1. Why is it so dangerous?
Cerebral edema and herniation (as well as seizures) are unique to acute hyperammonemia and usually occur only when arterial ammonia levels are > 200 μmol/L. Chronic brain damage has been noted.
2. What are the treatment choices?
Treat the intracranial hypertension created and treatment of cerebral edema.
Hypothermia can help, along with mannitol
Diltantin and Phenobarbital
Lactulose
Treat underlying urea splitting organism if present
Sodium Benzoate
IV arginine
PD/HD and CVVH have been used in the past!! and in most cases as a bridge to liver transplantation.
3. What are the causes?
Liver failure/cirrhosis
TPN,GI Bleed, Steroid use
Urea splitting organism infection, herpes infection
Urinary diversion
Multiple Myeloma
Budd chiari
Mushrooms/Herbs
Valproic Acid, carbamazepine, ribavarin, pyrimethamine use
Urea cycle disorders( usually children)
idiopathic hyperammonemia
TPN,GI Bleed, Steroid use
Urea splitting organism infection, herpes infection
Urinary diversion
Multiple Myeloma
Budd chiari
Mushrooms/Herbs
Valproic Acid, carbamazepine, ribavarin, pyrimethamine use
Urea cycle disorders( usually children)
idiopathic hyperammonemia
Few interesting points.
This can be very devastating and we might get called to do dialysis as a final removal of the ammonia. HD removes it the fastest. A nice review is listed below.
Another interesting cause that I learned was Multiple Myeloma and how that might lead to the elevated hyperammonemia syndrome needing dialysis. This might be associated with presence of peripheral blood myeloma cells and a possible MM with a burkitt type translocation t(2,8)(p12;q24)
Another interesting cause that I learned was Multiple Myeloma and how that might lead to the elevated hyperammonemia syndrome needing dialysis. This might be associated with presence of peripheral blood myeloma cells and a possible MM with a burkitt type translocation t(2,8)(p12;q24)
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