Coronary artery thrombosis in ulcerative colitis is a serious condition and can occur in the young population. The authors report a case of multifocal atherosclerosis complicated by acute myocardial infarction due to atherosclerotic coronary thrombosis in a young woman with ulcerative colitis.

The article discusses the high relevance of the problem of inflammatory bowel diseases (IBD). First of all, attention is drawn to the annual increase in the incidence of ulcerative colitis and Crohn’s disease. Factors that can influence the development of IBD are assessed. A diagram of the pathogenetic cascade of dysbiotic disorders is presented, manifested by an increase in the blood serum of metabolic products of the intestinal microflora, microvascular, metabolic and immune changes leading to the triggering of inflammation in the intestinal wall.

Summary Post- COVID syndrome refers to the long-term consequences of a new coronavirus infection COVID-19, which includes a set of symptoms that develop or persist after COVID-19. Symptoms of gastrointestinal disorders in post- COVID syndrome, due to chronic infl ammation, the consequences of organ damage, prolonged hospitalization, social isolation, and other causes, can be persistent and require a multidisciplinary approach. The presented clinical practice guidelines consider the main preventive and therapeutic and diagnostic approaches to the management of patients with gastroenterological manifestations of postCOVID syndrome. The Guidelines were approved by the 17th National Congress of Internal Medicine and the 25th Congress of Gastroenterological Scientifi c Society of Russia.

Metabolic syndrome is a series of pathologies united by a similar pathogenesis, the end of which, most often, is cardiovascular accidents, which are leaders among the causes of death in the population around the world. Non-alcoholic fatty liver disease (NAFLD) is the hepatic equivalent of the metabolic syndrome, registered earlier than all other equivalents, on the rights of the liver as a first-line energy depot. At the same time, according to multicenter studies, 95% of people with NAFLD (any stage) are not diagnosed with the disease. Clarification of additional risk factors for NAFLD and the presence of a specific biomarker of non-alcoholic liver steatosis would make it possible to stop the vicious cascade of metabolic processes, which in the future can lead to a significant increase in the life expectancy of the population. The potentially high role of Secreted Frizzled Related Protein-4 (SFRP4) adipokine in the early diagnosis of NAFLD is known. The aim of the study was to optimize the early diagnosis of non-alcoholic fatty liver disease using modern indices and biomarkers. Materials and methods. The work was carried out at the Department of Faculty and Hospital Therapy of the Chuvash State University named after I. N. Ulyanov” in the period from 2016 to 2020. This study included several stages: first of all, a retrospective analysis of 1150 outpatient records of patients from several medical organizations of the Chuvash Republic for the period 2016-2018 was carried out. to form two study groups: experimental and control. At the second stage, as a result of applying the exclusion criteria, 162 people remained in the experiment: 110 from the experimental group, 52 from the control group. The subjects of both groups were compared by gender and age, the age range of the subjects varied from 18 to 80 years old with an average value of 48.3 years. Further, the patients undergo a detailed examination, according to the presented plan: Collection of complaints, medical history, objective examination. Laboratory studies (general and biochemical blood tests, lipidogram, assessment of the level of serum adipokine SFRP4). Instrumental studies (ultrasound of the OBP, TE (SAR), ESP with elastometry). Evaluation of the most informative complex indices for the early diagnosis of NAFLD: MI, IVO indices, HSI, FLD-I. Further, all the necessary statistical processing and analysis of the obtained data were performed (Microsoft Office Excel 2016, StatTech v. 2.8.8 (developer - Stattech LLC, Russia)). Results. Accessible (not requiring the use of additional time and material costs) NAFLD indices with the highest sensitivity rates (99.1% and 98.2%, respectively) were MI and IVO. A noticeable direct correlation was traced between MI (p=0.640), moderate - between the IVO (p=0.398) and the elastographically determined index of non-alcoholic liver steatosis. High sensitivity and specificity of skin manifestations (xanthoma, xanthelasma - 69.6% and 89.7% and seborrheic dermatitis - 82.0% and 71.4%) were found in relation to early manifestations of NAFLD. From anthropometric indicators: the CW/CF index has a pronounced (ρ=0.643), CW - moderate (ρ=0.238), and BMI - a weak direct (ρ=0.223) correlation with the elastographically determined index of non-alcoholic liver steatosis. Adipokine SFRP4 correlates (ρ=0.841) with early manifestations of hepatic steatosis in patients, as determined by TE in CAP mode.

