Treat underlying disorder
If hepatic encephalopathy:
Lactulose 20g PO or (300mL in 700cc H2O retention enema x30min)
In colon degrades into lactic acid: acidic environment traps ammonia
Also inhibits ammonia production in gut wall
If secondary to inborn error of metabolism
sodium phenylacetate/sodium benzoate (Ammonul) 250mg/kg in D10 over 90min; then 250mg/kg/d infusion
Arginine 210mg/kg IV/IO in D10 over 90min; then 210mg/kg/d infusion
+/- carnitine, 400 mg IV/IO in consultation w/specialist
May require dialysis if refractory/severe
Lactulose 20g PO or (300mL in 700cc H2O retention enema x30min)
In colon degrades into lactic acid: acidic environment traps ammonia
Also inhibits ammonia production in gut wall
In colon degrades into lactic acid: acidic environment traps ammonia
Also inhibits ammonia production in gut wall
sodium phenylacetate/sodium benzoate (Ammonul) 250mg/kg in D10 over 90min; then 250mg/kg/d infusion
Arginine 210mg/kg IV/IO in D10 over 90min; then 210mg/kg/d infusion
+/- carnitine, 400 mg IV/IO in consultation w/specialist
May require dialysis if refractory/severe
Hyperammonemia
Liver failure
Ketonemia
Inborn errors of metabolism with urea cycle defects and organic acidemia
Lactic acidosis
Shock, severe dehydration
Ketoacidosis
Renal failure