Maroteaux-Lamy syndrome

Ma·ro·teaux-La·my syndrome (mr-tlä-m, mä-rô-)

n.

An inherited defect in mucopolysaccharide metabolism characterized by excretion of dermatan sulfate in the urine, retarded growth, lumbar kyphosis, sternal protrusion, knock-knee, and usually enlargement of the liver and spleen. Also called type VI mucopolysaccharidosis.

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Maroteaux-Lamy syndrome

[märōtō′ lämē′]

Etymology: Pierre Maroteaux, French physician, b. 1926; Maurice Emile Joseph Lamy, French physician, 1895-1975

a mucopolysaccharidosis characterized biochemically by the predominance of the mucopolysaccharide dermatan sulfate in the urine and the presence of coarse granules in the leukocytes and clinically by Hurler-like signs with normal intelligence. There are three clinical forms: the severe or classic form shows Hurler-like symptoms (see Hurler's syndrome ); the intermediate form has the same phenotype as pseudo-Hurler polydystrophy; and the mild form is difficult to distinguish from the Scheie syndrome. Also called mucopolysaccharidosis VI.

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Maroteaux-Lamy syndrome

mucopolysaccharidosis VI.