Maroteaux-Lamy syndrome

A form of mucopolysaccharidosis with onset before age three, Maroteaux-Lamy syndrome is characterized inability to metabolize dermatin sulfate. This leads to abnormal accumulation of dermatin sulfate, mostly in the peripheral tissues. The result in mild to severe changes in muscle, bone, skin, and other tissues, particularly the heart. Diagnosis is by examining leukocytes and cultured skin fibroblasts, or 24-hour urine collection to search for high levels of dermatin sulfate. There is no current treatment for Maroteaux-Lamy syndrome, but individual symptoms and problems may respond to physical therapy, medication, or surgery. Due to heart damage, death usually occurs before age 40. Also known as mucopolysaccharidosis Type VI. See also mucopolysaccharidosis.

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Mar·o·teaux-La·my syndrome mär-ō-'tō-lä-'mē- n a mucopolysaccharidosis that is inherited as an autosomal recessive trait and that is similar to Hurler's syndrome except that intellectual development is not retarded

Mar·o·teaux mȧ-rō-tō Pierre (b 1926)

French physician. Maroteaux served as director of genetics research at a medical research institute in Paris. His major field of interest was constitutional diseases of the bones.

La·my lȧ-mē Maurice Emile Joseph (1895-1975)

French physician. After a tenure as executive officer of a children's hospital in Paris, Lamy served from 1950 to 1967 as a professor at an institute for genetics research in Paris. He published works on scarlet fever immunity, twins, hereditary illnesses, and a handbook of medical genetics.

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Ma·ro·teaux-La·my syndrome (mah-ro-toґ lah-meґ) [P. Maroteaux; Maurice Emile Joseph Lamy, French physician, 1895–1975] see under syndrome.