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Experimental and Clinical Gastroenterology

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No 8 (2019)

LEADING ARTICLE

4-10 259
Abstract
The occurrence of hepatocellular carcinoma in patients with non-alcoholic fatty liver disease without cirrhosis is of particular relevance in view of the increasing number of patients, the absence of predictors of the development of the disease and its late diagnosis. The article is devoted to the analysis of pathogenetic aspects of hepatic carcinogenesis with the participation of bile acids and intestinal microbiome and promising directions in early diagnosis and possible prevention of the disease.

CLINICAL GASTROENTEROLOGY

11-15 296
Abstract
The aim of the research was to study the relationship between plasma concentrations of resistin and homocysteine and indicators of enzyme and pigment metabolism in patients with non-alcoholic fatty liver disease (NAFLD) and its combination with type 2 diabetes mellitus (DM-2). Materials and methods. A total of 90 patients were examined, including patients with NAFLD (n=20) and its combination with DM-2 with normal body weight (n=20) and obesity (n=50), as well as 20 healthy volunteers. A complex of clinical, laboratory and instrumental (including liver biopsy in 9 patients) examinations of patients was performed. Results. A direct relationship was established between the level of resistin and homocysteine and indicators of enzyme and pigment exchange in groups of patients with combined pathology. A significant increase of resistin and homocysteine plasma level, as well as disorders of the liver function were determined in all groups of patients in comparison with the controls. The most marked changes were revealed in patients with combination of NAFLD, DM-2 and obesity. Conclusions. The established relationship between the level of resistin, homocysteine and the indicators of the liver functional state suggests that an increase of resistin and homocysteine level may reflect the presence of impaired liver function in patients with NAFLD in combination with DM-2, predicting the progression of NAFLD. In order to detect the disorders of liver function in patients with DM-2, it is recommended to determine the level of resistin and homocysteine in patients with NAFLD, especially when concomitant obesity is present. Patients with resistin level (>8.06±0.23 ng/ml) and homocysteine level (>25.5±0.2 μmol/l) should be considered at risk of NAFLD progression.
16-22 290
Abstract
Nonalcoholic fatty liver disease (NAFLD) is a common cause of chronic liver disease worldwide, especially in patients with type 2 diabetes mellitus and obesity. Thirty-four patients with NAFLD were enrolled and administered empagliflozin 10 mg or dapagliflozin 10 mg once a day. Body weight and serum levels of aspartate aminotransferase (AST), alanine aminotransferase (ALT), triglycerides (TG), glycated haemoglobin (HbA1C) decreased significantly after 24 weeks of empagliflozin and dapagliflozin treatment. The ALT level decreased from 41.6±8.6 to 26.8±8.3 u/l (p=0.002), the AST level decreased from 35.3±6.8 to 21.9±7.1 U/l (p=0.0029). The risk of fibrosis was mild and reached statistical significance only when assessed with FIB-4 index, when comparing the initial values and after 24 weeks of treatment - 1.35±0.48 and 1.15±0.35, respectively (p=0.025). This fact can be explained by the minimal degree of fibrosis at the baseline. Conclusion. There were comparable effects of empagliflozin and dapagliflozin on estimated parameters at the end of the study. Further studies on big cohorts of patients with different stages of NAFLD are required to unambiguously answer the question about the effect of iSGLT2 on the progress of this disease.
23-28 272
Abstract
