Metabolically associated fatty liver disease is a major public health problem in all countries. In 2020, the criteria for diagnosing the disease have fundamentally changed, risk factors have been specified, which requires a revision of existing approaches to therapy. Metabolically associated fatty liver disease, formerly called non-alcoholic fatty liver disease, is a hepatic manifestation of a multisystem disorder. The 2020 consensus of international experts proposed new diagnostic criteria for metabolically associated fatty liver disease. The diagnosis is made in the presence of steatosis (according to instrumental methods and/or biomarker studies and/or histological signs) in combination with one or more additional criteria: overweight or obesity, type 2 diabetes mellitus or metabolic disorders. The purpose of the literature review is to present the results of modern clinical studies and meta-analyzes on the effect of physical activity and, in particular, aerobic exercise on the course of the disease. Results. Exercise programs have proven effective in treating disease. All types of aerobic exercise – continuous, interval and combination – have a positive effect on triglyceride levels and body mass index. Moreover, continuous and intermittent aerobic exercise improves liver enzymes, in particular alanine aminotransferase, compared to the management of patients without physical activity. Meta-regression analysis showed that shorter intervention times (≤ 12 weeks) were more effective in reducing alanine aminotransferase levels. In clinical practice, it is not always possible to achieve targets. The limiting factors are the fact that patients with concomitant obesity and other comorbid pathology do not comply with the necessary physical activity regimens, do not reach the target indicators for energy consumption. Equally important are the issues of control of load tolerance, taking into account age, gender and other characteristics. Effective physical activity programs for metabolically associated fatty liver disease should be based on energy targets, effective and safe regimens, and tailored to the individual patient.

Evaluation of the effectiveness of cycloferon when used in complex therapy and for the prevention of chronic Helicobacter pyloriinduced gastritis with gastric erosions associated with Epstein – Barr virus. The study included 105 patients with chronic Helicobacter pylori-induced gastritis with gastric erosions associated with the Epstein – Barr virus, divided into 2 groups: group 1 (65 patients) – patients who were prescribed the antiviral drug cycloferon in addition to anti-helicobacter therapy (12,5% 2,0 ml intramuscularly, the course of administration of the drug depended on the number of copies per 10⁵ cells of the Epstein – Barr virus infiltrate); group 2 (40 control patients), who were prescribed only anti-helicobacter therapy. In each group, patients were distributed depending on the severity of the disease and the number of copies of Epstein – Barr virus per 10⁵ infiltrate cells. The detection of Helicobacter pylori (H. pylori) was carried out by three methods: a rapid urease test based on the results of a Helpil test, a histological examination of smears taken from gastrobiopaths, and a PCR biopsy. To determine the number of Epstein – Barr virus copies per 10⁵ infiltrate cells, the PCR method was used using reagent kits for detecting Epstein – Barr virus DNA in clinical material with real-time hybridization-fluorescence detection Amplisens Epstein – Barr virus-screen-FL. In patients with chronic Helicobacter pylori-induced gastritis with gastric erosions, the use of meglumine acridone acetate in antihelicobacter therapy reduces the severity of the disease and the frequency of exacerbations. Interferon inducers, in particular meglumine acridone acetate, belong to a new class of antiviral drugs. Possessing bifunctionality, meglumine acridone acetate combines a wide range of pharmacological effects, which makes it possible to use it as a therapeutic and prophylactic agent.

