Systemic lysosomal storage disease marked by progressive physical deterioration and caused by a deficiency of L-sulfoiduronate sulfatase. This disease differs from MUCOPOLYSACCHARIDOSIS I by slower progression, lack of corneal clouding, and X-linked rather than autosomal recessive inheritance. The mild form produces near-normal intelligence and life span. The severe form usually causes death by age 15.
See also reference (FX) in Slovak
iduronátsulfatáza mukopolysacharidóza I
See also reference (FX) in English
Iduronate Sulfatase Mucopolysaccharidosis I
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