The maple syrup urine disease affects infants and is so named because it causes the urine to have a sweet maple syrup scent. The disease is technically called branched-chain ketoaciduria. It is an autosomal recessive metabolic disorder affecting branched-chain amino acids. The disease affects 1 out of 180,000 infants. Interestingly, the disease affects Amish, Mennonite, and Jewish children the most. Babies affected by the disease eat poorly, vomit, become dehydrated easily, lethargy, have seizures and neurological decline. It’s a very serious disease and can be fatal if not properly managed. Apart from the sweet smelling urine, ear wax can also be affected by a sweet smell. Infants appear healthy at first, but if the disease goes untreated, brain damage and death can occur.
Posted on: Thu, 06 Jun 2013 14:44:15 +0000
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