Scheie syndrome
Alternate 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
Prognosis (Expectations)
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.
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