Question of the Week

Question

Question :

Posted On : 19 Aug 2011

marble syrup urine disease
what is the available treatment_?

Expert Answer :

Answer Discussion :

A

arivoli kaliyan

0

specialised protein diet

13 years ago

B

Bal Mukund

0

acute stage- hemodialysis, peritoneal dialysis
chronis stage- diet devoid of branched chain amino acids, synthetic formulas

13 years ago

B

buddhala sudhakar

0

protein restricted diet

vitB1 ADMINISTRATION

CARNITINE ADMINISTRATION

13 years ago

S

sayed tohamy

0

short chain branched amino acids,leucin,isoleucin@valine

13 years ago

S

Srinivas prasad

0

no treatment

13 years ago

Answer 1

The 2 main aspects to the treatment of maple syrup urine disease {MSUD} are long-term management and the treatment of episodes of acute metabolic decompensation. The mainstay in the treatment of MSUD is dietary restriction of branched-chain amino acids. Aggressively treat episodes of metabolic decompensation. Initiate intravenous Glucose infusions {5-8 mg, kg, min for infants} as rapidly as possible.

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