Introduction. Clinical manifestations of inflammatory bowel diseases (IBD) are limited not only by the gastrointestinal tract syndromes, but also by a wide range of extra-intestinal manifestations, which include skin manifestations. Skin lesions in IBD occur in different forms. Some of them are associated with the activity of the main process, for example, gangrenous pyoderma. Others, such as psoriasis, are combined with pathogenetic factors common to IBD. Materials and methods. Three clinical observations of patients with IBD and extra-intestinal manifestations are presented: gangrenous pyoderma (case 1) and psoriasis (cases 2, 3). The stages of differential diagnostic search and treatment are described. The interleukin 12/23 inhibitor ustekinumab is prescribed to both bionaive patients (observations 1, 2), and to the patient who (observation 3) had high activity of the inflammatory process in the intestine and had received early treatment with GEBD. The extension of indications for use ustekinumab (ulcerative colitis, Crohn’s disease, psoriasis, psoriatic arthritis) of allows for its wider use in the treatment of patients. Conclusion. The interleukin 12/23 inhibitor ustekinumab has broad indications for use; its use allows to achieve a positive clinical, laboratory and endoscopic effect at the early stages of use. This effect has spread to skin manifestations, the reduction of which occurs in parallel with the healing of the intestinal mucosa.

AIM: to establish the consensus on controversial issues of the surgery for Сrohn’s disease by Delphi method. METHODS: a cross-sectional study was conducted by the Delphi method. 62 experts voted intramural and anonymous (31.03.23). 5 statements from the current edition of clinical guidelines were selected for correction by working group and further voting [2]. Based on the practical experience of the working group and literature data, 3 new statements were created also. Statements that do not reach the required level of agreement (80% or more) will be subjected to Round 2 of the Delphi method. RESULTS: all experts took part in the anonymous voting. The panel of experts is represented by 8 different areas of practical medicine and the median of the professional experience of the respondents was 30 (12–49) years. Of the 8 statements submitted for voting, consensus (80% or more) was reached on 6 out of 8. 2 statements have been revised by working group for the distance 2nd round of the Delphi study. Consensus (more than 80%) was reached on both. CONCLUSION: a cross-sectional study by the Delphi method provided the opinions of a panel of experts on controversial issues in the surgical treatment of Crohn’s disease. Statements that reach consensus will be included by the working group in a new edition of clinical guidelines of Crohn’s disease.

Clostridioides difficile infection (CDI) is the most common cause of antibiotic-associated diarrhea, and an important cause of nosocomial infection. Since the publication of the National Guidelines (2016, 2017), new data have been accumulated on the genetic structure and pathogenic properties of the most common causative agent of severe forms of antibiotic- associated diarrhea, which has led to the reclassifi cation of the pathogen, formerly known as Clostridium diffi cile, to Clostridioides difficile. Laboratory algorithms have been developed to diagnose CDI and determine the toxigenicity of strains reliably. New data on the effectiveness of antibacterials have been published, monoclonal antibodies to toxin B (bezlotoxumab) have been introduced into clinical practice to prevent CDI recurrence, and fecal microbiota transplantation has been proposed. Over the past 5 years, many international guidelines on the management of adult patients with CDI have also been updated (USA, EU). In the last decade, including due to the COVID-19 pandemic, there has been an increase in CDI incidence. Considering therelevance of CDI, new data on the pathogen, and domestic features, the Russian Scientific Medical Society of Internal Medicine, the Gastroenterological Scientific Society of Russia, and the North-West Society of Gastroenterologists and Hepatologists developed these clinical guidelines.

