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maple syrup urine disease

maple syrup urine disease (aminoacidopathy) (may-pŭl si-rŭp) n. an inborn defect of amino acid metabolism causing an excess of valine, leucine, isoleucine, and alloisoleucine in the urine, which has an odour like maple syrup. Treatment is dietary; if untreated, the condition leads to mental retardation and death in infancy.

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maple syrup urine disease

maple syrup urine disease A rare genetic disease affecting the metabolism of the amino acids leucine, isoleucine, and valine leading to accumulation of high concentrations of these three amino acids and the immediate products of their metabolism in the plasma and urine. The urine has a characteristic smell like that of maple syrup.

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"maple syrup urine disease." A Dictionary of Food and Nutrition. . Encyclopedia.com. 23 Jan. 2018 <http://www.encyclopedia.com>.

"maple syrup urine disease." A Dictionary of Food and Nutrition. . Encyclopedia.com. (January 23, 2018). http://www.encyclopedia.com/education/dictionaries-thesauruses-pictures-and-press-releases/maple-syrup-urine-disease

"maple syrup urine disease." A Dictionary of Food and Nutrition. . Retrieved January 23, 2018 from Encyclopedia.com: http://www.encyclopedia.com/education/dictionaries-thesauruses-pictures-and-press-releases/maple-syrup-urine-disease

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