Abstract
Hyperammonemia is an acute or chronic intoxication with ammonia and ammonium associated with elevated ammonia levels in serum due to either its increased production and/or decreased detoxification. Hyperammonemia can result from a variety of causes and clinically presents with unspecific signs and symptoms, including asthenia, encephalopathy, liver steatosis or fibrosis, and sarcopenia. With impaired liver function, hyperammonemia most frequently manifests in (micro)encephalopathy. Thus in case of unexpect change in mental status hyperammonemia must be excluded as fast as possible. An express method of photometric assay is informative enough to determine the ammonia levels. The following hyperammonemia classification is proposed: a) by ammonia levels (normal level: ≤ 60 μmol/L; mild (Grade 1): ≤ 100 μmol/L; moderate (Grade 2): ≤ 200 μmol/L; and severe (Grade 3): > 200 μmol/L); b) by etiopathogenesis (hereditary (congenital), functional (physiological), acquired (hepatic, extrahepatic, mixed)); c) by clinical presentation (transient, recurrent or persistent, constant (stable, without treatment), covert). Treatment for hyperammonemia is aimed at treating the primary disease and includes a diet that is restricted in animal protein but contains sufficient vegetable protein, limited physical activities, and use of intestinal non-absorbable antibiotics (rifaximin- alpha) as well as pre- and probiotics. L-ornithine- L-aspartate (LOLA) is a baseline therapeutic product administered in a number of scenarios to correct the level of hyperammonemia.
Highlights
This results in an Myostatin, produced by myocytes, largely increased glutamate concentration in the extracellular inhibits the growth of muscle mass, and leads space, which in turn leads to cytotoxicity through the to its decrease [15], which in hyperammonemia in activation of excitatory neurotransmitters [13]
Pathogenetic therapy for hyperammonemia, regardless of the grade of severity, is aimed at treating the primary disease; this includes a diet that is restricted in animal protein and contains sufficient vegetable protein, limitation of physical exertion, and administration of intestinal non-absorbable antibiotics, or pre- and probiotics
Hyperammonemia is a condition characterized by an elevated level of ammonia and ammonium in the blood
Summary
Hyperammonemia is an acute or chronic intoxication with ammonia and ammonium associated with elevated ammonia levels in serum due to either its increased production and/or decreased detoxification. L-ornithine-L-aspartate (LOLA) is a baseline therapeutic product administered in a number of scenarios to correct the level of hyperammonemia. 6 ФГБОУ ВО «Южно-Уральский государственный медицинский университет» Минздрава России, 454092, Российская Федерация, Челябинская область, г. Улица Воровского, 64 7 ФГБОУ ВО Российский национальный исследовательский медицинский университет имени Н. И. Пирогова Минздрава России, Москва 117997, ул. 17 ФГБОУ ВО «Пермский государственный медицинский университет имени академика Е. А. Вагнера» Минздрава России, Россия, 614990, г. 4 ФГБОУ ВО Новосибирский государственный медицинский университет Минздрава России, Новосибирск, Российская Федерация, 630091, г. НИИ ОЧБ» ФМБА России, 197110 Россия, Санкт-Петербург, Пудожская ул., д. 7 ФГБОУ ВО РостГМУ Минздрава России, пер. Ключевые слова: гипераммониемия, азот, аммиак, аммоний, L-орнитин L-аспарагинат, рифаксимин-альфа, пробиотики, пребиотики, лактитол, лактулоза, минимальная печеночная энцефалопатия, фиброз печени. Given the large number of reports on the peculiarities of liver lesions related to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection [1], a team of experts who participated in the 23rd Congress of the Scientific Society of Gastroenterologists of Russia and the 15th National Congress of Therapists on November 19, 2020, decided to make additions to the Russian Consensus, “Hyperammonemia in Adults,” published in early 2020 [2, 3]
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