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The objective: to compare liver function of respiratory tuberculosis patients during the intensive phase of treatment with first line drugs (chemotherapy regimen 1) and reserve drugs (chemotherapy regimens 4 and 5).Subjects and Methods: Data of 144 respiratory tuberculosis patients were analyzed, those patients were divided into 2 groups. Group 1 included 67 patients receiving chemotherapy regimen 1 and Group 2 included 77 patients treated with chemotherapy regimens 4 or 5. During the first 3 months of treatment, the frequency and severity of changes in the liver function were assessed.Conclusion: in patients with chronic viral hepatitis, parenchymal liver damage is more frequent and it improves slower. Transaminase levels returned to normal levels more frequently and rapidly with chemotherapy regimen 1, whereas with chemotherapy regimens 4 and 5, reduction of alanine aminotransferase level was observed only from the 2nd month of treatment.

The growth hormone/insulin-like growth factor-1 (IGF-1) system is an important humoral mediator of liver function with an inherent potential for assessing liver condition and survival prognosis following liver transplantation (LT). Aim . Analysis of associations between growth hormone and IGF-1 levels in blood and markers of liver function and post-LT survival in children. Materials and methods . We examined 143 children with end-stage liver disease (ESLD) aged 3 to 73 (median 8) months after related LT. The growth hormone and IGF-1 concentrations in plasma were evaluated in ELISA assays. Results . In children with ESLD, concentrations of growth hormone and IGF-1 were significantly higher and lower, respectively, compared to healthy children of the same age ( p

Introduction. Today, non-alcoholic fatty liver disease (NAFLD) is increasingly associated with the presence or risk of developing type 2 diabetes (D2). The term “hepatogenic diabetes”, proposed to refer to SD2 in patients with cirrhosis of the liver (CP), has acquired a new meaning, since this combination is of growing interest. The authors analyzed the current literature and summarized data on the pathogenesis, risk factors, and possible therapy of NAFLD.Objective: to evaluate the effectiveness of L-ornithine-L-aspartate (HEPA-Merz, Merz Pharma GmbH & Co) in combination with biguanides in the treatment of NAFLD patients in combination with D2.Materials and methods. The study included 30 patients aged 26 to 60 years with a verified diagnosis of NAFLD of varying degrees of activity in combination with D2. All patients were prescribed combination therapy with the drug HEPA-Merz (“Merz Pharma GmbH and Co”) at a dose of 3 grams 3 times a day in combination with biguanides. The examination was performed on the 1st, 28-th and 56-th days of treatment. In order to determine the effectiveness of therapy, we evaluated the dynamics of clinical symptoms (asthenovegetative, dyspeptic syndromes, pain syndrome in the ball system), biochemical parameters of liver function (changes in markers of cytolysis, cholestasis), lipidogram indicators, glucose levels, glycated hemoglobin, and ultrasound results of abdominal organs.Results. On day 56, asthenovegetative, dyspeptic and pain syndrome were stopped during therapy. Most patients showed a decrease in body weight from 3 to 5 kg. When evaluating changes in biochemical parameters on the 28-th day, the activity of ALT, AST, GGTP and glucose levels significantly decreased against the background of the therapy. On the 56th day of treatment, the activity of transaminases, bilirubin, GGTP and GFR in all patients were within the reference values.Conclusion. Understanding the multifactorial nature of NAFLD and the mechanisms of associated diseases, including D2, will allow us to assess the prognosis of the disease and prescribe adequate timely therapy. The effectiveness of the original ornithineaspartate (Merz Pharma GmbH & Co.) is manifested by a decrease in the processes of cytolysis of hepatocytes, normalization of lipid and carbohydrate metabolism.

