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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

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Key Considerations for Standard Maintenance Dose: Frequency: Monthly injections are standard for maintenance