RESUMO
Ataxia-telangiectasia (AT) is a rare genetic disorder leading to neurological defects, telangiectasias, and immunodeficiency. We aimed to study the clinical and immunological features of Latin American patients with AT and analyze factors associated with mortality. Referral centers from 9 Latin American countries participated in this retrospective cohort study, and 218 patients were included. Median (IQR) ages at symptom onset and diagnosis were 1.0 (1.0-2.0) and 5.0 (3.0-8.0) years, respectively. Most patients presented recurrent airway infections, which was significantly associated with IgA deficiency. IgA deficiency was observed in 60.8% of patients and IgG deficiency in 28.6%. T- and B-lymphopenias were also present in most cases. Mean survival was 24.2 years, and Kaplan-Meier 20-year-survival rate was 52.6%, with higher mortality associated with female gender and low IgG levels. These findings suggest that immunologic status should be investigated in all patients with AT.
RESUMO
Objetivo: Investigar a associação entre o mês de nascimento e a sensibilização a ácaros da poeira doméstica e a polens de gramíneas em pacientes encaminhados a uma clínica privada de alergia do município de Passo Fundo-RS. Pacientes e Métodos: Estudo transversal com análise de 4650 prontuários no período de 1987 a 2006. Incluídos pacientes com até vinte anos de idade, diagnóstico de doença atópica (asma, rinite alérgica e/ou eczema) e monossensibilizados para ácaros da poeira doméstica ou polens de gramíneas. O grupo de comparação consistiu no total de nascidos vivos em Passo Fundo durante o mesmo período do estudo. Foram analisados idade, gênero e sensibilização alérgica, e todos os pacientes foram submetidos a testes cutâneos (puntura) para ambos os alérgenos estudados. Resultados: Neste estudo, 1303 pacientes (28 per cent) preencheram os critérios de inclusão, sendo que 42,8 per cent eram do sexo feminino, com idade variando entre dois e 20 anos. Desses, 1125 (86,3 per cent) apresentaram positividade apenas para ácaros da poeira doméstica e 178 (13,7 per cent) apenas para polens de gramíneas, sendo que a média de idade para os grupos foi, respectivamente, de 9,4 ± 5,1 anos (IC95 per cent: 9,1 a 9,7) e de 11,8 ± 5,8 anos (IC95 per cent = 11,0 a12,7). Não foi encontrada associação estatisticamente significativa entre o mês de nascimento e a sensibilização a ácaros da poeira doméstica (p = 0,55) e polens de gramíneas (p = 0,10). Conclusão: Não houve associação estatisticamente significativa entre mês de nascimento e sensibilização a aeroalérgenos nos pacientes estudados.
Purpose: To investigate the association between the month of birth and sensitization to house dust mites and grass pollen in patients evaluated in an outpatient allergy c1inic in Passo Fundo-RS. Methods: Cross-sectional study was performed with 4650 patient reports from 1987 to 2006. The patients were aged from 2 to 20 years old, had the diagnosis of atopic disease (asthma, allergic rinitis and/or eczema) and were monosensitizated to house dust mites or grass pollen. The comparison group consisted in total live births in Passo Fundo during the same period of the study. Age, gender and allergic sensitization were analyzed and prick test was performed in all the patients. Results: In this study, 1303 patients (28 per cen) full field the inclusion criteria, 42,8 per cent were female, with age varying from 2 to 20 years. Among the patients, 1125 (86,3 per cent) showed positivity only to house dust mites and 178 (13,7 per cent) only to grass pollen, and the mean of age for the both groups was, respectively, 9,4 ± 5,1 years (95 per cent CI = 9,1 - 9,7) and 11,8 ± 5,8 years (95 per cent CI = 11,0 - 12,7). Statistically significant association was not found between the month of birth and sensitization to house dust mites (p = 0,55) or grass pollen (p = 0,10). Conclusion: Month of birth did not show statistically significant association with sensitization to aeroallergens among the patients evaluated in the study. .
