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What medical treatment is given to those with MSUD?
When diagnosed as an infant specific baby formula is made in place of the breast milk. Then one becomes older with MSUD they are given a specific diet that is free or has low levels of protein. This is a life long diet that must be followed, because of inability to breakdown the proteins: leucine, isoleucine, and valine. This is a mandatory treatment that a patient must follow to prevent neurological damage that could lead to death.
Other options are given if there are complications. For instance, in rare incidents hemodialysis and peritoneal dialysis is used to remove the harmful levels of amino acids in the blood. Hemodialysis, according to the Mayo Clinic, is a machine that filters out harmful substances in the blood and peritoneal dialysis is another way to filtrate blood, but can be done when traveling, at work, or at home.
Then if MSUD needs to be treated with aggression IV infusions of glucose are given due to a metabolic decomposition and insulin if needed.
These harsher treatments of hemodialysis, peritoneal dialysis, and IV infusions of glucose and insulin are only when high levels of branched-amino acids accumulate in the blood. Otherwise the best treatment for MSUD is following a strict dietary plan monitored by ones primary physician and dietary physician. Also a routine blood test is given every week and after 6 - 12 months every month to monitor amino acid levels in the blood.
Sources:
O'Halloran, R. (2014, March 17). What Is Maple Syrup Urine Disease?
Retrieved from
http://hubpages.com/health/What-Is-Maple-Syrup-Urine-Disease
Bodamer, O. (2014, November 4). Maple Syrup Urine Disease Treatment
& Management.
Retrieved from
http://emedicine.medscape.com/article/946234-treatment#d6
Mayo Clinic. (n.d.). Retrieved from http://www.mayoclinic.org/
Haldeman-Englert, C. (2013, May 7). Maple syrup urine disease:
MedlinePlus Medical
Encyclopedia. Retrieved from
https://www.nlm.nih.gov/medlineplus/ency/article
/000373.htm
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