Hunter syndrome - Treatment

Alternative Names

Mucopolysaccharidosis type II, Iduronate sulfatase deficiency

Treatment:

The U.S. Food and Drug Administration has approved the first treatment for Hunter syndrome. The medicine, called idursulfase (Elaprase), is given through a vein (intravenously). Talk to your doctor for more information.

Bone marrow transplant has been tried for the early-onset form, but the results can vary.

Each health problem should be treated separately.

Expectations (prognosis):

People with the early-onset (severe) form usually live for 10 - 20 years. People with the late-onset (mild) form usually live 20 - 60 years.

Complications:

Airway obstruction
Carpal tunnel syndrome
Hearing loss that gets worse over time
Loss of ability to complete daily living activities
Joint stiffness that leads to contractures
Mental function that gets worse over time

Calling your health care provider:

Call your health care provider if:

You or your child has a group of these symptoms
You know you are a genetic carrier and are considering having children

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.

References

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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