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.

Medical dictionary. 2011.

Look at other dictionaries:

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  • syndrome — The aggregate of symptoms and signs associated with any morbid process, and constituting together the picture of the disease. SEE ALSO: disease. [G. s., a running together, tumultuous concourse; (in med.) a concurrence of symptoms, fr. syn,… …   Medical dictionary

  • Lamy — Maurice, French physician, 1895–1975. See Maroteaux L. syndrome …   Medical dictionary

  • Maroteaux — Pierre, French medical geneticist, *1926. See M. Lamy syndrome …   Medical dictionary

  • Liste der Syndrome — Diese Seite listet in alphabetischer Reihenfolge und ohne Anspruch auf Vollständigkeit Syndrome und Komplexe aus unterschiedlichen medizinischen Fachgebieten auf. Bitte nur Verweise auf den tatsächlichen Titel des Beitrags und keine… …   Deutsch Wikipedia

  • Hunter syndrome — Classification and external resources ICD 10 E76.1 ICD 9 277.5 …   Wikipedia

  • Hurler syndrome — Classification and external resources ICD 10 E76.0 ICD 9 277.5 …   Wikipedia

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