Aim. To evaluate effects of ursodeoxycholic acid (UDCA) on inflammation, steatosis and liver fibrosis and atherogenesis factors in patients with non-alcoholic fatty liver disease (NAFLD). Materials and methods. The study included 139 patients with NAFLD who received 15 mg/kg of UDCA (Ursosan ® ) per day for 24 weeks. Before and after the study, liver function and lipid metabolism, liver steatosis index FLI, intimamedia thickness (IMT) of the carotid arteries were determined. The cardiovascular risk was calculated according to the ASCVD 2013. Results. The use of UDCA in patients with NAFLD led to a decrease in ALT ( p < 0.001), AST ( p < 0.001) and GGT ( p < 0.001) activity; total cholesterol concentration ( p < 0.001); triglycerides ( p < 0.001); and LDL ( p < 0.001) in the blood and the FLI liver steatosis index ( p < 0.001). In addition, the rate of cardiovascular risk (p = 0.022) and IMT (р = 0.048) in women also decreased. Conclusions . The use of UDCA in NAFLD reduces the activity of inflammatory processes in the liver, its steatosis, and also improves lipid metabolism and exerts potential anti-atherogenic effects. Against the background of UDCA administration, there was no progression of liver fibrosis. None of the participants in the observational programme reached a normal body mass by the end of the program; therefore, the attained positive changes should be attributed directly to the effects of UDCA.

Aim. To study the effect of intermittent normobaric hypoxia (INH) during local irradiation of the rat liver in therapeutic doses (total focal dose 30 Gy for 10 fractions) on the biochemical parameters of blood serum characterizing liver function. Materials and methods. Blood sampling in the animals of the control group and in three tested groups was performed prior to the experiment and following the experiment (after 20 days), respectively. The following criteria of hepatocyte toxicity were investigated: the level of the general and direct bilirubin, general cholesterol and triglycerides, high- and low-density lipoproteins, alanine aminotransferase and aspartate aminotransferase activity. Blood tests were performed using a Sapphire-400 (Tokyo Boeki ltd, Japan) automatic clinical analyzer with the standard Cormay (Poland) sets of reactants. Results. The experiment showed that the local radiation therapy on the liver region led to a growth in the indicators of pigment exchange, total cholesterol, low-density lipoproteins, triglycerides zdfl and transaminase activity. At the same time, the high-density lipoprotein level decreased. In the tested animals, INH application caused an increase in the bilirubin level and a change in lipid exchange indicators of the opposite direction in comparison with the animals receiving radiation therapy. INH did not change the transaminase activity. Liver radiation against the INH background led to less pronounced changes in blood biochemical indicators than in the group of animals having received exclusively radiation therapy. Such changes in blood indicators can be explained by a radioprotective effect of intermittent normobaric hypoxia. Conclusion. Intermittent normobaric hypoxia has a normalizing effect on the blood biochemical indices when performing local radiation therapy on the liver region.

Blood level of transforming growth factor beta 1 (TGF-β1) is associated with liver function and immune homeostasis, which suggests it as a potential biomarker for immunosuppressant tacrolimus dose requirement at liver transplantation (LT).Aim.To evaluate diagnostic efficacy of TGF-β1 blood level at determination of individual tacrolimus dose requirement in children at LT.Materials and methods.89 children with end stage liver disease aged from 3 to 73 months were examined. Children underwent living related LT, then the recipients received 2–3 component immunosuppressive therapy, including tacrolimus. Blood concentration of tacrolimus and TGF-β1 was measured by ELISA.Results.TGF-β1 blood level in children before LT was significantly lower than in healthy children: 3.7 (1.3–8.4) and 19.3 (12.6–25.5) ng/ml, p = 0.001. A month after LT, its concentration increased to 8.1 (1.8–15.3) ng/ml (p = 0.02). A year after LT, the cytokine level remained higher than before transplantation: 6.6 (1.9–12.6) ng/ml, p = 0.01. TGF-β1 level did not correlate with tacrolimus blood concentration, determined 12 hours after the last administration of the drug, neither a month, nor a year after transplantation. At the same time, the cytokine level one month after LT was associated with a tacrolimus daily dose one year after the operation (r = –0.23, p = 0.04). In the recipients, who received smaller daily doses (0.4–2.5 mg) of tacrolimus, TGF-β1 level was higher than in those receiving large doses (3.0–6.0 mg) of the drug: 9.1 (2.6–16.2) ng/ml vs. 4.2 (1.3–9.2) ng/ ml, p = 0.04. Evaluation of diagnostic efficacy of the TGF-β1 level as a test for the detection of tacrolimus dose requirement showed that the area under the ROC curve (AUC) was 0.66 ± 0.07; 95% CI [0.53–0.79], the sensitivity and specificity of the test were 60 and 74% at threshold value 6.7 ng/ml. Relative risk of higher tacrolimus dose requirement was 3.14 ± 0.48; 95% CI [1.24–7.96].Conclusion.TGF-β1 blood level in one month after LT less than 6.7 ng/ml is 3 times higher risk factor of tacrolimus dose requirement more than 3.0 mg per day. The likehood of the test is 66%, the sensitivity and specificity – 60 and 74%.

