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Maroteaux-lamy syndrome

Maroteaux-lamy syndrome

(Science: biochemistry, syndrome) An error of mucopolysaccharide metabolism due to deficiency of the lysosomal enzyme arylsulphatase B.

It is characterised by excretion of dermatan sulfate in the urine, growth retardation, lumbar kyphosis, sternal protrusion, genu valgum, usually hepatosplenomegaly, and no mental retardation.

Onset occurs after two years of age.

Inheritance: autosomal recessive.

Synonym: polydystrophic dwarfism, mucopolysaccharidosis type VI.


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