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Síndrome de Hunter - Overview

Nombres alternativos

Mucopolisacaridosis tipo II; Deficiencia de iduronato sulfatasa

Definición:

Es una enfermedad hereditaria en la cual las cadenas largas de moléculas de azúcar ( mucopolisacáridos) no se descomponen correctamente y se acumulan en el cuerpo.

Causas:

El síndrome de Hunter es una afección hereditaria y los niños varones son quienes resultan afectados con mayor frecuencia.

La afección es causada por la falta de la enzima iduronato sulfatasa. Sin esta enzima, los mucopolisacáridos se acumulan en diversos tejidos del cuerpo causando daño.

La forma severa y de aparición temprana de la enfermedad comienza poco después de los 2 años. Una forma leve y de aparición tardía hace que se desarrollen síntomas menos severos posteriormente en la vida.

  • Reviewed last on: 5/16/2011
  • 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

Muenzer J, Wraith JE, Beck M, et al. A phase II/III clinical study of enzyme replacement therapy with idursulfase in mucopolysaccharidosis II (Hunter syndrome). Genet Med. 2006 Aug;8(8):465-73.

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

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