Definição:
Systemic lysosomal storage disease marked by progressive physical deterioration and caused by a deficiency of L-sulfoiduronate sulfatase. This disease differs from 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.
Sinônimos:
gargolismo da sandrome de hunter /
sandrome de hunter /
deficiaancia de sulfoiduronato sulfatase /
deficiaancia de iduronato 2-sulfatase /
DOENÇAS
Doenças Nutricionais e Metabólicas