DEFINITION
Maple syrup urine disease is a disorder that occurs due
kekurangn enzyme required for the metabolism of amino acids. A byproduct of the
amino acids it causes urine smell like maple syrup.
CAUSE
Children with maple syrup urine disease are not able to
metabolize certain amino acids. Byproduct of amino acids is becoming accumulate
and cause neurological disorders, including seizures and intellectual
disorders.
SYMPTOMS
Products also causes body fluids, such as urine and sweat,
smell like maple syrup.
There are many forms of maple syrup urine disease. In the
most severe forms, infants affected with neurologic abnormalities, including
seizures and coma, when the first week of life and can die within a few days to
a few weeks. In the less severe cases, the child looks normal, but when the
child is exposed to infection, surgery, or obtain other physical stress,
children may experience vomiting, confusion, and even coma.
DIAGNOSIS
Several checks that can be done is the examination of plasma
amino acids and amino acid urine (urine). There will be signs of ketosis and
acidosis in the blood.
TREATMENT
Infants with severe disease is treated with dialysis. Once
the disease is under control, the patient should always consume special foods
containing three amino acids in low quantities (leucine, isoleucine, and
valine).
REFERENCE
- S, Lee M. Disorders of Amino Acid Metabolism. Merck Manual
Handbook. 2009.
- E, Chad H. Maple Syrup Urine Disease. Medline Plus. 2011.
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