False Diagnosis of Maple Syrup Urine Disease Owing to Ingestion of Herbal Tea

Abstract
Maple syrup urine disease is an inborn error of the metabolism of branched-chain amino acids named after the characteristic sweet aroma, reminiscent of maple syrup, present in the body fluids of affected patients.1 This aroma has been described variously as like that of burnt sugar, malt, curry, or Maggi (a widely available flavoring). We describe a case of “pseudo–maple syrup urine disease” caused by drinking fenugreek tea.

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  • Published by Wiley ,1999