There’s a life-threatening disorder that makes pee smell like maple syrup!

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. There are various forms of the disease and the classic form is the most serious. 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. Dietary restrictions and frequent blood chemistry testing is done.

Apart from the sweet smelling urine, the ear wax can also be affected by a sweet smell. Infants appear healthy at first, but if the disease goes untreated, then brain damage and death can occur.