Pseudo-maple syrup urine disease due to maternal prenatal ingestion of fenugreek

J Paediatr Child Health. 2001 Aug;37(4):403-4. doi: 10.1046/j.1440-1754.2001.00617.x.


Fenugreek, maple syrup and the urine of maple syrup urine disease (MSUD) patients all share a characteristic odour originating from a common component, sotolone. Ingestion of fenugreek by mothers during labour resulted in a maple syrup-like odour in their newborn infants, leading to a false suspicion of MSUD.

Publication types

  • Case Reports

MeSH terms

  • Diagnostic Errors
  • Female
  • Humans
  • Infant, Newborn
  • Male
  • Maple Syrup Urine Disease / diagnosis*
  • Maternal-Fetal Exchange
  • Medicine, Traditional
  • Plant Extracts / pharmacology*
  • Pregnancy
  • Trigonella


  • Plant Extracts
  • fenugreek seed meal