Hyperammonemia

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Hyperammonemia
ICD-10 E72.2
ICD-9 270.6
DiseasesDB 20468
eMedicine neuro/162  ped/1057
MeSH D022124

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Hyperammonemia

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Overview

Hyperammonemia (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 non-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.

Types

Primary vs. secondary

Specific types

In all cases Hyperammonemia results from defects of the Urea cycle. The following list includes such examples:

Sequelae

Hyperammonemia is one of the metabolic derangements that contribute to hepatic encephalopathy.

See also

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