Maple+Syrup+Urination+Disease



====**CAUSE!!!** Maple syrup urination disease is also called branched-chain ketoaciduria. It's an autosomal metabolic disorder that affects the branched-chain amino acids. This disease gets its name from the distinct sweet smell of an infected infant's urine. This disease affects 1 out of 180,000 people and is most common in people of Amish, Mennonite, and Jewish ancestry. There is four genes that have become mutated causing a person to have this disease, BCKDHA, BCKDHB, DBT, and DLT. Maple syrup urine disease is a recessive autosomal disease, so both parents have to at least be carriers for their child to have the disease and, even then, there's only a 25% chance of inheritance.====

For infants
 * Poor feeding
 * Vomiting
 * Poor weight gain
 * Increasing lethargy (difficult to wake up)
 * Characteristic burned sugar smell to urine
 * Changes in muscle tone, muscle spasms, and seizures.
 * Coma, brain damage, and death are possible.

Treatment
Treatment for MSUD involves a diet that would involve absolutely no protein. During a flare up, fluids, sugars, and fats are given to the person through an IV. Dialysis is sometimes used to reduce the levels of amino acids. A diet that has no branched-chain amino acids is started immediately after finding out a person has MSUD. Anyone who has the disease has to stay on their specific diet for as long as they live so that they don't get brain damage. In order to make sure that their diet is working correctly for them, they have to get blood tests done regularly. __
 * MSUD have been known in infants for more than 70 years
 * In 1954 is where they really started to research MSUD.
 * The MSUD have been added to the birth room machines to check newborns before they are given to the parents in that last 50 years.
 * MSUD has been recorded to interfere with intellectual development.
 * In 1954 it was reported that 4 infants died within the first 3 months of their lives because of MSUD.

Research sites
 * 1) [|http://emedicine.medscape.com/article/946234-overview]
 * 2) []