Maple Syrup Urine Disease

Maple syrup urine disease is a rare inherited disorder in which the body cannot properly break down the amino acids leucine, isoleucine and valine, so they and their toxic byproducts build up. Little research on supplements for it has been reviewed here so far, though a BCAA-free medical formula and thiamine are the main options discussed. Thiamine is thought to help only in thiamine-responsive variants, while carnitine, vitamin D and calcium play supporting roles.