Tratamiento de interferón gamma recombinante en niños con Esclerodermia
Keywords:
esclerodermia, interferón gamma recombinanteAbstract
La esclerodermia se caracteriza por la presencia de fibrosis en múltiples órganos y sistemas, afectando fundamentalmente la piel, tubo digestivo, pulmones, corazón y riñones. La afectación básica es un trastorno de la microcirculación y del tejido conectivo, lo que conlleva lentamente a obliteración vascular y fibrosis secundaria en los órganos diana. Su tratamiento es difícil. Las terapias se dirigen a mejorar la circulación periférica y a disminuir la fibrosis con diversas terapéuticas entre las que se encuentra el Interferón gamma recombinante. Se utilizó el INF ? R en 15 pacientes pediátricos, 13 niñas y 2 niños, 10 Blancos y 5 mestizos. La edad fue de 8 ± 4 años al momento de iniciar tratamiento. Los tipos de esclerodermia: 14 localizadas, (7 morfeas y 7 lineales) y una ESP. Con tratamientos anteriores: 9 con D-penicilamina, 7 con Vit E. La evaluación de los score de Rodnan modificado demostró una disminución de los mismos en 66.7% de los pacientes y estabilización en otros 3. Existió una disminución promedio de 1 y 3 puntos, en los score 1 y 2, respectivamente. En ambos casos las disminuciones (final vs inicio) fueron estadísticamente significativas (P<0.05). En total el 80% mejoró o estabilizó el estado de la enfermedad, lo cual en gran medida se corresponde con lo obtenido en estudios previos. El IFN g fue bien tolerado. Estos resultados sugieren que el IFN g (Heberon Gamma R®), producto cubano, puede tener un efecto beneficioso en el tratamiento de la esclerodermia, al menos en su forma localizadaDownloads
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