В ранее проведенных нами опытах установлено, что среди исследованных нами производных 3-оксипиридина и пиридина наибольшей актопротекторной активностью в обычных условиях и после воздействия гипокинезии обладает фенилэтилпроизводное 3-оксипиридина под шифром СК-132. По влиянию на продолжительность физической работоспособности экспериментальных животных после воздействия гипокинезии соединение превосходит другие исследованные соединения, а также известные актопротекторы бемитил и бромантан. Данное соединение представляет интерес для дальнейшего экспериментального исследования в качестве потенциального актопротектора, включая изучение возможного актопротекторного механизма действия., In our previous experiments, it was found that among the studied derivatives of 3-oxypyridine and pyridine, the phenylethyl derivative of 3-oxypyridine with the designation SK-132 has the greatest actoprotective activity under normal conditions and after exposure to hypokinesia. According to the effect on the duration of physical performance of experimental animals after exposure to hypokinesia, the compound is superior to other studied compounds, including the well-known actoprotectors bemitil and bromantane. This compound is of interest for further experimental research as a potential actoprotector, including the study of a possible actoprotective mechanism of action., Международный научно-исследовательский журнал, Выпуск 5 (107) 2021, Pages 119-121

Aim of the case presentation. To demonstrate the rare case of extranodal lymphoma with primary extensive liver involvement which caused difficulties at diagnosis verification. Despite of rapid disease progression with development of severe liver failure, that resulted in poor prognosis, patient demonstrated significant response to high-dose chemotherapy and achieved long-term remission. Summary. Male 38-year-old patient developed acute symptoms: sweating, general weakness, febrile fever, weight loss (50 kg for 4 months), abdominal pain combined to hepatomegaly and multiple focal lesions in the liver according to abdominal US, with 5 unsuccessful attempts for 6 months to receive informative tissue sample. Laboratory tests revealed progressive signs of liver failure at normal level of alpha-fetoprotein and carcino-embryonic antigen; clinical signs included progressive manifestations of liver encephalopathy, skin hemorrhages, severe edema, ascites, pruritis and excoriations. Empirical therapy by prednisolon resulted in no responce and was complicated by development of steroid diabetes. The diagnosis of diffuse B-macrocellular lymphoma was established only via open liver biopsy. Four sessions of high-dose polychemotherapy according to mNHL-BFM-90 program resulted in rapid reduction of liver size, recovery of consciousness, resolution of encephalopathy signs, however the liver and the spleen remained enlarged with focal parenchimatous lesions that required laparoscopy with biopsy of the pathological foci, the latter revealed no remaining neoplastic cells. During 10 years of patient follow-up liver function remains compensated, focal lesions in the liver and the spleen are undetectable, that indicates achievement of 113-months complete tumor remission. Conclusion. Presence of multiple focal lesions in the liver and signs of progressive liver failure at normal level of tumor markers requires taking into account primary liver lymphoma in the spectrum of possible etiologies. Potentially poor life prognosis in similar clinical situations at detection of primary liver lymphoma can flip to favorable due to high efficacy of modern chemotherapeutic agents.

