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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 .
The diagnosis of hepatic encephalopathy is a clinical one, once other causes for confusion or coma have been excluded; no test fully diagnoses or excludes it. Serum ammonia levels are elevated in 90% of people, but not all hyperammonaemia (high ammonia levels in the blood) is associated with encephalopathy.
Ammonia is normally metabolized by the liver; as cirrhosis causes both decreased liver function and increased portosystemic shunting (allowing blood to bypass the liver), systemic ammonia levels gradually rise and lead to encephalopathy. [137] Most pharmaceutical approaches to treating hepatic encephalopathy focus on reducing ammonia levels. [138]
Liver failure; Pancreatitis (these two usually occur in first 6 months and can be fatal) Leukopenia (low white blood cell count) Neutropenia (low neutrophil count) Pure red cell aplasia; Agranulocytosis; Extrapyramidal syndrome (including parkinsonism, may be reversible) Brain problems due to high ammonia levels; Low body temperature
Urea cycle disorders may also be diagnosed in adults, and symptoms may include delirium episodes, lethargy, and symptoms similar to that of a stroke. [14] On top of these symptoms, if the urea cycle begins to malfunction in the liver, the patient may develop cirrhosis. [15] This can also lead to sarcopenia (the loss of muscle mass). [15]
Patients presenting as acute and hyperacute liver failure are at greater risk of developing cerebral edema and grade IV encephalopathy. The pathogenesis remains unclear, but is likely to be a consequence of several phenomena. There is a buildup of toxic substances like ammonia, mercaptan, serotonin and tryptophan in the brain.
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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.
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