Maroteaux-Lamy syndrome /märōtō′ lämē′/ [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; the intermediate form has the same phenotype as pseudo-Hurler polydystrophy; and the mild form is difficult to distinguish from Scheie’s syndrome. Also called mucopolysaccharidosis VI. Compare Scheie’s syndrome. See also Hurler’s syndrome, pseudo-Hurler polydystrophy.
