Russian Consensus on “Hyperammonemia in Adults”: The 2021 Version (in English)

Hyperammonemia is an acute or chronic intoxication with ammonia and ammonium associated elevated levels in serum due to either its increased production and/or decreased detoxification. can result from a variety of causes clinically presents unspecific signs symptoms, including asthenia, encephalopathy, liver steatosis fibrosis, sarcopenia. With impaired function, hyperammonemia most frequently manifests (micro)encephalopathy. Thus case unexpect change mental status must be excluded as fast possible. An express method photometric assay informative enough determine the levels. The following classification proposed: a) by (normal level: ≤ 60 μmol/L; mild (Grade 1): 100 moderate 2): 200 severe 3): > μmol/L); b) etiopathogenesis (hereditary (congenital), functional (physiological), acquired (hepatic, extrahepatic, mixed)); c) clinical presentation (transient, recurrent persistent, constant (stable, without treatment), covert). Treatment for aimed at treating primary disease includes diet that restricted animal protein but contains sufficient vegetable protein, limited physical activities, use intestinal non-absorbable antibiotics (rifaximin- alpha) well pre- probiotics. L-ornithine- L-aspartate (LOLA) baseline therapeutic product administered number scenarios correct level hyperammonemia.