Pseudo-maple syrup urine disease due to maternal prenatal ingestion of fenugreek.
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.
Korman
S H
SH
Departments of Clinical Biochemistry, Hadassah University Hospital, Jerusalem, Israel. korman@hadassah.org.il
Cohen
E
E
Preminger
A
A
D003951:Diagnostic Errors; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D008375:Maple Syrup Urine Disease / Q000175:diagnosis*; D008431:Maternal-Fetal Exchange; D008519:Medicine, Traditional; D010936:Plant Extracts / Q000494:pharmacology*; D011247:Pregnancy; D029910:Trigonella
D010936:Plant Extracts; C056164:fenugreek seed meal