542 Descriptive Analysis of the Immunological Behavior of Patients with Ataxia Telangiectasia Attended in the National Institute of Pediatrics in the Past 30 Years

BACKGROUND: Ataxia Telangiectasia is an autosomal recessive disease characterized by progressive neurological impairment, ataxia, oculo-cutaneous telangiectasia, immunodeficiency, recurrent infections sinopulmonary and cancer predisposition processes. It does not exist in clinical practice guidelines for boarding and management of these patients, neither a suggestion of monitoring. METHODS: Automated search was requested file to the department of clinical records to identify patients diagnosed with Ataxia Telangiectasia. We included all that had clear and complete information to the variables analyzed. RESULTS: It was a description of variables by central tendencies and dispersion for continuous and categorical variables that were analyzed for frequencies and/or proportions. Included is determination of immunoglobulins IgG and IgA in 35 patients, 34 of 35 IgM and IgE in 9 patients. We observed in 5 patients hypogammaglobulinemia and 13 patients hypergammaglobulinemia. In relation to IgA, 17 patients had a deficiency and 6 of them high levels for their age, IgM in 13 patients reported figures above the percentile for their age. Altered IgE was found in one patient. IgG subclasses were determined in 9 patients and showed alteration in 9 of them. The IgG1 was not altered in anyone of the patients, low IgG2 was found according to age in 7 patients, 2 patients with low IgG3, and low IgG4 in 8 patients. The presence of lymphopenia was observed from the first test, in 14 from 28 patients. In a second measurement was observed in 17 from 25 patients. In the third measurement was observed in 13 form 21 patients. CONCLUSIONS: In the Ataxia Telangiectasia it has been reported that it could be affected almost all the subtypes and subclasses of immunoglobulins, as hypogammaglobulinemia that could be corrected by exogenous administration. We suggest supervise levels of IgG during follow-up of patients and establish decision-subclasses according to the possibilities of each working group, according to the context of clinical infections. Based on our result we suggested that the monitoring of this disease is through an algorithm of clinical boarding.