Table 3.

Nutritional treatment of acute hyperammonaemia.

Mild Hyperammonaemia (<150 μmol/L) Alert Patient Good Oral Enteral Tolerance

Severe Hyperammonaemia (>150 μmol/L) Decreased Conscience Level Decreased Tolerance

Reduce protein supply. Provide small amounts of protein-free food (broth, fruits, juices, etc.) frequently. Protein-free caloric supplements might be required to ensure sufficient intake for age. In cases with oral intolerance or a mildly decreased state of consciousness, a nasogastric or gastrostomy tube can be useful. Special dietary formulas can be maintained. Follow individualized emergency nutritional recommendations.

Stop enteral nutrition. Stop protein supply until normal ammonia levels and no longer than 48 h. Ensure sufficient caloric administration. Using a 10% glucose + ions solution perfusion age-related rate of administration would be: 1–12 months: 8–10 mg/kg/min (5–6 mL/kg/h) 1–3 years: 7–8 mg/kg/min (4–5 mL/kg/h) 4–6 years: 6–7 mg/kg/min (3.5–4 mL/kg/h) 7–12 years: 5–6 mg/kg/min (3–3.5 mL/kg/h) Adolescents: 3–5 mg/kg/min (2.5–3 mL/kg/h) Adults: 3–5 mg/kg/min (2–3 mL/kg/h) Neonates: adequate for age fluid solution with 10–12 mg glucose/kg/min. If possible (available central line), consider a higher glucose concentration and less volume. Consider an insulin perfusion (0.05–0.2 U/kg/h) if persistent glucose levels >140–180 mg/dL.