Maple syrup urine disease is a disease inherited where the body is unable to process certain protein building blocks (amino acids) properly. Symptoms of this disease includes: coma, feeding difficulties, lethargy, seizures, urine that smells like maple syrup, and vomiting. Tests performed to search for this disease include: Plasma amino acid test, Urine organic acid test, or genetic testing. When diagnosed, treatment involves eating a protein-free diet. Fluids, sugars and sometimes fats are given through a vein (IV). Long-term treatment required a special diet. Infants need a formula that has low levels of amino acids leucine, isoleucine, and valine.
