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Síndrome de Hurler - Treatment

Nombres alternativos

Deficiencia de alfa-L-iduronidasa; Mucopolisacaridosis tipo I; MPS 1 H

Tratamiento:

La terapia de reemplazo enzimático le agrega una forma funcional de la enzima faltante al cuerpo.

Se ha utilizado un trasplante de médula ósea en algunos pacientes con esta afección y el tratamiento ha tenido resultados variados.

Otros tratamientos dependen de los órganos que están afectados.

Grupos de apoyo:

Para mayor información y apoyo, se recomienda ponerse en contacto con una de las siguientes organizaciones:

The National MPS Society: www.mpssociety.org
Canadian Society for MPS and Related Diseases: www.mpssociety.ca
Society for MPS Diseases: www.mpssociety.co.uk

Pronóstico:

El síndrome de Hurler es una enfermedad con un pronóstico desalentador. Los niños con esta enfermedad presentan problemas del sistema nervioso y pueden morir a temprana edad.

Cuándo contactar a un profesional médico:

Consulte con el médico si:

Tiene antecedentes familiares de síndrome de Hurler y está considerando la posibilidad de tener hijos.
Su hijo empieza a mostrar síntomas del síndrome de Hurler.

Puente nasal bajo

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.

Referencias

Staba SL, Escolar ML, Poe M, et al. Cord-blood transplants from unrelated donors in patients with Hurler's syndrome. N Engl J Med. 2004 May 6;350(19):1960-9.

National Institute of Neurological Disorders and Stroke. Mucolipidoses Fact Sheet. Office of Communications and Public Liaison. Bethesda, MD; Publication No. 03-5115. February 13, 2007.

Wraith JE. Mucopolysaccharidoses and oligosaccharidoses. In: Fernandes J, Saudubray J-m, van den Berghe G, Walter JH, eds. Inborn Metabolic Diseases: Diagnosis and Treatment. 4th ed. New York, NY: Springer;2006:chap 39.

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