(or 'hyperammonaemia') is a metabolic disturbance characterised by an excess of ammonia
in the blood. It is a dangerous condition that may lead to encephalopathy
and death. It may be primary or secondary.
Ammonia is a substance that contains nitrogen. It is a product of the catabolism of protein. It is converted to the less toxic substance urea prior to excretion in urine by the kidneys. The metabolic pathways that synthesise urea are located first in the mitochondria and then into the cytosol. The process is known as the urea cycle, which comprises several enzymes acting in sequence.
Primary vs. secondary
The following list includes such examples:
Treatment centers on limiting intake of ammonia and increasing its excretion. Dietary protein (a source of ammonium) is restricted and caloric intake is provided by glucose and fat. Intravenous sodium phenylacetate and sodium benbenzoate
are pharmacologic agents commonly used to treat hyperammonemia. Sodium phenylacetate and sodium benzoate can serve as alternatives to urea for the excretion of waste nitrogen. Phenylacetate conjugates with glutamine
to form phenylacetylglutamine, which is excreted by the kidneys. Similarly, sodium benzoate reduces ammonia content in the blood by conjugating with glycine
to form hippuric acid
, which is rapidly excreted by the kidneys. A preparation containing sodium phenylacetate and sodium benzoate is available under the trade name Ammonul.
Hyperammonemia is one of the metabolic derangements that contribute to hepatic encephalopathy