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Revista Cubana de Pediatría

versión impresa ISSN 0034-7531versión On-line ISSN 1561-3119

Resumen

GUNTINAS ZAMORA, Ma. Victoria; TOLEDO RODRIGUEZ, Isabel; VARGAS DIAZ, José  y  POZO ALONSO, Albia J.. Inmunodeficiencia con ataxia telangiectasia: presentación de 4 casos. Rev Cubana Pediatr [online]. 2004, vol.76, n.1. ISSN 0034-7531.

4 patients with symptoms and signs typical of an immunodeficiency with ataxia telangiectasia from the clinical point of view and from the point of view of the serological and cellular markers are presented. In all the cases, ataxia was confirmed when the patients began to walk. Telangiectasies had a later appearance and the infections manifested at very early ages. The immunological defects of B and T cells were heterogeneous. The patients maintain infectious pictures despite being under immunostimulating treatment. The neurological disease is in progress. It is recommended the close follow-up of the cases due to the possible emergence of severe complications, such as chronic pulmonary disease or lymphoreticular neoplasias.

Palabras clave : TELANGIECTASIA [complications]; ATAXIA TELANGIECTASIA [immunology]; COMMUNICABLE DISEASES; RECURRENCE; IMMUNODEFICIENCY SYNDROMES; INFANT.

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