Aim of review. To present modern options of comprehensive infusion therapy at various liver diseases, indications and contraindications for application of basic blood substitute groups, and pathogenically justified approaches for their use. Summary. Chronic and acute liver diseases are associated to end the genius intoxication, electrolyte disorders, shift of acid-base state, tissue and cellular hypoxia. Infusion therapy with application of modern plasma substitute solutions provide increase in treatment efficacy for this group of patients. Application of basic group infusion solutions along with complex anti-hypoxia agents helps to correct water and electrolyte imbalance as well as disorders of acid-base state, carry out body detoxication, normalize blood pressure, treat edema and ascites syndrome, protein-energy malnutrition, prevent renal value and other complications in liver cirrhosis patients. Comprehensive treatment of liver diseases includes application of modern infusion agents (anti-hypoxia/anti-oxidation) containing pharmacologically active metabolic substances that include succinate-containing drugs developed on the basis of succinic acid and its salts. Succinic acid is presumed to act as the paracrine agent produced by damage hepatocytes (e.g. at ischemia), affecting pericytes (Ito's cell) in the liver through SUCNR1receptors. It results activation of pericytes providing synthesis of extracellular matrix components that are involved in metabolism and regeneration of liver parenchyma cells. Conclusion. Addition of succinic acid-based medications to infusion therapy program for chronic liver diseases provides alleviation of basic clinical syndromes (intoxication, asthenic and autonomous nervous disorders, dyspeptic, cholestatic), and at patients with hepatocellular failure improvement of protein-synthetic liver function.

A gold standard for the treatment of metastases of colorectal cancer in the liver is combined treatment with postoperative or perioperative chemotherapy. Despite the improvement of antitumor drugs, the main condition for the successful treatment of patients with metastatic colorectal cancer is the operation in a radical volume, implying a macroscopic and microscopic absence of a residual tumor. The main contraindications to anatomic resections of the liver include a marked decrease in liver function, insufficient volume of the remaining parenchyma of the liver, and for a long time, bilobar liver damage was considered, which excludes the possibility of simultaneous removal of all foci. The traditional variant of the surgical solution of the problem of bilobar metastatic liver injury is the implementation of two-stage anatomical resections. It has been proved that the reduction of liver and FLR functional reserves of less than 20% at normal and 40% in the case of compromised liver parenchyma, the implementation of surgical interventions is associated with a high risk of hepatic insufficiency. One of the promising ways to overcome this obstacle is to perform various modifications of ALPPS liver resections. In the present work, we present a clinical case of performing a two-stage liver resection according to the type of ALPPS in a patient with metastatic colorectal liver cancer who had previously undergone PVL with unrealized vicar hypertrophy. The described observation testifies to the justification of performing repeated liver resections in patients with metastatic colorectal cancer and demonstrates the possibilities of ALPPS technique.

