Maple Syrup Disease in Children
Maple syrup urine disease (MSUD) is an autosomal recessive genetic disease caused by a congenital defect in branched ketoacid dehydroxylase, which results in the obstruction of the catabolism of branched amino acids. It is named because children with the disease excrete a large amount of α-keto-β-methylvaleric acid in their urine, which has the sweet smell of maple syrup.
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Site of disease:
whole body -
Infectious :
Not contagious -
Frequent population:
child -
Related symptoms:
Full fontanelle baby feeding difficulties unsteady gait hypertonia weight loss -
Concurrent disease:
Related Drugs
Gliclazide Tablets
Indication: Non-insulin dependent diabetes mellitus (type 2)
Manufacturer:
Beijing Shuguang Pharm Co., Ltd.
Related Ingredients