J Paediatr Child Health. 2001 Aug; 37(4): 403-4.

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

Korman SH, Cohen E, Preminger A.

Departments of Clinical Biochemistry, Hadassah University Hospital, Jerusalem, Israel. korman@hadassah.org.il

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.