What is the treatment for maple syrup urine disease?
The two main approaches to the treatment of maple syrup urine disease (MSUD) include (1) long-term daily dietary management and (2) treatment of episodes of acute metabolic decompensation. The mainstay in the treatment of maple syrup urine disease is dietary restriction of branched-chain amino acids (BCAAs).Click to see full answer. Accordingly, what treatment option is…
The two main approaches to the treatment of maple syrup urine disease (MSUD) include (1) long-term daily dietary management and (2) treatment of episodes of acute metabolic decompensation. The mainstay in the treatment of maple syrup urine disease is dietary restriction of branched-chain amino acids (BCAAs).Click to see full answer. Accordingly, what treatment option is the best for maple syrup urine disease?Orthotopic liver transplantation is an effective therapy for classic MSUD. Prevention of primary manifestations: Dietary management should allow age-appropriate tolerance of leucine, isoleucine, and valine, and maintain stable plasma BCAA concentrations and BCAA concentration ratios.Subsequently, question is, how do you get maple syrup urine disease? Maple syrup urine disease (MSUD) is inherited, which means it is passed down through families. It is caused by a defect in 1 of 3 genes. People with this condition cannot break down the amino acids leucine, isoleucine, and valine. This leads to a buildup of these chemicals in the blood. Thereof, what is the life expectancy of someone with maple syrup urine disease? If untreated, life-threatening coma or respiratory failure could occur within 7 to 10 days and most will die within several months. Upon any lapse of treatment, classic MSUD can cause brain damage. People with the disease are particularly prone to crisis during illness, infection, fasting, or after surgery.What part of the body does maple syrup urine disease affect?Maple syrup urine disease is an inherited disorder in which the body is unable to process certain protein building blocks (amino acids) properly. The condition gets its name from the distinctive sweet odor of affected infants’ urine. If untreated, maple syrup urine disease can lead to seizures, coma, and death.
