Abstract

TERAN ANTEZANA, Claudia Marcela  and  MUZNER JIMENEZ, Ximena Karina. IPEX SYNDROME - IMMUNODYSREGULATION, POLYENDOCRINOPATHY AND ENTEROPATHY, X - LINKED. Rev. méd. (Cochabamba) [online]. 2010, vol.21, n.1, pp. 69-73. ISSN 2074-4609.

We describe the case of a Newborn patient who develops Diabetes Mellitus Neonatal on 2nd day of life, the 8th day starts diarrhea for Rotavirus and then Amoebae, with persistence of the diarrhea despite treatment for these pathologies, presents recurrent anemia needing several blood transfusions, recurrent septic resistant to antibiotics, cutaneous Ictiocitosis, elevations of Ig E and Eosinofilia as proper characteristics of the pathology. Due to the persistent Diarrhea, Diabetes Mellitus is difficult to control in spite of the insulin therapy and constant changes in the dose, presents septic that cause her the death.

Keywords : Síndrome de IPEX; Autoinmunidad; Sépsis.