Introduction: What is Maple Syrup Urine Disease?
Maple Syrup Urine Disease or branched-chain ketoaciduria is an autosomal recessive metabolic disorder affecting branched-chain amino acids. It is a type of organic acidemia. It makes the urine of infants smell like maple syrup. 1 in every 180,000 infants worldwide are affected, and there is a larger percentage in Mennonite and Amish communities. The condition is named because the smell of an affected infant's urine is sweet like maple syrup. It does not affect adults whatsoever if they are treated properly. It is passed down when two people have the recessive trait that causes MSUD.