Цель. Оценка роли модификаций липидов тканей печени в ее детоксикационной способности при остром перитоните. Методика. В эксперименте у собак моделировали острый перитонит различной степени тяжести. Степень эндогенной интоксикации оценивали по уровню токсических продуктов гидрофильной и гидрофобной природы. Представлен новый способ оценки детоксикационной функции печени на основе определения уровня токсических продуктов в крови, притекающей к органу и оттекающей от него. Для характеристики качественного и количественного состава липидов из биопсийного материала печени выделяли липиды, осуществляли их фракционирование и денситометрию. Оценивали интенсивность перекисного окисления липидов, активность фосфолипазы А2 и супероксиддисмутазы. Результаты. Показано, что функциональное состояние печени при хирургическом эндотоксикозе коррелирует с модификацией липидов ее тканевых структур, проявлением чего были существенные изменения состава основных липидов и резкое увеличение хаотропных фракций. Установлена сопряженность изменений состава липидов тканей печени со степенью тяжести патологического процесса. Одними из значимых триггерных механизмов липиддестабилизирующих явлений в тканевых структурах печени являются оксидативный стресс и высокая фосфолипазная активность. При тяжелом течении острого перитонита высока вероятность такого нарушения функциональнометаболического состояния печени, при котором она приобретает самостоятельное патогенетическое значение в продукции токсических субстанций. Заключение. В патогенезе синдрома эндогенной интоксикации при остром перитоните важную патофизиологическую значимость имеет модификация липидов тканей печени, которая является основой острой печеночной недостаточности и формирования нового состояния органа источника токсинов. Полученные данные определяют векторы патогенетической терапии по предупреждению необратимых повреждений печени с высоким риском неблагоприятного исхода., Background. Despite the interest to the issue of surgical endogenous intoxication, mechanisms of impaired hepatic detoxification function in endogenous intoxication induced by acute peritonitis are studied insufficiently. Aim. To elucidate the role of modifications in membrane lipids of liver cells in imparing its detoxification ability in acute peritonitis. Methods. Acute peritonitis of different severity was modeled in dogs. The authors presented a new method for evaluating the liver detoxification function based on measuring concentrations of toxic products in the blood inflowing to and outflowing from the liver. Hepatic lipids were separated, fractionated and studied by densitometry. The qualitative and quantitative composition of liver lipids, intensity of lipid peroxidation, and activities of phospholipase A2 and superoxide dismutase were measured in liver tissue in the dynamics of experimental peritonitis. Results. In surgical endogenous intoxication, the liver function correlated with the modifications of tissue lipids evident as considerable changes in the composition of major membraneforming lipids and an increase in chaotropic fractions (fatty acids, lysophospholipids). Changes in the tissue lipid composition correlated with severity of the pathological process. Significant triggering mechanisms for the hepatic lipid modification were oxidative stress and a high phospholipase activity. Conclusion. In severe acute peritonitis, there is a high probability of profound functional and metabolic disorders in the liver so that it acquires an independent pathogenetic significance as a producent of toxic substances., №2 (2019)

Objective : improve the results of treatment of patients with metastatic cancer of liver by reducing the risk of post-resection liver failure based on the assessment of liver functional reserve. Materials and methods. The study included 2 independent samples of patients underwent surgery for liver metastases in the department of abdominal oncology at the P. A. Herzen Moscow Oncological Research Institute. Group 1 included 47 patients: in addition to the standard treatment algorithm they underwent 13C methacetin breath test and dynamic scintigraphy of liver in the preoperative stage. Patients from the group 2 (n = 30) underwent standard clinical and laboratory examination, without preoperative evaluation of liver functional reserves; the level of total bilirubin, albumin and prothrombin time showed no decrease in liver function. Post-resection liver failure was established based on 50/50 criterion when evaluated on the 5th postoperative day. Results. The analysis of operational characteristics of functional tests showed absolute sensitivity of 13C methacetin breath test (SE ≥ 100 %) and negative predictive value (–VP ≥ 100 %) in case of integrated application of 2 diagnostic methods. An incidence of post-resection acute liver failure in the study group was significantly 2.2-fold lower than in the control group – 10.6 % and 23.3 %, respectively (p < 0.001). Conclusion. Combination of preoperative dynamic scintigraphy of liver with 13C methacetin breath test allows to perform comprehensive assessment of liver functional reserves, and it can greatly improve preoperative assessment and postoperative results of anatomic resections in patients with liver metastases.

Purpose of the study. Improvement of results of treatment in patients with primary and metastatic liver cancer by decreasing the risk of post-resection liver failure on the basis of the evaluation of the functional reserves of the liver. Materials and Methods. The study included two independent samples of patients operated about primary or metastatic lesions of the liver at the Department of abdominal Oncology, P. A. Hertsen MORI. The first group included 53 patients who carried out 13C-breath test metallimovie and dynamic scintigraphy of the liver in the preoperative stage in addition to the standard algorithm of examination. Patients of the 2nd group (n=35) had a standard clinical and laboratory examination, the patients were not performed the preoperative evaluation of the functional reserve of the liver, the incidences of total bilirubin, albumin and prothrombin time did not reveal a reduction of liver function. Post-resection liver failure have been established on the basis of the 50/50 criterion in the evaluation on day 5 after surgery. Results. Analysis of operating characteristics of the functional tests showed the absolute methacin breath test sensitivity (SE≥100%), high specificity (SP≥67%) of scintigraphy of the liver and the negative predictive value of outcome (VP≥100%) at complex use of two diagnostic methods. The incidence of PROPS in the study group was significantly 2 times higher in the control group –15,1% and 26.8%, respectively (p

