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Initial medical management guidelines In the initial medical management of acute hyperammonaemia, the patient should be immediately stabilized (circulation, airway and breathing), vital signs should be addressed, blood glucose levels monitored, intravenous access should be established, airways should be maintained by intubation and ventilation if necessary, and adequate rehydration should be started, typically using a dextrose-containing fluid at a high infusion rate 23

Solvent : For reconstitution, use a slightly acidic buffer (pH ~4-5) to slow deamidation

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Cysteine and glycine: Amino acids that support glutathione synthesis, contributing to cellular protection and reducing oxidative stress