Nutricion saludable

Nutricion saludable
Comida Saludable

viernes, 5 de junio de 2009

PROGERIA

La progeria o síndrome de Hutchinson-Gilford es un síndrome poco frecuente. Consiste en la aparición de signos de envejecimiento en niños entre su primer y segundo año de vida. La mayoría de los casos de progeria son esporádicos, lo cual plantea la posibilidad de un patrón de herencia autosómico dominante por mutación de novo. El diagnóstico diferencial de esta entidad debe plantearse con cualquiera de los otros síndromes progeroides descritos en la literatura.

Los pacientes con progeria son niños sanos al nacer, quienes en el transcurso del primer a segundo año presentan rasgos de envejecimiento precoz y progresivo. Se describe que inicialmente aparecen placas esclerodérmicas en la piel de la cadera y región superior de las extremidades inferiores, zonas que cada vez son de mayor tamaño y que comprometen casi todo el cuerpo, respetando fundamentalmente los genitales y algunas áreas de los miembros inferiores. Al tiempo disminuye la producción de sudor por parte de las glándulas sudoríparas y empiezan a hacerse evidentes la hipotricosis y la alopecia. Como parte de los signos más tardíos se encuentran la hiperpigmentación de la piel de áreas expuestas a la luz solar, así como la hipoplasia de las uñas.


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