The primary aims of
treatment are to lower the concentration of toxic substances in the body and to
prevent further accumulation.The mainstay in the treatment of MSUD is dietary
restriction of branched-chain amino acids.
Acute
treatment
Acute
interventions are often required as soon as the diagnosis is made, or when a
person already diagnosed seeks medical attention for acute neurological
symptoms. They may be triggered by fasting, infections, injuries or other
stressful situations, but in some cases the precipitating factor is not so
obvious.
Administering
glucose and minimising and regulating protein and amino acid intake helps in lowering the concentration of toxic substances in the body. Glucose stimulates
insulin release, which in turn blocks protein degradation. Special amino
acid solutions can also be administered intravenously. In acute situations the
risk of brain oedema must be considered, as specific treatment is required.
Blood sodium and glucose imbalances may occur, and there is a risk of
developing pancreatitis.
This involves minimizing the intake of
branched-chain amino acids and staying away from any potential attack triggers. In mild
forms of the disease it is often sufficient to restrict protein intake. In
intermittent forms, with normal levels of amino acid between attacks, dietary
restrictions are sometimes not needed. However, for majority of the time, special formulas are required to compensate the nutrients lost as it contains all the essential
amino acids except branched-chain amino acids. These formulas can be supplemented with
low protein products and small, carefully measured amounts of natural
protein.
The aim is to maintain the level of branched-chain amino acids in the
body within a safe and acceptable range. The diet of the patient should be adjusted according to the blood concentration of the amino acids.Nutrients such as vitamins, minerals and trace elements should also be supplied in
sufficient amounts. It can be difficult to maintaining adequate
levels of isoleucine and valine while striving to lower leucin to acceptable
concentrations. Dietary suppliments may be necessary due to low valine and isoleucine levels which can cause nutritional deficiency symptoms.
It has been shown to prevent future organ damage in MSUD, but
does not reverse complications already present. The new liver can supply enough
functional enzyme to regulate amino acid concentrations, even without dietary
restrictions.
All surgical
procedures imply risks to individuals with MSUD and certain safety precautions
should be taken, as both anaesthesia and the surgical intervention cause stress
and trauma. Surgery should be carried out when the metabolic balance is
as good as possible and certain safety precautions should be taken such as minimal preoperative fasting and administration of intravenous glucose
administered throughout the surgery. Additionally catabolism should be avoided before and after the
surgery, blood concentration of amino acids monitored and the patient should be given special medicinal formulas as early as
possible after the procedure.
References:
MSUD Family Support Group, 2009. Description and Treatment of Maple Syrup Urine
Disease. [online]. Available at:< http://www.msudsupport.org/index.php?option=com_content&view=article&id=219%3Adescription-a-treatment-of-maple-syrup-urine-disease&Itemid=120>
The New York Times, 2011. Maple Syrup Urine Disease. [online]. Available
at:<http://health.nytimes.com/health/guides/disease/maple-syrup-urine-disease/overview.html>
Medscape Reference, 2012. Maple Syrup Urine Disease. [online]. Available
at:<http://emedicine.medscape.com/article/946234-treatment>
Out of all these treatment, which is the most commonly used?
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ReplyDeleteIs the dietary treatment a long term or short term solution? And is there any other potential treatment or avenues for research that you would suggest?
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