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Hyperammonemia, or high ammonia levels, is a metabolic disturbance characterised by an excess of ammonia in the blood. Severe hyperammonemia is a dangerous condition that may lead to brain injury and death. It may be primary or secondary. Ammonia is a substance that contains nitrogen. It is a product of the catabolism of protein.
Carglumic acid, sold under the brand name Carbaglu among others, is used for the treatment of hyperammonaemia. [3] [6] [7] Carglumic acid is a carbamoyl phosphate synthetase 1 (CPS 1) activator.
During an acute hyperammonemic episode, oral proteins must be avoided and intravenous (I.V.) lipids, glucose and insulin (if needed) should be given to promote anabolism. I.V. nitrogen scavenging therapy (with sodium benzoate and/or sodium phenylacetate) should normalize ammonia levels, but if unsuccessful, hemodialysis is recommended.
Deficiency in N-acetylglutamate synthase or a genetic mutation in the gene coding for the enzyme will lead to urea cycle failure in which ammonia is not converted to urea, but rather accumulated in blood leading to the condition called type I hyperammonemia. This is a severe neonatal disorder with fatal consequences, if not detected immediately ...
These acute episodes are typically brought on by increased protein load [16] or intercurrent infections. [7] During metabolic crisis, moderate hyperammonemia, [7] hypoglycemia, and metabolic acidosis have been noted. [17]
This page was last edited on 28 May 2007, at 16:14 (UTC).; Text is available under the Creative Commons Attribution-ShareAlike 4.0 License; additional terms may apply ...
Hyperammonemia: A condition caused by high levels of ammonia, which is due to inborn errors of metabolism (including urea cycle disorder or multiple carboxylase deficiency), a diet with excessive levels of protein, deficiencies of specific nutrients such as arginine or biotin, or organ failure.
Hyperinsulinism-hyperammonemia syndrome (HIHA) due to glutamate dehydrogenase 1 gene. Can cause intellectual disability and epilepsy in severe cases. [3] Gastric dumping syndrome (after gastrointestinal surgery) Other congenital metabolic diseases; some of the common include Maple syrup urine disease and other organic acidurias