Post-Covid syndrome (PCS) is considered as a consequence of a previous coronavirus infection. The liver in COVID-19 is one of the most frequently affected organs, with the development of asthenia, cognitive impairment, as well as increased activity of alanine and aspartic transaminases (ALT and AST), which persist in some patients up to 4-6 months after discharge from the hospital. The cause of asthenia may be hyperammonemia (HA), which in COVID-19 is a manifestation of liver dysfunction against the background of the direct cytotoxic effect of SARS-CoV-2 on hepatocytes, which was previously shown as part of the clinical observational program protocol “LIRA - COVID”. Due to the great relevance of the problem of the combination of PCS, non-alcoholic fatty liver disease (NAFLD) and HA a post-hoc analysis of the observational clinical program LIRA - COVID was carried out. It was shown that NAFLD has a significant effect on the level of AST before the start of treatment with L-ornithine - L-aspartate (LOLA). The presence of NAFLD has a significant effect on ammonia levels after 14 days of treatment, since in the group of patients with NAFLD the ammonia level was significantly higher than in the group without NAFLD. It was concluded that it is advisable to include in the examination scheme of patients with PCS and NAFLD, determination of the level of ammonia in the blood, when elevated, the administration of the drug to such patients is justified LOLA course for 4 weeks at a standard dose of 9 g per day.

Background. First-line therapy does not always provide a high level of Helicobacter pylori eradication due to the increase of H. pylori resistance to antibiotics; therefore, it remains necessary to identify the most effective rescue treatments. The purpose of this study was to evaluate the efficacy and safety of empirical H. pylori furazolidone-containing regimens. Materials and methods. Adult H. pylori infected patients empirically treated with furazolidone-containing eradication regimens were registered in an international, prospective, multicenter non-intervention European registry on H. pylori management (Hp-EuReg). Data were collected at AEG-REDCap e-CRF from 2013 to 2021 and the quality was reviewed. Modified intention-to-treat (mITT) effectiveness analyses were performed. Results. Overall 106 patients received empirical furazolidone-containing therapy in Russia. Furazolidone was prescribed in a sequential scheme along with amoxicillin, clarithromycin and a proton pump inhibitor in 68 (64%) cases, triple regimens were prescribed in 28 (26%) patients and quadruple regimens in 10 (9.4%). Treatment duration of 7 days was assigned to 2 (1.9%) patients, 10-day eradication therapy in case of 80 (75%) and 14 days in 24 (23%) patients. Furazolidone was mainly used in first- (79%) and second-line (21%) regimens. The methods used to diagnose H. pylori infection were: histology (81%), stool antigen test (64%), 13C-urea breath test (6.6%), and rapid urease test (1.9%). The mITT effectiveness of sequential therapy was 100%; 93% with the triple therapy and 75.5% with quadruple therapy. Compliance was reported in 98% of cases. Adverse events were revealed in 5.7% of patients, mostly nausea (3.8%). No serious adverse events were reported. Conclusion. Furazolidone containing eradication regimens appear to be an effective and safe empirical therapy in Russia.

These recommendations are intended for primary care professionals: internists, general practitioners, paramedics and include a statement of the basic requirements for the follow-up of patients with a number of diseases of the digestive system. The main material of the methodological recommendations is presented by adapted sections from modern clinical recommendations of specialized societies on certain nosological forms of diseases and current regulatory federal documents on dispensary supervision of the adult population. The guidelines were approved by the Perm Regional United Organization “Professional Medical Community of the Perm Region” and the Ministry of Health of the Perm Region. Also, the main provisions of the recommendations were presented and approved at the National Congress of the Society of Therapists (November 19, 2021).