The goal was to determine the role of inflammation and insulin resistance in early forms of nonalcoholic fatty liver diseases (NAFLD) - hepatic steatosis (HS) and steatohepatitis of mild activity (SHMA). Materials and methods. 220 patients with NAFLD were examined: 70 (31.8%) with HS and 150 (68.2%) with SHMA. The diagnosis was made according to the Russian Gastroenterological Association and Scientific Society of Gastroenterologists of Russia recommendations. Blood levels of tumor necrotic factor alpha (TNF-α) (ELISA, Human TNFα Platinum ELISA, eBioscience, Austria), interleukin-6 (IL-6) (Interleukin-6 - IFA-Best, Russia), insulin (Insulin TEST System, USA), cytokeratin-18 (CK-18) (ELISA, TPS ELISA, Biotech, Sweden), HOMA-IR (glycemia x insulin/22.5) and NAFLD fibrosis score (NAFLD FS) were evaluated. The control group consisted of 43 healthy donors. Statistical data processing was performed using «Statgraph 2.1» with estimation of Mann-Whitney and Spearman methods. Results. The frequency of the increase of TNF-α in blood was 74.3% in HS, 84.0% in SHMA, 47.1% and 50.7% for IL-6, and 40.0% and 54.0% for HOMA-IR, respectively. The content of TNF-α in HS was 5.9±1.7 pg/ml, in SHMA - 6.4±2.0 pg/ml, in healthy individuals - 4.3±1.3 pg/ml (p<0.05); as well as the content of IL-6 in HS - 5.4±2.8 pg/ml, in SHMA - 11.2±7.1 pg/ml, in healthy individuals - 1.0±0.4 pg/ml (p<0.05). The level of HOMA-IR in HS was 4.7±2.6, in SHMA - 10.5±2.3, in healthy individuals - 1.1±0.6 (p<0.05). TNF-α was correlated with HOMA-IR (r=0.64, p=0.003), CK-18 (r=0.66, p=0.008), leukocytes (r=0.59, p=0.002), NAFLD FS (r=0.34, p=0.04), HDL (r=-0.42, p=0.04) in HS; with HOMA-IR (r=0.67, p=0.004), NAFLD FS (r=0.60, p=0.016) and ALAT (r=0.23, p=0.04) in SHMA. IL-6 was correlated with ESR (r=0.51, p=0.04), leukocytes (r=0.49, p=0.04), triglycerides (r=0.73, p=0.016) and HDL (r=-0.52, p=0.04) in HS; with NAFLD FS (r=0.70, p=0.02) in SHMA. Conclusion. In the early forms of NAFLD - hepatic steatosis and steatohepatitis of mild activity - an increased levels of pro-inflammatory cytokines - TNF-α, IL-6 and HOMA-IR were observed. Both cytokines correlated with conventional markers of inflammation, dyslipidemia, fibrosis and TNF-α - with HOMA-IR and hepatocyte damage markers. These facts confirmed the role of low-level inflammation and insulin resistance in the progression of early forms of NAFLD.
29-35 756
Abstract
Background/Aims. Our study retrospectively evaluated the predictors of spontaneous viral clearance and treatment-induced viral clearance of acute viral hepatitis C. Methods. The study included the data of 286 patients (156 male aged 18-79 years and 130 female aged 18-81 years) with acute hepatitis C in Latvia over a 5 year period (from 2008 till 2012). We performed retrospective analysis of the epidemiology, mode of transmission, clinical course, changes of laboratory tests (alanine aminotransferase, aspartate aminotransferase, total bilirubin, INR), changes of ultrasonography in an attempt to identify factors that could predict the outcome of the disease. Results. Among the 286 patients studied, transmission associated with healthcare-related exposure was the primary risk factor in 45% of patients, intravenous drug use in 11%, tattooing, body piercing, manicures in 7%, sexual transmission in 6%, needle stick injuries in 3% and source of infection undetermined in 28% of patients. In our study 92% of patients noted complaints, 79% of patients presenting with jaundice. Hepatomegaly in ultrasound was reported in 13% of cases and intra-abdominal lymphadenopathy was reported in 38% of cases. The spontaneous clearance of acute hepatitis was observed in 41% patients. We did not establish any significant correlation between spontaneous viral clearance and patient age, gender, clinical features or changes of laboratory tests. But we observed that patients with intra-abdominal lymphadenopathy in ultrasonography recovered more often compared to patients without intra-abdominal lymphadenopathy (r=0,219, p=0,016). Treatment with interferon alpha 2b was generally initiated by 228 patients. 86% of patients had a SVR after a 6 months post-treatment follow-up, 4% relapsed. We found that treatment with interferon alpha 2b was more effective in women compared to men (r=0.170, p=0.047). We did not did not determine any significant correlation between efficacy of antiviral therapy and clinical features and changes of laboratory tests. Conclusion. Hepatitis C virus (HCV) infection spontaneously clears in 41% of patients. We cannot predict spontaneous viral clearance by patient age, gender, clinical features or changes of laboratory tests, but patients with intra-abdominal lymphadenopathy in ultrasonography recovered more often compared to patients without intra-abdominal lymphadenopathy. None of the parameters accurately predicts spontaneous resolution at the individual level.
