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Contenido en el Idioma Español de University of Maryland Medical Center

Síndrome de Scheie - Treatment

Nombres alternativos

Mucopolisacaridosis tipo I S; MPS I S

Tratamiento:

La terapia de reemplazo enzimático para pacientes con un defecto en la enzima a-L- iduronidasa ahora es posible. Esto incluye no sólo a individuos con el síndrome de Scheie, sino a personas con los síndromes de Hurler y Hurler-Scheie.

El reconocimiento y tratamiento oportunos de la compresión de la médula espinal puede prevenir un daño permanente al nervio. Asimismo, se administra tratamiento para problemas cardíacos causados por válvulas permeables.

Grupos de apoyo:

The National MPS Society:www.mpssociety.org

Pronóstico:

El síndrome de Scheie es compatible con una expectativa de vida casi normal; sin embargo, es probable que se presenten algunas discapacidades como limitación de las articulaciones, ceguera o sordera posteriormente en la vida.

Posibles complicaciones:

Compresión de la médula espinal que puede conducir a la pérdida de la función nerviosa
Deformaciones de la extremidades
Hipoacusia y sordera
Problemas con la válvula aórtica
Problemas visuales

Cuándo contactar a un profesional médico:

Consulte con el médico si tiene síntomas de este trastorno.

Reviewed last on: 5/15/2011
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.

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