The National Registry of Patients with Inflammatory Bowel Disease (IBD) in the Russian Federation was established to study the epidemiological and clinical characteristics of patients and evaluate the actual clinical practice of medical and surgical treatment. AIM : to analyze the data of patients with IBD in the Russian Federation including clinical and demographic characteristics, medical status of patients, the frequency of use of various classes of drugs and response to treatment, the survival rates of advanced therapy drugs and the reasons for their cancellation. METHODS : from May 2017 to August 2021, depersonalized data of 3827 adult patients with IBD (ulcerative colitis (UC) – 2358 pts, Crohn's disease (CD) - 1469 pts) from 80 regions of the Russian Federation were included in the registry, both with previously and newly diagnosed UCor CD, who are in inpatient or outpatient care. RESULTS : in our population, the ratio of UC: CD was 1.6:1. The distribution of patients by sex was the same. The average age of patients in the registry was 40.6±13.1 (13-83 years) for UC and 38.5±14.3 (15-75 years) for CD, the half of patients were in the age range of 21-40 years for both diseases. The average age of disease onset did not differ for UC and CD and was 35.3 year (12-75 years) and 31.2 year (14-72 years) respectively. The duration between the onset of symptoms and the establishment of a diagnosis was 13.2 months in UC, and statistically significantly longer in CD - 34.8 months (P<0.01). The proportion of smokers in CD patients was statistically significantly higher than in UC ones (14.6% versus 9.6%, respectively, P<0.001). The incidence of disability was also significantly higher in CD than in UC patients (41.7% vs. 29.8%, P<0.01). The diagnosis of mild UC was established in 36% of cases, moderate UC occurred in 48.9% of patients, severe UC in 14.2% of patients. For the first time, the frequency of acute severe UC (1%) was estimated. The majority of patients had total UC (56.8%), 33% had left-side colitis, and 9.4% had proctitis. CD was divided by localization into ileocolitis 55.9%, terminal ileitis 23.9%, colitis 20.2%, perianal lesions were noted in 32.5% of cases. The overall complication rate in CD was 46% (681 patients), of which the most common were strictures - 48% and fistulas- 25.1%.Frequencyof extra intestinal manifestations did not differ in UC and CD patients and was 20.1% (473 patients) and 24.5% (360 patients), respectively. Of these, musculoskeletal lesions were more common (41.6% in UC, 42% in CD), lesions of the skin, eyes, mucous membranes, liver, anemia were also noted. In the treatment of IBD, steroids were used most often (79.3% and 65% in UC and CD, respectively), followed by 5-ASA - 47% in UC, 32.4% in CD. Immunosuppressant’s in CD were prescribed significantly more often (28.4%) than in UC (11%) (p<0.05). Biologics were used in 20.6% of UC patients and in 30% of CD patients. The highest 2-year survival of advanced therapy was noted for ustekinumab in CD (96%), for tofacitinib in UC (89.3%), and for vedolizumab in both UC and CD (92.5% and 88.4% respectively). The survival rates of all TNF-α inhibitors were approximately the same and varied within 58.1-72.4% in UC and 60-70% in CD. The most common reasons for discontinuation of advanced treatment were lack of efficacy/loss of response in both UC and CD. The second common reason was achieving remission. Certolizumab pegol in CD was canceled for this reason most often- 22.7% of cases. A small number of patients discontinued treatment due to adverse events: for UC - 1 patient each on adalimumab, golimumab, and tofacitinib, and 7 patients on infliximab, for CD- 5 patients on infliximab and adalimumab (9.6% and 7.5%, respectively) and 2 patients (4.6%) on certolizumab. Unfortunately, the proportion of discontinuation for non-medical reasons was significant and varied from 7% to 50% for different drugs. In some patients, the reason for discontinuation of therapy remained unknown. CONCLUSION : The difficulties of differential, often untimely diagnosis of CD and UC, the predominance of complicated and severe forms against the background of an increase in morbidity and prevalence, and at the same time the lack of adequate statistical accounting of CD and UC, make it necessary to create a unified clinical register of patients with IBD. The register of IBD patients will provide a holistic picture of the IBD situation in the country, including optimizing the use of budget funds for the treatment of patients with CD and UC, ensuring their rational planning.

The article reveals the high importance of taking into account the presence of skin changes when diagnosing diseases of the gastrointestinal tract. The following recommendations are given to practicing doctors: 1) for persons with rosacea, vulgar psoriasis and acne, comprehensive management by dermatologists-cosmetologists and gastroenterologists-hepatologists is advisable; 2) for early detection of NAFLD, the first contact physician is recommended to pay attention to the patient's presence of acne, rosacea and psoriatic changes, which can serve as a “marker of susceptibility” to NAFLD and should be taken into account in the process of early diagnosis of NAFLD.