36-40 236
Abstract
The aim was to assess the functional state of the liver and biliary tract in children with diabetes mellitus type I and their correlation with the duration of the disease and the severity of metabolic changes. Materials and methods. There were examined 74 patients with diabetes mellitus type I aged from 7 to 17 years. There were formed 2 groups: 1 group - children with a disease duration up to 5 years (33 people), 2 group - with a disease duration of more than 5 years (41 people). There were studied the functional state of the hepato-biliary system on the basis of clinical, laboratory and instrumental parameters. Results. With an increase in the duration of the disease, increased the number of patients with a change in the echogenicity and homogeneity of the liver parenchyma: an increase in liver echogenicity was detected in group 1 in 24.2% of cases, in group 2 - in 36.6% (p<0.05). The fatty liver index characteristic of non-alcoholic fatty liver disease were more often detected when the disease lasted more than 5 years - 17.1% versus 6.1% (p<0.05). Biochemical parameters of patients remained within the normal range with the exception of alkaline phosphatase (AP) and gamma-glutamyl transpeptidase (GGTP), significantly increasing with a duration of diabetes more than 5 years (433.91±205.01 and 411.05±220.32 U/l; 16.22±7.07 and 9.74±3.56 U/l).
41-47 516
Abstract
The aim - to determine the etiology and clinical features of the acute-on-chronic liver failure (ACLF) in patients with acute decompensation of liver cirrhosis (LC). Materials and methods. A retrospective analysis of the clinical features of ACLF was performed in 71 patients with LC, who died within 28 days of hospital stay. The etiology of the LC was determined, traditional clinical, laboratory and instrumental data were performed, the chronic liver failure organ failure score (CLIF OF S), chronic liver failure-consortium acute-on-chronic liver failure score (CLIF-C ACLF S), model for end stage liver disease score (MELD S) and Child-Pugh score (Ch-P S) were calculated. Results. Alcoholic genesis of LC was detected in 63.4% of patients, alcoholic in combination with metabolic (NAFLD) - in 15.5%, alcoholic in combination with viral - in 4,2%, metabolic - in 1,4%, viral - in 4, 2%, autoimmune - in 1,4%, drug - in 1,4%, unidentifiable - in 8.5%. Triggers for the development of ACLF were active alcoholism in 39.4% of patients, bacterial infection in 19.7%, esophageal bleeding in 15.5%, active HCV infection in 4.2%, autoimmune attack in 1,4%. ACLF grade 1 was revealed in 26.8%, ACLF grade 2 - in 19.7%, ACLF grade 3 - in 53.5% of patients. The frequency of organ failures were: liver - 73.2%, kidney - 54.9%, coagulation - 54.9%, cerebral - 21.6%, circulation - 18.3%, lungs - 11.3%. CLIF OF S, CLIF-C ACLF S significantly increased already at ACLF grade 2, and MELD S and Ch-P S - only at ACLF grade 3. Conclusion. The alcoholic genesis of LC was revealed in the most patients (63.4%) and active alcoholism was the trigger for ACLF development (39.4%). The most frequent clinical manifestations of ACLF were liver failure (73.2%), kidney (54.9%) and coagulation (54.9%). CLIF OF S, CLIF-C-ACLF S had better diagnostic and prognostic significance than MELD S and Ch-P S.
48-52 277
Abstract
The purpose of the study was to determine the clinical and diagnostic role of hepatocyte apoptosis in various forms of alcoholic liver disease (ALD). Materials and methods. 98 patients with ALD were examined: 11 (11.2%) with liver steatosis (LS), 17 (17.3%) with seatohepatitis (SH), 70 (71.4%) with liver cirrhosis (LC). Among patients with LC, 13 (13.2%) had class A, 24 (24.5%) - class B, and 33 (47.1%) had class C on the Child-Pugh scale. The diagnosis of ALD was established on the basis of generally accepted criteria: anamnestic data, clinical, laboratory, instrumental (abdominal sonography, Doppler sonography of the hepatic blood flow, esophagogastroduodenoscopy). The level of fragments of cytoceratin-18 (CK-18) was determined by ELISA (test systems "TPS ELISA", "Biotech", Sweden). Statistical data processing was performed using the «StatGraphics» 2.1 software, the