hurler–scheie syndrome hurler–scheie syndrome hurler–scheie syndrome is a genetic disorder caused by the buildup of glycosaminoglycans (gags) in various organ tissues. it is a cutaneous condition, also characterized by mild mental retardation and corneal clouding. respiratory problems, sleep apnea, and heart disease may develop in adolescence. hurler-scheie syndromeother namesmucopolysaccharidosis type i h-sstructure of dermatan sulfate, one of the molecules that accumulates in the lysosomes of mps i patientsusual onsetage 3-8 yearscausesdeficiency of the alpha-l iduronidase enzymedifferential diagnosisother forms of mps i; hunter syndrome; other mucopolysaccharidosestreatmentenzyme replacement therapy with iduronidase; surgeryprognosislife expectancy is generally into the late teens or early 20s, but may vary depending on the severity of the diseasefrequency1:115,000 (hurler-scheie syndrome/intermediate) hurler–scheie syndrome is classified as a lysosomal storage disease. patients with hurler–scheie syndrome lack the ability to break down gags in their lysosomes due a deficiency of the enzyme iduronidase. all forms of mucopolysaccharidosis type i (mps i) are a spectrum of the same disease. hurler-sheie is the subtype of mps i with intermediate severity. hurler syndrome is the most severe form, while scheie syndrome is the least severe form. some clinicians consider the differences between hurler, hurler-scheie, and scheie syndromes to be arbitrary. instead, they classify these patients as having "severe", "intermediate", or "attenuated" mps i.