Medical Definition of Maroteaux-Lamy syndrome
1.
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.
(05 Mar 2000)
Maroteaux-Lamy Syndrome Pictures
Click the following link to bring up a new window with an automated collection of images related to the term: Maroteaux-Lamy Syndrome Images
Lexicographical Neighbors of Maroteaux-Lamy Syndrome
Other Resources Relating to: Maroteaux-Lamy syndrome
