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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.
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]
Ornithine transcarbamylase deficiency also known as OTC deficiency is the most common urea cycle disorder in humans. Ornithine transcarbamylase, the defective enzyme in this disorder, is the final enzyme in the proximal portion of the urea cycle, responsible for converting carbamoyl phosphate and ornithine into citrulline.
The inability of the liver to metabolize ammonia to urea damages the brain cells. The cause is thought to be predominantly related to abnormal ammonia metabolism. [6] Other metabolic encephalopathies (often in conjuction with drowsiness or stupor), especially in decompensated cirrhosis or acute liver failure. Kidney failure and azotemia. Wilson ...
Hyperammonemia occurs when the body produces excess ammonia. This ammonia primarily exists as ammonium ion that has a concentration less than or around 35 μmol/L in normal referenced serum levels. [3] Excess ammonia is processed in the liver through the urea cycle to produce urea. [3]
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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
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