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Progeria - All Information

Nombres alternativos

Síndrome de progeria de Hutchinson-Gilford; SPHG

Definición:

Es una rara enfermedad genética que produce un envejecimiento rápido en los niños.

Causas:

La progeria es una rara afección que se destaca porque los síntomas se asemejan bastante al envejecimiento humano normal, pero ocurre en niños pequeños. Por lo regular, no se transmite de padres a hijos. En raras ocasiones, se observa en más de un niño en una familia.

Síntomas:

Pruebas y exámenes:

El médico llevará a cabo un examen físico y ordenará pruebas de laboratorio. Esto puede mostrar:

  • Resistencia a la insulina.
  • Cambios en la piel similares a los que se observan en la esclerodermia (el tejido conectivo se torna áspero y endurecido).

Las pruebas de esfuerzo cardíaco pueden revelar signos de ateroesclerosis temprana de los vasos sanguíneos.

Las pruebas genéticas pueden detectar cambios en el gen que causa la progeria.

Tratamiento:

No hay ningún tratamiento específico para la progeria.

Grupos de apoyo:

Progeria Research Foundation, Inc.: www.progeriaresearch.org.

Pronóstico:

La progeria causa la muerte prematura. Los pacientes generalmente sólo viven hasta los años de la adolescencia; sin embargo, algunos pacientes pueden vivir hasta poco más de los 20 años. La causa de muerte generalmente está relacionada con el corazón o un accidente cerebrovascular.

Posibles complicaciones:

Cuándo contactar a un profesional médico:

Solicite una cita con el médico si su hijo no parece estar creciendo o desarrollándose normalmente.

  • Reviewed last on: 8/4/2011
  • Chad Haldeman-Englert, MD, Wake Forest School of Medicine, Department of Pediatrics, Section on Medical Genetics, Winston-Salem, NC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

Referencias

Brown WT. Progeria. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th Ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 90.

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