To study the incidence, pattern, and predictive factors of concurrent kidney and liver dysfunctions in patients with decompensated heart failure (HF).The kidney and liver function indicators were estimated in 322 patients aged 69.5±10.6 years with decompensated HF (hypertension in 87%, myocardial infarction in 57%, atrial fibrillation in 65%, chronic kidney disease in 39%, type 2 diabetes in 42%, a left ventricular ejection fraction (EF) of 38±13%, EF35% 39%, NYHA Functional Class IV in 56%). Cardiohepatic syndrome (CHS) was diagnosed if at least one indicator of liver function was increased; acute kidney injury (AKI) was diagnosed using the KDIGO criteria.AKI and CHS had been previously diagnosed in 60 (18.6%) and 274 (85.1%) patients, respectively. Among the patients with signs of kidney and/or liver dysfunction, the incidence of isolated CHS, concurrent AKI and CHS, and isolated AKI was 78.4, 20.1, and 1.5%, respectively. The patients with concurrent kidney and liver dysfunctions were observed to have more profound systemic hemodynamic changes (hypoperfusion and congestion). The risk of concurrent AKI and CHS increased glomerular filtration rate (GFR)45 ml/min/1.73 m2, admission systolic blood pressure110 mm Hg, needs for vasopressors, hydropericardium, and EF35%. The concurrence of AKI and CHS was associated with longer hospital stay (15.7±6.5 and 13.5±4.8 days, respectively; p0.05).The incidence of concurrent AKI and CHS in patients with decompensated HF is 20.1%. Concurrent kidney and liver dysfunctions is associated with more obvious signs of hypoperfusion and congestion and characterized by worse prognosis.Цель исследования. Изучить частоту, характер и прогностические факторы сочетанного нарушения функции почек и печени у пациентов с декомпенсацией сердечной недостаточности (СН). Материалы и методы. У 322 пациентов с декомпенсацией СН (возраст 69,5±10,6 года, артериальная гипертония у 87%, инфаркт миокарда у 57%, фибрилляция предсердий у 65%, хроническая болезнь почек у 39%, сахарный диабет 2-го типа у 42%; фракция выброса - ФВ левого желудочка35% у 39%, NYHA IV функциональный класс у 56%) оценены показатели функции почек и печени. Сердечно-печеночный синдром (СПС) диагностировали при повышении хотя бы одного показателя функции печени, острое повреждение почек (ОПП) - по критериям KDIGO. Результаты. Раннее ОПП диагностировано у 60 (18,6%) пациентов, СПС - у 274 (85,1%). Среди пациентов с признаками нарушения функции почек и/или печени частота изолированного СПС, сочетания ОПП и СПС, изолированного ОПП составила 78,4, 20,1 и 1,5% соответственно. У пациентов с сочетанным нарушением функции почек и печени наблюдались более выраженные изменения системной гемодинамики (гипоперфузии и застоя). Риск сочетанного ОПП и СПС увеличивали СКФ45 мл/мин/1,73 м2, систолическое артериальное давление110 мм рт.ст. при поступлении, потребность в вазопрессорах, гидроперикард, ФВ35%. Сочетание ОПП и СПС ассоциировалось с большей длительностью пребывания больных в стационаре (15,7±6,5 и 13,5±4,8 дня соответственно; p0,05). Заключение. Частота сочетанного ОПП и СПС у пациентов с декомпенсированной СН составляет 20,1%. Сочетанное нарушение функции почек и печени ассоциируется с более выраженными признаками гипоперфузии, застоя и характеризуется худшим прогнозом.

