Valoracion clinica de las inmunodeficiencias primarias. / Clinical evaluation of primary immunodeficiencies

Las manifestaciones clinicas de las inmunodeficiencias se presentan en la siguientes formas: A.-Aumento en la susceptibilidad a las infecciones. B.-Mayor incidencia de enfermedades autoinmunes. C.-Aumento de la frecuencia de neoplasias y D.-Mayor incidencia de enfermedades atopicas. El diagnostico debera estar basado en una buena historia clinica y en pruebas de laboratorios y gabinete: biometria hematica, teleradiografia de torax,dosificacion de inmunoglobulinas, pruebas cutaneas para inmunidad tardia como prueba a la tuberculina, candidina, varidasa, fitohemaglutinina,sensibilizacion con nitroclorobenceno.Pruebas in vivo para inmunidad celular: con nitroclorobenceno. Pruebas in vitro para inmunidad celular: conteo de linfocitos T, MIF reduccion de nitroazol de tetrazolio y dosificacion de algunas fracciones de complemento

[Severe mixed immunodeficiency. Report of a case]. / Inmunodeficiencia mixta grave. Presentacióm de un caso

The case of a 3 month old child with severe combined sex linked immunodeficiency is presented. The diagnosis was well doccumented, during his life. The child presented as a case of mucocutaneous moniliasis resistant to treatment. There was a history of similar cases in the family; diagnosis was made at post-mortem in one cousin and death occurred at early age in all kins so affected. Blood marrow transplant was not feasible in our case because histocompatibility was lacking in the kins studied. Three units of transfer factor were given as well as hyperimmune plasma but the child died in respiratory failure. Autopsy demonstrated pulmonary infection by Pneumocystic carinii and generalized citomegalic inclussion virus infection; almost complete absence of immune tissue was also demonstrated.