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2016, Número 3

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Alerg Asma Inmunol Pediatr 2016; 25 (3)


Inmunodeficiencias del complemento. Revisión de la literatura Parte I. Generalidades y deficiencias de la vía clásica

Fuentes PJM, Jiménez PEN, Espinosa PSE

Idioma: Español
Referencias bibliográficas: 22
Paginas: 84-88
Archivo PDF: 221.06 Kb.


RESUMEN

Las inmunodeficiencias del complemento se definen como trastornos hereditarios del sistema inmunológico que conducen a la ausencia total del nivel o función de la proteína. Podemos encontrar deficiencias en vías de activación del complemento: clásica alterna, lectinas, o proteínas reguladoras. Entre las deficiencias de la vía clásica encontramos: deficiencia de C1q, C1r/s, C4, C2 y C3, las cuales se asocian de menor a mayor grado a lupus eritematoso sistémico e infecciones por microorganismos piógenos. El tratamiento incluye medidas de soporte y se ha reportado un caso de un adolescente Pakistaní que a la edad de 16 años se realizó trasplante de células hematopoyéticas de donador vivo relacionado (hermano) con buenos resultados.


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