maple-syrup urine disease

A form of maple syrup urine disease — Branched-chain ketoaciduria, an autosomal recessive metabolic disorder affecting branched-chain amino acids and causing the urine to have a distinctive sweet odour.

Meanings

nounmaple-syrup urine disease (uncountable)
  1. alternativeuncountableAlternative form of maple syrup urine disease.

    It is also elevated in phenylketonuria (35) and in maple-syrup urine disease (163, 164) as stated above.

    1965, Herbert Sprince, ““Abnormal Metabolites” of Amino Acid Origin”, in Anthony A. Albanese, editor, Newer Methods of Nutritional Biochemistry: With Applications and Interpretations, volume II, New York, N.Y.; London: Academic Press, →LCCN, section V (Indolic Compounds as Abnormal Metabolites), page 216
    2 more examples

    David, now nearly 3, is a living — and healthy — example of Georgia’s aggressive approach to screening all the state’s newborns for genetic diseases — even rare ones such as maple-syrup urine disease, the protein imbalance that is named for the sickly sweet-smelling urine it produces.

    1991 July 11, “Mandatory testing saves newborns”, in Tallahassee Democrat, 86th year, number 192, page 5A

    Most states offer other routine screening tests for newborns, including: […] • maple-syrup urine disease—to determine if some amino acids must be restricted for baby

    2004, Glade B. Curtis, Judith Schuler, Your Pregnancy Quick Guide: Tests And Procedures, Da Capo Press, →ISBN
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APA

maple-syrup urine disease. (n.d.). In WatkinsX Dictionary. Retrieved November 5, 2026, from https://dictionary.watkinsx.com/word/maple-syrup%20urine%20disease

MLA

“maple-syrup urine disease.” WatkinsX Dictionary, https://dictionary.watkinsx.com/word/maple-syrup%20urine%20disease. Accessed November 5, 2026.

Chicago

WatkinsX Dictionary, s.v. “maple-syrup urine disease,” accessed November 5, 2026, https://dictionary.watkinsx.com/word/maple-syrup%20urine%20disease.

Source: maple-syrup urine disease on Wiktionary, CC BY-SA 4.0.