Mann-Whitney and Spearman methods. Results. The level of CK-18 in all forms of ALD exceeded that in the control group (60.1±18.2 U/l) and significantly differed between different forms of ALD: in SP - 98,2±17.8 U/l (p<0.05), in SH - 335.9±38.6 U/l (p<0.05), in LC - 1236.5±80.1 U/l (p<0.05). Traditional markers of hepatocellular death - aminotransferases - were: in LS - ALAT - 28.5±9.2 U/l, ACAT - 25.2±8.7 U/l; in SH - ALAT - 93,4±44,1 U/l (p<0.05), ACAT - 320.9±11.2 U/l (p<0.05); in LC - ALAT - 75.5±24.6 U/l (p>0.05), ACAT - 151.5±39.1 U/l (p>0.05). The level of CK-18 in ALD negatively correlated with indicators of synthetic liver function: with albumin (r=-0.42, p<0.05), with prothrombin (r=-0.48, p<0.05), cholesterol (r=-0.41, p<0.05) and positively with the level of bilirubin (r=0.54, p<0.05). The level of CK-18 increased according to the degree of severity of the LC: in A class of LC - 885.2±71.6 U/l, in class B - 1401.1±50.3 U/l (p<0.05), in class C - 1443.9±73.2 U/l. The content of CK-18 depended on the level of the MELD scale: in MELD<20, it was 690.7±19.8 U/l, in MELD>20-1318.8±82.3 (p<0.05). Conclusion. In all forms of alcoholic liver disease, apoptosis of hepatocytes prevailed over necrosis, reaching a maximum level in case of liver cirrhosis, correlating with the main functional liver tests and the severity of ABP.
53-60 224
Abstract
Actuality. Everolimus is an oral drug from the group of immunodepressives, a derivative of macrocyclic lactone, the mechanism of action of which is to inhibit serine-threonine kinase (mTOR), which is the Central mechanism in the proliferation of T cells. The drug is used in a complex scheme of immunosuppressive therapy to prevent rejection of kidney and heart transplant. Everolimus is characterized by a narrow therapeutic interval and demonstrates clinically significant variability of pharmacokinetics due to the peculiarities of resorption, biotransformation, elimination, and inter-drug interactions. Liver pathology occupies an important place in the structure of factors influencing the achievement of target concentrations of everolimus. Such lability of the unfavorable pharmacokinetics of the influence of et on the achievement of target concentrations of the drug in the blood, reducing the effectiveness of the therapy or increase the risks of side effects. Thus, patients taking everolimus need regular laboratory control of the drug concentration. Special control require patients with concomitant pathology of the liver. The aim of the study was to consider the factors affecting the achievement of target concentrations of everolimus with a closer study of the contribution of liver disease to the effectiveness and safety of therapy, as well as the possibility of using laboratory methods for the optimization of therapy. Material and methods of research. To study the factors influencing the achievement of the target concentrations of everolimus in the blood, we studied the current literature data and the results of the study of everolimus pharmacokinetics in patients after heart transplantation on the background of liver disease. To evaluate the contribution of pathology of the liver in the efficacy and safety of therapy, as well as the possibility of using laboratory techniques to optimize the therapy were conducted the original prospective research in the group of patients (n=16) who underwent the transplantation of the heart in the i "national medical research center named after V. A. Almazov" Mr 2012-2016, as well as the host merely-Mus at a dose of 1.25 mg/day to about 5.4 mg/day (mean dose 2.5 mg/day). Patients were observed in the period from 2016-2017. Patients were the distribution of HN into two groups in accordance with the presence and absence of the signs of Neal-Cogolin fatty liver disease (NAFLD). To control the concentrations of everolimus in whole blood, a method of high-performance liquid-bone chromatography with mass-spetromtric detection was used, for the use of which an original analytical technique for the quantitative determination of everolimus in whole blood was developed in the laboratory of therapeutic drug monitoring of the national medical research center of the Ministry of health of the Russian Federation. Results. According to the literature data, the