Maple Syrup Urine Disease

Maple syrup urine disease is an inborn error of metabolism. Children with this disorder are unable to process certain protein building blocks. The condition got its name from the sweet odor of an affected infant's urine. Children with this disease may have symptoms such as avoiding foods and vomiting. Maple syrup urine disease is treated by avoiding protein in the diet. In severe cases, Children's Hospital offers liver transplant as a successful treatment option for children with maple syrup urine disease.