The article describes the course of the cholestatic variant of congenital cytomegalovirus infection in children with an assessment of the obstetric and gynecological history of mothers and the characteristics of the newbornneonatal period. Pregnancy in women proceeded with signs of fetal-placental insufficiency, which led to the birth of children with a low gestational age, since cytomegalovirus infection developed in an acute form. The newborns had a low Apgar score and signs of hypotrophy of I-II degrees. The clinical picture of congenital cytomegalovirus hepatitis was characterized by a protracted course and had an undulating character, a complete recovery of mass-growth parameters, a symptom of jaundice took place within 3 months. It was revealed that a longer cholestasis syndrome was observed in children who had both congenital cytomegalovirus infection and impaired bilirubin uptake, in particular, Gilbert's syndrome. Ursodeoxycholic acid is the only generally accepted starting drug for the drug effect on cholestasis syndrome in children with liver disease. Complex therapy with the use of the drug Ursodeoxycholic acid at a dose of 15-20 mg/kg per day, at the early stages of treatment, this infectious pathology made it possible to stabilize the clinical and laboratory parameters. In the absence of complaints and clinical manifestations, the levels of ALT, AST and ALP were restored for a long period within 5-6 months, while the cholesterol values returned to normal by 3 months. The chosen scheme of therapy with Ursodeoxycholic acid at a dose of 15-20 mg/kg per day contributed to the prevention of severe complications from the biliary tract, such as chronic cholecystocholangitis, primary biliary cirrhosis. The use of therapeutic tactics, taking into account the individual characteristics of young children, was not accompanied by the presence of side effects.

The article discusses modern approaches to the treatment of ulcerative colitis. It is indicated that for the choice of tactics and treatment algorithm, the extent of the lesion, as well as the severity of the current exacerbation, classified as mild, moderate and severe, are important. The criteria are useful for assessing the need for hospitalization, but do not take into account such important signs as nocturnal symptoms, extraintestinal manifestations, endoscopic activity. The severity of an exacerbation of a disease characterizes its severity at the moment, but may not reflect the long-term cumulative burden of the disease. The extent of the lesion, in turn, may influence the choice of route of administration. There are: proctitis (spread of inflammation to the rectosigmoid angle), left-sided (inflammation is limited to the splenic flexure) and widespread lesion (inflammation extends proximal to the splenic flexure, including pancolitis). To achieve the goals of therapy, the time period from the start of treatment to the expected clinical response, remission and endoscopic healing was determined. The immediate and short-term goals are clinical response and remission, as well as the normalization of the level of C-reactive protein. Reduction of fecal calprotectin to the optimal range is recommended as an official intermediate treatment goal. The degree of increase in the concentration of fecal calprotectin correlates with the severity of inflammation, and with a mild degree of the disease, its values may be normal or borderline. In this situation, repeated monitoring of fecal calprotectin over time can clarify the clinical picture. This was demonstrated in a recent prospective cohort study, where consistent determination of fecal calprotectin at 1-month intervals was the best predictor of exacerbation before the onset of clinical symptoms. Along with endoscopic mucosal healing, the long-term goal is to restore the quality of life and the absence of disability. The article discusses the use of drugs of the mesalazine group, topical and systemic steroids for the treatment of ulcerative colitis, as well as approaches to monitoring the disease and long-term management of patients.

Irritable bowel syndrome is a chronic disease of the gastrointestinal tract, with such characteristic symptoms as pain and discomfort in the abdomen in the absence of an organic pathological process, the frequency of which in recent years has begun to increase due to post-COVID-19 postinfectious irritable bowel syndrome. Purpose of the study was to evaluate the effectiveness of including the probiological drug in the therapy of patients with post-infectious irritable bowel syndrome after a previous COVID-19 infection. We examined 70 patients with postinfectious irritable bowel syndrome that developed after a previous infection with COVID-19. The patients were divided into 2 groups. The main group consisted of 35 people who were treated with irritable bowel syndrome based on the latest recommendations of the Russian Gastroenterological Association, including antispasmodics, antidiarrheals. Additionally, the patients of the main group were included in the combined probiotic enteric capsules for 2 weeks at a dose of 3 capsules per day. In patients of the main group, whom included the probiotic in therapy, there was a more pronounced relief from the clinical manifestations of the disease with an overall assessment of the effectiveness of treatment of 82,7% in the main group and 56,9% in the comparison group. When assessing the indicators in points according to the IBS-SSS test and the IBS-QOL scale, both groups of patients showed statistically significant improvements in indicators (p < 0.001), however, the best indicators of intestinal symptoms and quality of life were in the main group. The combined probiotic is an interesting combination in the treatment of patients with post-COVID-19 postinfectious irritable bowel syndrome, contributing to a more pronounced regression of the clinical manifestations of the disease and improving the quality of life of patients.

