On recognition of the infant's hyperammonemia, intravenous sodium phenylacetate and sodium benzoate (Ammonul
) was immediately administered, and he was transferred to a tertiary referral center for emergency hemodialysis.
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Since extracorporeal elimination treatments cannot be performed at this stage, patient was loaded with Na phenylacetate/Na benzoate (Ammonul
%10/%10 50 ml), with a dosage of 250 mg/kg for 2 hours, and then 250 mg/kg/24-hour infusion, based on previous studies on reducing branched-chain amino acid levels in urea-cycle disorder patients using Na phenylacetate/Na benzoate and NaPB [4-6].
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