URL: http://www.nlm.nih.gov/medlineplus/ency/article/000373.htm
URL: http://ghr.nlm.nih.gov/condition=maplesyrupurinedisease
URL: http://www.pearsonsuccessnet.com/snpapp/login/login.jsp
Tuesday, April 22, 2008
Treatment for the diaease
Treatment involves a protein-free diet. During a flare up, fluids, sugars, and fats are given by IV. Peritoneal dialysis, or hemodialysis are used to reduce the level of amino acids.
A special diet free of branched-chain amino acids is started immediately.
Long term treatment requires a special diet. The diet includes a synthetic infant formula with low levels of the amino acids leucine, isoleucine, and valine. Persons with this condition must remain on this diet permanently. It is very important to always stick to this diet to prevent neurological damage. This requires frequent blood tests and close supervision by a registered dietitian and a physician, as well as parental cooperation.
A special diet free of branched-chain amino acids is started immediately.
Long term treatment requires a special diet. The diet includes a synthetic infant formula with low levels of the amino acids leucine, isoleucine, and valine. Persons with this condition must remain on this diet permanently. It is very important to always stick to this diet to prevent neurological damage. This requires frequent blood tests and close supervision by a registered dietitian and a physician, as well as parental cooperation.
Wednesday, April 16, 2008
New Info
About 1 in 185,000 infants are effected each year. The genes that are mutated include BCKDHA, BCKDHB, DBT, and DLD.
Subscribe to:
Comments (Atom)