Inflammatory bowel disease (IBD) is a chronic, relapsing, systemic and immune-mediated conditiondis that frequently involve extraintestinal manifestations. Latest studies showed increased risk of cardiovascular complications, which is the main cause of death in developed countries, in chronic inflammatory disorders, especially during IBD relapses. IBD patients are at increased risk of conditions such as early atherosclerosis, ischaemic heart disease, myocardial infarction, stroke, venous thrombosis, heart failure, аtrial fibrillation. Hypotheses for the mechanism underlying the association of IBD and atherosclerotic cardiovascular diseases include adverse effects of both the IBD itself (chronic inflammation, еndothelium dysfunction, dyslipidemia, thrombocytosis, gut microbiome dysfunction) and its treatment. The predominant role in atherogenesis is currently assigned to disruption of the endothelium. Endothelium plays an important role in physiologic regulation of vascular tone, cell adhesion, migration and resistance to thrombosis. Also, its dysfunction is associated with increased risk of atherosclerosis development. Early multifocal atherosclerosis is a serious complication of ulcerative colitis and can occur in young people without traditional cardiovascular risk factors. Untimely diagnosis, lack of pathogenetic treatment, correction of basic anti-inflammatory therapy and comprehensive consideration of a problem of high cardiovascular risk can lead to acute myocardial infarction and stroke and disability of a patient of working age. The authors present a case report of multifocal atherosclerosis complicated by acute coronary syndrome in a young man with ulcerative colitis, who required a radical revision of the therapy.

Epidemiological data suggests that approximately 10-65% of patients with mild to moderate COVID-19 have persistent symptoms already after an acute infection, which may last for six months or more. Previous clinical studies have shown that similar symptoms may also occur in the liver. Russian scientists have studied the phenomenon of hyperammonemia in the acute phase of COVID-19, but the problem of elevated blood ammonia levels in post - COVID syndrome has not been studied before. One possible option for the correction of hyperammonemia is the L-ornithine - L-aspartate (LOLA) preparation. The aim of the study was to investigate the manifestation of hyperammonemia in post - COVID syndrome and the possibility of its correction by using LOLA. Eighty-three patients (mean age 51.71 ± 12.82) with post-COVID syndrome, attention and memory impairment, chronic fatigue, hyperammonemia, 2-fold increase of alanine transaminase (ALT) and aspartate transaminase (AST) levels were included into multicenter, prospective observational study. L-ornithine-L-aspartate (LOLA) was given (3 g 3 times per day orally) for 4 weeks to correct hyperammonemia. Patients were accessed by the number connection test (NCT), fatigue evaluation test, measurement of ammonia and ALT, AST levels at baseline, and after 4 weeks. At week 4 the ammonia level was significantly decreased in 81 patients (97.6%). Time (sec) to complete the NCT was 49,43 ± 15,85 vs 64.67 ± 24.95 at baseline (p<0.001). Before treatment, the majority of patients had stage 1 hepatic encephalopathy (HE) - 50.6% and stage 1-2 (intermediate) HE - 30.1%, stage 2 HE - 10.9%, stage 3 HE - 2.4%. After LOLA treatment, the distribution changed: 41% of patients had no HE, 36.1% had stage 1 of HE, 22.9% had stage 1-2 (intermediate) of HE, and stage 2 and 3 of HE was no longer defined. The mean score in fatigue evaluation test was 46,27 ± 10.0 vs 64,71 ± 19,18 at baseline (p<0.001). ALT and AST levels were 47,83 ± 38,21 units/l vs 95,75 ± 68.99 units/l at baseline and 40,10 ± 35,97 units/l vs 78,51 ± 90,90 units/l at baseline respectively (p<0.001). This study showed that the inclusion of LOLA in a dose of 3 g 3 times a day for 4 weeks was associated with significant improvement in the objective and subjective outcomes in patients with post-COVID syndrome.

Objective. We analyzed clinical features and laboratory markers of COVID-19 patients according to favorable outcomes versus fatal outcomes. Material and methods. The medical history of 80 patients was analyzed: 51 patients with favorable outcomes were included in group №1, 29 patients with a fatal outcome were included in group №2. Demographic data, duration of the disease, comorbid-ities, laboratory markers, and results of the instrumental studies were included. The ammonia level in the peripheral blood was de-termined by the express method using a PocketChem BA 4140 photometric portable analyzer. Results. Patients in group №2 were older (68±11 years) had hypertension stage 3 with high cardiovascular risk;every third had a history of myocardial infarction. At admission, patients from group №2 were most likely with febrile fever and high levels of inflammatory markers — predictors of a cytokine release syndrome. In addition, 71% of patients at admission had elevated ammonia levels. Hyperammonemia correlated with high ferritin levels, leukopenia, non-alcoholic fatty liver disease in patients, and lethal outcomes. Conclusions. The risks of poor COVID-19 outcomes are higher in comorbid patients of the older age group. Hyperammonemia may be one of the predictors of poor COVID-19 outcomes. © 2022, Media Sphera Publishing Group. All rights reserved.