achievement of the target concentrations of everolimus is influenced by the activity of the efflux vector of p-glycoprotein drugs, the rate of metabolic transformations of the drug mediated by cytochromes CYP3A4, 3A5, 2C8, inter-drug interactions, consumption of fatty foods, as well as non-compliance with the prescribed treatment regimen. Non-alcoholic fatty liver disease is one of the leading factors adversely affecting achievement of target everolimus concentrations which significantly increases the risk of toxic effects. For laboratory monitoring in the laboratory of therapeutic drug monitoring of the "national medical research center" (Russia) was developed an original analytical technique for the quantitative determination of everolimus in human whole blood, successfully validated. Conclusion. Everolimus is a powerful immunosuppressant, which is actively and successfully used in patients as part of immunosuppressive therapy after transplantation of solid organs. However, it has a high risk of side effects or reduced efficiency, because it has a narrow therapeutic interval and pronounced variability of pharmacokinetics. One of the leading factors influencing the maintenance of target concentrations is nonalcoholic fatty liver disease. To reduce the risk of side effects and ensure the proper effectiveness of therapy with everolimus, monitoring of drug concentrations and assessment of liver function should be carried out.
61-66 227
Abstract
Objective: to assess the effectiveness of anesthetic maintenance of orthotopic liver transplants in pediatric recipients under conditions of epidural analgesia as a component of general anesthesia. Materials and methods: Twelve children aged between four months and fourteen years who had been involved in the observational research had an orthotopic liver transplant. Anesthetic management included inhalation anesthesia with sevoflurane and thoracic epidural analgesia. Results: hyperdynamia was the most significant, the level of which was closely correlated with the volume of intraoperative blood loss. High level of lactate was not associated with severe acidosis and need not to be corrected. Epidural analgesia did not affect hemodynamic parameters, that allowed to use minimal doses of opioid analgesic, and the safety of anesthesia was undoubtedly increased. The pace and volume of infusion-transfusion support was dictated by the rate and volume of blood loss, while it was important to maintain a rate that was higher than the rate of blood loss. Conclusion: epidural analgesia when orthotopic liver transplants are performed does not create an additional risk for child recipients, including infants, and improves the quality of pain control. The possibility of early extubation of children after orthotopic liver transplantation is determined by the volume of intraoperative infusion. Therefore, it is necessary to improve the methods of calculating the rate and volume of infusion.
67-71 312
Abstract
The aim to study the structure and characteristics of clinical and laboratory manifestations of acute drug poisoning (DP) among adolescent children. Materials and methods: 177 children were admitted to the Department of Toxicology N. F. Filatov Children’s City Clinical Hospital № 13 in the period from 2016 to 2018 with acute poisoning of drug poisoning. The diagnosis was confirmed by chemical-toxicological examination of urine by thin layer chromatography. The features of clinical manifestations and laboratory studies in patients with acute drug poisoning have been studied. Results: From 2016 to 2018, the number of acute DP among adolescents in Moscow increased 7 times, with a predominance of males (71%). Plant or synthetic cannabinoids, as well as lysergic acid derivatives (LSD), occupy a leading position in the choice of DP. In 39% of patients reported severe severity of poisoning. All patients had damage to the central nervous system (CNS) in the form of depression or arousal syndrome, the majority - disorders of the cardiovascular system, respiratory organs, liver and kidneys. As a result of acute toxic damage to the DP in patients already after 24 hours from the onset of the disease, changes in laboratory parameters in the form of cytolysis syndrome, hypocholesterolemia, lactic acidosis were noted.

