See Also: Disease, maple syrup urine(health)
disease, maple syrup urine(medicine)
maple syrup urine disease(medicine)
maple syrup urine(medicine)
maple syrup(dictionary)
Maple syrup(recipes)
Porridge with maple syrup(recipes)
Maple syrup and pecan cake(recipes)
Canadian buttermilk pancakes with maple syrup(recipes)
American pancakes with crisp bacon and maple syrup(recipes)

Disease, maple syrup urine (health)


Hereditary disease due to deficiency of an enzyme involved in amino acid metabolism, characterized by urine that smells like maple syrup. The three branched-chain amino acids: (leucine, isoleucine, and valine) cannot be metabolized (processed) and build up in the blood to the detriment of brain function. Untreated maple syrup urine disease (MSUD) causes mental retardation, physical disability and death.