Scheie syndrome
Alternative Names
Mucopolysaccharidosis type I S; MPS I STreatment
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
Outlook (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.
Possible Complications
- Vision problems
- Hearing loss and deafness
- Extremity deformities
- Compression of the spinal cord, which can lead to loss of nerve function
- Problems with the aortic valve
When to Contact a Medical Professional
Call your health care provider if you have a family history of this disease or if you have symptoms suggestive of this disorder.
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