The article describes a case of Mucha – Habermann varioliform (smallpox-like) parapsoriasis in a teenager. Parapsoriasis is a heterogeneous group of chronic inflammatory dermatoses that are characterized by the presence of spots and/or papules and plaques covered with small scales. The relevance of parapsoriasis is due to the ambiguity of the etiopathogenetic mechanisms of the disease, the lack of a generally accepted classification. Interest in the disease is partly due to the possibility of transformation of parapsoriasis to T-cell lymphoma (fungal mycosis). Description of the clinical case. Patient A., 14 years old, went to the skin and Venereological dispensary in Surgut with complaints of multiple rashes on the skin of the trunk and limbs, accompanied by minor soreness, burning and moderate itching. Ill for about 10 days, the beginning of the disease was associated with hypothermia. The appearance of the rash was preceded by a sharp rise in body temperature to 38 °C, chills, weakness, malaise. Objectively: the pathological skin process was widespread. Rashes were localized in the torso area on the skin of the chest, back, abdomen, upper and lower limbs. It is represented by multiple miliary and lenticular papules of pink-red color, pustules. In the Central part of most elements, zones of necrosis or ulcerative defects from 0,2 to 2 cm in diameter, hemorrhagic crusts were visualized. The diagnosis of acute varioliform parapsoriasis was made on the basis of clinical and anamnestic data and the results of additional research methods. The treatment used antibacterial drugs, detoxification methods, physiotherapy (light therapy). The outcome of treatment is improvement. When actively called after 6 months, hypertrophic and smallpox-like scars of the skin of the trunk and extremities were found during examination. This clinical example is of interest to dermatovenerologists and pediatricians. It illustrates the relative goodness of the course of dermatosis, despite the brightness and acuteness of clinical manifestations. Timely treatment has significantly improved both the child's condition and quality of life. Due to the rare occurrence of acute lichenoid parapsoriasis, doctors may experience difficulties in making a diagnosis. The case is presented in order to attract the attention of the medical community to the problem of parapsoriasis.

Breast milk oligosaccharides represent a main component of human milk and are contained in it in an amount of 5 to 20 g/l, which is below only lactose and lipids and is competing with the content of total protein in this respect. Breast milk oligosaccharides are composed by a large number (about 200) of oligosaccharide structures, which are based on a structural link based on D-glucose, D-galactose, and N-acetylglucosamine monosaccharide residues, which undergoes additional modification to varying degree by fucose and sialic acid. Breast milk oligosaccharides are unique to human breast milk and practically do not occur in the milk of dairy farm animals, such as cow's milk. Initial ideas about the role of breast milk oligosaccharides as an exclusively «bifidus factor» have now undergone significant changes. So, numerous studies have shown a variety of beneficial effects of breast milk oligosaccharides in the development of the child's body, which includes a stimulating effect on the formation of a normal microbiome of the colon, the ability to form an effectively functioning protective intestinal barrier, binding and neutralizing a large number of pathogenic microorganisms and viruses, immunoregulatory action. Individual components of breast milk oligosaccharides, produced using modern bioengineering technologies, in particular, 2'-fucosyllactose (2'FL), have become very popular recently for the enrichment of infant formulas, replacing traditionally used types of prebiotics, such as galactooligosaccharides, fructooligosaccharides and inulin. In numerous experimental and clinical studies, the expediency and effectiveness of including breast milk oligosaccharides in the composition of adapted milk formulas have been confirmed. Good tolerability of such mixtures and their ability to provide indicators of the physical development of children within the normal range have been shown. In some works, a positive effect of artificially obtained breast milk oligosaccharides on the formation and composition of the intestinal microbiota in children of the first year of life has been established. The clinical effectiveness of the use of infant formula enriched with breast milk oligosaccharides in relation to the incidence of acute respiratory infections has been shown. Thus, breast milk oligosaccharides produced by the methods of modern biotechnology are a safe and effective functional component of milk formulas for healthy infants. Research to expand the use of this components in the composition of children's nutrition products, including medicinal products, is ongoing.

