What Is Maple Syrup Urine Disease?
Reviewed: Dr. Gromatzky
1. Where Did the Name Maple Syrup Urine Disease Come From?
Maple syrup urine disease gets its name due to the smell of the urine associated with individuals who have the disorder. Sufferers from the condition can go into a type of metabolic emergency during which the urine begins to smell strong and sweet, similar to the smell of maple syrup.
Not all areas of the world are familiar with the smell of maple syrup. An herb called fenugreek, often used by breastfeeding mothers to increase milk production, has a similar smell. In areas unfamiliar with maple syrup, the smell of fenugreek in the urine can be used as a diagnostic tool for MSUD.