EXPERIMENTAL GASTROENTEROLOGY

72-76 351
Abstract
Bariatric surgery is widely used for weight regression in patients with obesity and metabolic syndrome, and improves course of non-alcoholic fatty liver disease (NAFLD) in obese patients. However, there are very little researches devoted to the evaluation of morphological liver’s changing after bariatric surgeries on decompensated T2DM without severe obesity. The aim of the study was to evaluate the effect of various types of bariatric operations on the morphological changes in the liver of rats with severe, decompensated form of diabetes. The study shows that the implementation of bariatric surgery of sleeve gastrectomy contributes to the regression of liver steatosis. The obtained data can be the basis for optimizing approaches when using bariatric operations in the treatment of patients with NAFLD.

CLINICAL PHARMACOLOGY

77-81 2537
Abstract
The purpose of the study is to develop and introduce into clinical practice an algorithm for managing perimenopausal patients suffering from menopausal metabolic syndrome (MMS) and having hepatobiliary system disorders (non-alcoholic fatty liver disease (NAFLD), cholestasis, biliary sludge), to identify the effectiveness of menopausal hormone therapy (Femoston) in combination with ursodeoxycholic acid. 60 women with the specified syndromes aged 45 to 60 years were included in the study. Prospective clinical and laboratory examinations were performed, a modified menopause index (MMI) was measured before treatment and after 6 months. The levels of follicle-stimulating hormone (FSH) and estradiol have changed significantly. Beneficial changes in the lipid spectrum and a statistically significant decrease in transaminases and bilirubin levels were identified. The combined use of low-dose menopausal hormone therapy and udhc leads to a pronounced positive dynamics in the lipid spectrum by reducing total serum cholesterol (OHS) from 5.92±0.45 mmol/l to 4.90±0.46 mmol/l, triglycerides from 2.01±0.87 to 1.07±0.65 mmol/l, increasing HDL from 1.41±0.25 to 1.81±0.31 mmol/l, reducing LDL from 3.48±0.72 up to 2.70±0.53 mmol/l and VLDL from 1.26±0.58 mmol/l to 0.55±0.34 mmol/l); It was found that the combined use of low - dose MGT and udhc effectively reduces the activity of enzymes: ALT - from 27.76±9.1 to 17.35±7.6 units⁄l; AST - from 22.56±4.7 units⁄l to 17.45±6.64 units⁄l, schf-from 146.26±63.77 units⁄l to 90.37±29.1 units⁄l. the level of bilirubin and its fractions is Normalized: the total bilirubin in the main group decreased from 11.45±6.34 to 7.32±4.56 mmol/l. The coagulogram has not changed. Femoston and ursodeoxycholic acid improved the climacteric syndrome, normalized the lipid profile, and had a beneficial effect on the hepatobiliary system. Thе combination therapy improved liver function, improved the quality of life of patients, and reduced the risk of NAFLD progression.

