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What is the SYMPTOMS ?
- Feeding difficulties. - Avoiding food. - Urine smells like maple syrup. - Coma - Seizures - Lethargy What are the TREATMENTS ?
- The treatment involves eating a protein-free diet. Fluids, sugars, and possibly fats are given through a vein (IV). Peritoneal dialysis or hemodialysis can be used to reduce the level of abnormal substances. - Long term treatment requires a special diet. The diet includes a man-made infant formula with low levels of the amino acids leucine, isoleucine, and valine. |
What is MSUD ?
- Maple syrup urine disease (MSUD) is a rare inborn error in the metabolism of the keto acids of the three branched-chain essential amino acids leucine, isoleucine and valine. - The disease called Maple Syrup Urine Disease because the odor of the urine just maple syrup. What is the CAUSES ? - This disease are transmitted through an autosomal recessive gene of unknown frequency. - This condition is occurred when the amino acids (leucine, isoleucine and valine) are cannot break down. - That means, baby will easily be detected whether they have this gene or not. However, sometimes the infant which are normal in birth, will show the symptoms as poor feeding and apathy occur at the end of the first week after their births. The abnormalities that occur are elevated the blood levels of the branched-chain of amino acids and three keto acids which produces then excreted in the urine. |