Диагностика болезни Крона – сложный, нередко длительный процесс, в основе которого лежит последовательный дифференциально-диагностический поиск. У значительной доли пациентов с болезнью Крона могут развиться внекишечные проявления. Нередко поражение кожи предшествует манифестации кишечной симптоматики или возникает параллельно с кишечными проявлениями. Спектр их достаточно широк, а механизм возникновения неоднороден. В частности, выделяют аутоиммунные внекишечные проявления, связанные с активностью воспалительного процесса, которые появляются вместе с основными кишечными симптомами обострения и при современном назначении адекватной патогенетической терапии разрешаются параллельно со снижением степени активности кишечного воспаления. Поражения кожи при воспалительных заболеваниях кишечника, по данным различных авторов, встречаются в 6-43% случаев. Изменения кожи при воспалительных заболеваниях кишечника объясняют общими патогенетическими механизмами: гиперпродукцией ИЛ-12 и интерферона гамма, синтезируемых Th1-лимфоцитами, ИЛ-5 и ИЛ-13, продуцируемых Th2-лимфоцитами, ИЛ-17 – Th17-лимфоцитами. Одним из тяжелых кожных поражений при болезни Крона является гангренозная пиодермия, воспалительный нейтрофильный дерматоз, характеризующийся болезненными язвенными дефектами кожи (язвы полициклических очертаний с приподнятыми краями и покрытые некротическими массами) и часто ассоциирующийся с заболеваниями внутренних органов. В статье приводится описание клинического cлучая пациента с болезнью Крона и гангренозной пиодермией. Описаны этапы дифференциально-диагностического поиска и лечения. Продемонстрирована высокая эффективность ингибитора интерлейкина-12/23 устекинумаба в достижении клинического, лабораторного и эндоскопического эффекта уже на ранних этапах лечения. При этом параллельно с заживлением слизистой терминального отдела подвздошной кишки редуцировали и кожные проявления. Данный клинический случай также подтверждает важность таких факторов, как тяжесть течения заболевания и наличие внекишечных проявлений, при выборе первого биологического препарата для терапии болезни Крона. Diagnosis of Crohn's disease is a complex, often lengthy process, which is based on a consistent differential diagnostic search. A significant proportion of patients with Crohn's disease may develop extraintestinal manifestations. Often, skin lesions precede the manifestation of intestinal symptoms or occur in parallel with intestinal manifestations. Their spectrum is quite wide, and the mechanism of occurrence is heterogeneous. In particular, autoimmune extraintestinal manifestations associated with the activity of the inflammatory process are isolated, which appear along with the main intestinal symptoms of exacerbation and, with the modern appointment of adequate pathogenetic therapy, are resolved in parallel with a decrease in the degree of activity of intestinal inflammation. Skin lesions in inflammatory bowel diseases, according to various authors, occur in 6-43% of cases. Skin changes in inflammatory bowel diseases are explained by common pathogenetic mechanisms: hyperproduction of IL-12 and interferon-gamma synthesized by Th1-lymphocytes, IL-5 and IL-13 produced by Th2-lymphocytes, IL-17 by Th17-lymphocytes. One of the severe skin lesions in Crohn's disease is pyoderma gangrenosum, an inflammatory neutrophilic dermatosis characterized by painful ulcerative skin defects (ulcers of polycyclic outlines with raised edges and covered with necrotic masses) and often associated with diseases of the internal organs. The article describes a clinical case of a patient with Crohn's disease and pyoderma gangrenosum. The stages of differential diagnostic search and treatment are described. The interleukin 12/23 inhibitor ustekinumab has been shown to be highly effective in achieving clinical, laboratory and endoscopic effects already at early stages of treatment. At the same time, in parallel with the healing of the mucosa of the terminal ileum, skin manifestations were also reduced. This clinical case also confirms the importance of such factors as the severity of the course of the disease and the presence of extraintestinal manifestations when choosing the first biological drug for the treatment of Crohn's disease.

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.