REVIEW

82-89 554
Abstract
Prurit (itchy skin) is a common symptom accompanying chronic cholestasis. The pathogenesis of pruritus in cholestatic diseases is not well understood. It may include a specific neural pathway regulated by substances that can cause skin itching, such as bile acids, opioids, serotonin and lysophosphatidic acid. In exceptional cases, itch refractory to therapy may lead to the need for invasive therapy, including liver transplantation. While the therapeutic management of cholestatic pruritus is well known in adults, children do not agree on the difficulty of conducting controlled clinical trials. The strategy to combat cholestatic pruritus in children is currently based on several areas of specific therapy, which should be associated with skin care and non-specific treatment of cholestasis, including ursodeoxycholic acid. Prurit should be evaluated during treatment as objectively as possible. The first line of treatment for cholestatic pruritus is rifampicin. Second-line therapy is discussed in each case. In the most severe cases, invasive treatments may be suggested. The aim of this work was to review the pathophysiological mechanisms associated with cholestatic pruritus in children and suggest potential therapeutic methods.

DISCUSSION

90-94 256
Abstract
The aim: Improving the diagnosis of non-alcoholic fatty liver disease in children with obesity. Materials and methods: 40 children aged from 8 to 17 years with exogenous constitutional obesity (BMI from 22.84 to 45.00) were examined. Evaluated clinical and anamnestic data, biochemical blood tests, incl. ALT and AST, ultrasound examination of the hepatobiliary system, FibroMax test, polypathography (PGG). Viral hepatitis B and C are excluded in all children. Compared 2 groups of children. The first group included 16 children diagnosed with NAFLD according to the FibroMax test. The second control group consisted of 24 children without NAFLD. The comparison was carried out on all the signs stipulated by the study protocol. Wе used the contingency table analysis method and discriminant analysis. Statistical analysis of the results was performed using software and the Statistica 10.0 for Windows package. Results: A new method for the diagnosis of NAFLD in obese children has been developed. The method uses the following available parameters: BMI, duration of obesity, ultrasound data (increased liver echogenicity and depletion of the vascular pattern), polyhepathography data (organic changes on the SGBP and impaired hemodynamics due to venous influx), followed by calculating the discriminant function using the calculated formula. The percentage of correct diagnosis when using this method is 97.4%, sensitivity = 0.9545 (95.45%), specificity = 1.00 (100%).

CLINICAL CASES

95-100 243
Abstract
Lysomal acid lipase deficiency is a rare inherited metabolic disorder and is characterized by the accumulation of cholesteryl esters and triglycerides in many tissues due to a deficiency of lysosomal acid lipase. We report a family case of the disease in a girl aged 5 years and 2 months and her younger brother 3 years. The disease was accompanied by moderate hepatomegaly from birth, serum aminotransferase increased later, and dyslipidemia was observed. An elder child underwent a liver biopsy. Morphological examination of the liver showed microvesicular steatosis with a distinct tendency to the formation of micronodular cirrhosis and cholesterol crystals in hepatocytes. The diagnosis was confirmed by a decrease in the activity of acid lipase in leukocytes and by mutational analysis of the LIPA gene.
101-104 427
Abstract
A case of abdominal tuberculosis, an isolated lesion of liver tuberculosis, is given. The diagnosis of this form of tuberculosis presented certain difficulties, the histological examination of the surgical material after atypical resection of the liver was of decisive importance. Clinical observation is of interest in view of the patient having a long period of low-grade temperature reaction with the absence of any changes according to the routine methods of laboratory diagnostics, as well as the difficulties of differential diagnosis, with the participation of doctors of various specialties.
105-109 328
Abstract
The article describes the case of diffuse B-cell lymphoma, which occurred under the guise of a chronic liver disease. In the presented clinical case, the disease debuted with hepatosplenomegaly syndrome with minimal cytolysis. Subsequently, against the background of lymphoproliferative disease, an infectious process (pneumonia) developed, which masked B-symptoms and made it difficult to verify the diagnosis. As a result, the tumor diagnosis was established on the basis of morphological examination of the surgical material (spleen) using histological and immunohistochemical methods.


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