All of the following are accepted therapeutic measures initialated in hyperammonemic states in an infant except
Correct Answer: Exchange transfusion to remove ammonia
Description: Treatment for urea cycle defects,resulting in hyperammonemia and deranged aminoacid metabolism,is based on the management of acute presentation and also included removal of ammonia,inhibition of its production, along with treatment of any intercurrent illness & correction of dehydration or electrolyte imbalance.It includes intravenous administration of 10% dextrose with lipids(1 g/kg/24 hrs).Maintenance therapy includes nutritional management with restriction of protein,pharmacological therapy with sodium benzoate/phenylacetate(250-500 mg/kg/day),essential amino acids(0.25 g/kg/day) and arginine (200-600 mg/kg/day). Reference:Essential pediatrics-Ghai,8th edition,page no:654.
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