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Table 7 Dosage of drugs in acute hyperammonemia

From: Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia

Glucose IV L-carnitine IV Hydroxo-cobalamin# IV/IM Biotin IV/PO Sodium benzoate* (to be given IV in glucose 10%) 'sodium phenylbutyrate* (to be given IV in glucose 10%) §L-arginine-HCl* (to be given IV in glucose 10%) N-carbamyl-glutamate PO
Age dependent (see Table 8) 100 mg/kg as bolus, then maintenance 100 mg/kg/d 1 mg/day 10 - 40 mg/day 250 mg/kg as bolus in 90-120 min, then maintenance dose 250 mg/kg/d 250 mg/kg as bolus in 90-120 min, then maintenance dose 250 mg/kg/d 250 mg/kg as bolus in 90-120 min, then maintenance dose 250 mg/kg/d 100 mg/kg bolus, then 25-62 mg/kg every 6 h
  1. #Vitamin B12 is preferably given in the form of hydroxocobalamin; cyanocobalamin is less efficient but may be used temporarily.
  2. *Maximal daily drug dosages: sodium benzoate 5, 5 g/m2or 12 g/d, sodium PBA 5, 5 g/m2or 12 g/d, L-arginine 12 g/day.
  3. 'sodium phenylbutyrate should only be used in urea cycle defects or when the cause of hyperammonemia is unknown. In severe acute decompensation both sodium benzoate and sodium PBA/phenylacetate should be given in parallel as "ultima ratio". In less severe cases, a stepwise approach with initial sodium benzoate and if hyperammonemia persists or worsens, the addition of sodium PBA/phenylacetate can be chosen.
  4. §Arginine should only be used when the cause of hyperammonemia is unknown or when plasma arginine is low.
  5. Grade of recommendation: D.