A person ingesting herbal tea may have urine that has the odor of maple syrup, causing an unnecessary workup for maple syrup urine disease (MSUD).
The cause of the odor is sotolone (4,5-dimethyl-3-hydroxy-2[5h]-furanone), which is the chemical causing the odor in maple syrup urine disease.
Possible clinical scenario:
(1) An infant or young child ingests an herbal tea, possibly for an illness.
(2) The child is taken to a clinician.
(3) A urinalysis is ordered and the presence of an odor of maple syrup is noted.
(4) The patient is worked up for MSUD.
Ways to avoid an unnecessary workup:
(1) Ask about any herbal remedies taken recently.
(2) Recollect urine the next day, at which point the sotelone should have cleared.
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Specialty: Clinical Laboratory, Obstetrics & Gynecology, Cardiology, Emergency Medicine, Critical Care
