What puts a person at risk for maple syrup urine disease?

MSUD: Msud is a genetic, inherited disorder that involves an abnormality in the way your body processes amino acids. It is inherited in an autosomal recessive pattern, meaning that you need two copies of the defective gene, one from each parent, to exhibit signs of the disease. In other words, to be at ris for msud, both parents need to be carriers of the gene, or suffer from msud themselves.

Origin/genes: This is a rare defect of metabolism named for the sweet maple syrup smell of patients urine.It occurs in about 1/150,000 births worldwide with a higher frequency in the older Mennonite community.New cases appear when two asymptomatic "carrier" parents give their one defective gene to create the gene pair in the affected infant. Most parents of a new case never knew they were at risk.