. Individualizing the OIT protocol would be helpful to save time and perhaps to relieve the allergic symptoms after ingesting cow's milk intake.