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Scheie syndrome - Treatment

Alternative Names

Mucopolysaccharidosis type I S; MPS I S

Treatment:

Enzyme replacement therapy for patients with a defect in the enzyme a-L-iduronidase is now possible. This includes individuals with Scheie syndrome, but also Hurler and Hurler-Scheie syndromes.

Early recognition and treatment of spinal cord compression can prevent permanent nerve damage. Treatment is also given for heart problems caused by leaky valves.

Support Groups:

The National MPS Society -- www.mpssociety.org

Expectations (prognosis):

Scheie syndrome is compatible with an almost normal lifespan. However, some disabilities such as limitation of joints, blindness, or deafness are likely to occur later in life.

Complications:

  • Compression of the spinal cord, which can lead to loss of nerve function
  • Extremity deformities
  • Hearing loss and deafness
  • Problems with the aortic valve
  • Vision problems

Calling your health care provider:

Call your health care provider if you have symptoms of this disorder.

  • Reviewed last on: 5/11/2009
  • Diana Chambers, MS, EdD, Certified Genetics Counselor (ABMG), Charter Member of the American Board of Genetic Counseling, University of Tennessee, Memphis, TN. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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