Dietary
treatment
Minimise the intake of branched-chain amino acids and to avoid
potential attack triggers. In mild forms of this disease, it is often
sufficient to restrict protein intake. In most cases, replacing of dietary
proteins with special formulas containing all important amino acids except for
branched chain amino acids will be sufficient enough. These formulas can be
supplemented with low protein products and small, carefully measured amounts of
natural protein.
The goal of the treatment is to maintain the level of
branched-chain amino acids in the body within a safe range.
Dietary management of MSUD may be quite tedious, and should be
regulated and carried out with the assistance from experienced dietician from a
medical centre for diagnosis and treatment of metabolic disorders.
Liver Transplant
Liver transplantation is another alternative option for treatment
of MSUD. As the liver consists of new functioning enzymes, they are being used
to replace those abnormal enzymes that causes MSUD and therefore a successful
liver transplant will allow the patient to not have severe dietary restrictions
which will in turn lessen the risk of having a recurrence of the symptoms of
MSUD. Liver transplantation is a great solution to treat MSUD, however there are
some risk factors to it as well. For example, organ rejection from the
patients, risk of surgery, anti-rejection medications are needed to be
monitored in this liver transplant process.
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