Despite significant progress in haemophilia treatment, achieved in Russia recently, number of unmet medical needs still exist in routine clinical practice. Particularly, the annual bleeding rates are still high, leading to frequent development of synovitis and haemophilic arthropathy, resulting in chronic pain and impaired joint motility. Thus, the quality of life of patients with haemophilia is not optimal. One of potential approaches to improve the situation could be more wide use in routine clinical practice recombinant clotting factors with extended half-life. According to modern clinical guidelines on haemophilia treatment, such products could potentially significantly decrease number of infusions for prophylactic therapy without decrease in efficacy of haemostasis control or keeping the same infusion frequency could increase the clotting factors activity, improving the patient’s protection from bleeds. Thus, use of recombinant clotting factors with extended half-life can increase the patient’s compliance to treatment and the results of therapy. One of such molecules is single-chain recombinant clotting factor VIII (lonoctocog alfa). The modification of this molecule leads to improvement of its stability and affinity to von Willebrand factor, allowing to increase the intervals between infusion up to 2-3 times per week for all age groups. This article describes the clinical trial program of lonoctocog alfa and summarize the results of its use among haemophilia patients in Russia. Large international clinical program AFFINITY has demonstrated high efficacy and favorable safety profile of lonoctocog alfa and the analysis of Russian patient’s treatment results demonstrated, that they are in line with such results worldwide.

Congenital epidermolysis bullosa refers to orphan (rare – no more than 10 cases per 100,000 population) diseases. In the Russian Federation, its prevalence rate in 2016 was 3,9 per 1 million population. The objective was to study the clinical and genetic characteristics, prevalence, and family history of сongenital epidermolysis bullosa patients in the Republic of Dagestan. The clinical picture, features of the course and family history of 127 patients with the diagnosis of сongenital epidermolysis bullosa living in the Republic of Dagestan were studied, and the type and subtype of the disease were verified. In 12 patients, the genetic nature of сongenital epidermolysis bullosa was confirmed by full-exomal DNA sequencing. According to the severity of сongenital epidermolysis bullosa, patients were divided into four groups. Most of them were in the group with mild to moderate severity: Weber – Cockayne сongenital epidermolysis bullosa – 53 (41,73 ± 4,38%, n = 127) and Dowling – Mear herpetiform – 11 (8,66 ± 2,5%, n = 127), dominant dystrophic congenital epidermolysis bullosa – 3 (2,36 ± 1,35%, n = 127) and recessive generalized non – mutating dystrophic congenital epidermolysis bullosa – 1 (0,79 ± 0,79%, n = 127), borderline dystrophic congenital epidermolysis bullosa – 8 (6,3 ± 2,16%, n = 127), and Kindler syndrome – 1 (0,79 ± 0,79%, n = 127). Patients with generalized recessive dystrophic congenital epidermolysis bullosa were included in the group with severe or very severe course-7 (5,51 ± 2,07%, n = 127) and 21 (16,54 ± 3,32%, n = 127), respectively. More than half of the cases of congenital epidermolysis bullosa in the Republic of Dagestan (63,78 ± 4,26%) were children, up to 6,3 ± 2,16% were aged from 56 to 63 years, which required a differentiated approach in their management. The majority of patients with recessive dystrophic congenital epidermolysis bullosa and borderline dystrophic congenital epidermolysis bullosa had closely related parental marriages, which was most typical for 12 districts of the Republic.