Scientifically proven close relationship of nonalcoholic fatty liver disease with development of metabolic syndrome and its individual components involves the conclusion that the target organ in metabolic symptom, even regardless of the severity of obesity, the liver occupies a dominant position, as the body undergoes the first characteristic of non-alcoholic fatty liver disease changes, involving violation of metabolism in the body. Dislipoproteinemia plays an important role in the formation of metabolic syndrome in obesity and other obesity-associated diseases. Altered liver function are the root cause of violations of processes of lipid metabolism and, consequently, abnormal functioning of the liver may be a separate, additional and independent risk factor for development of dyslipidemia and obesity as the main component of the metabolic syndrome.

Currently, HIV infection is becoming epidemic. More than 42% of HIV-infected people in the world are women of childbearing age. With the onset of antiretroviral therapy, chemoprophylaxis during pregnancy, and planned cesarean section, the risk of perinatal HIV transmission decreases to 1–2%. However, various side effects such as anemia, neutropenia, thrombocytopenia, fatty liver dystrophy, toxic hepatitis, acute pancreatitis, increased serum transaminases, changes in biochemical parameters of liver function, impaired glucose tolerance, and hypocoagulation may occur in the context of antiretroviral therapy. This poses to the anesthesiologist a number of new tasks to ensure patient safety during the perioperative period.

The paper presents the results of a study of the functional state of the hepatobiliary system and rehabilitation capacities in patients with diabetes mellitus type 2. Most patients were laced absorption (9 patients (50%) — Tmax=14,989±1,477 minutes), excretory (9 patients (50%) — T1/2=61,644±10,761 min.) the hepatocyte function and motor function of the gallbladder (8 patients (44,4%) — Tmax=51,775±11,294 min.). Violations of the absorptive and excretory functions of the liver depended on the duration of diabetes, glycemic level, the status of fat metabolism and body weight. Rehabilitation capacity of the organism was high 55,6% of patients and was dependent on the state of absorption of liver function, age, diabetes mellitus, and compensation of carbohydrate and fat metabolism.

Aim . To present rare clinical observation of successful surgical treatment of the patient with double-chamber right ventricle in combination with ventricular septal defect, subaortic fi brosis and muscular stenosis, insufficiency of the aortic valve and extrahepatic portal hypertension. Description . Patient F., 15 years old, was diagnosed with Congenital Heart Disease – double-chambered right ventricle, ventricular septal defect. Fibromuscular subaortic stenosis. Aortic valve insuffi ciency II. Circulatory failure II A., functional class III. Extrahepatic portal hypertension. Splenomegaly. Thrombocytopenia. The state after the imposition of splenorenal anastomosis in September 1998 and the imposition of mesocaval H-shaped anastomosis in 1998. Non-functioning anastomoses. Patient indicated for surgical correction of CHD, however, given the low level of platelets, expressed splenomegaly, leucopenia, the patient was referred for a preliminary treatment to Rogachev’s Pediatric Hematology, Oncology and Immunology Center. The treatment (stimulating thrombopoiesis using romiplostim) gave no signifi cant effect. Platelet count reached 70–90 A~ 10 9 . Seeing of frequent bleeding from esophageal varices, the patient underwent varix sclerosis. At the time of hospitalization – no esophageal varices. The patient appealed to V.I. Shumakov Federal Research Center of Transplantology and Artifi cial Organs where he was recommended for surgical treatment. Result . The patient performed surgery: radical correction of CHD: resection of the stenosis of the outfl ow tract of the right ventricle, subaortic stenosis resection, closure of ventricular septal defect, AV plasty under cardiopulmonary bypass. The early postoperative period was uneventful. Leukopenia was observed to 1,2 A~ 10 9 , thrombocytopenia 70–90 A~ 10 9 . Despite the low level of platelets bleeding in pre-and postoperative period was not registered. Antibiotic therapy with tienam. Good postoperative results were obtained. Liver function was not affected. Conclusion . Presented clinical case demonstrates the possibility of surgical intervention of combined heart disease with cardiopulmonary bypass in patient with extrahepatic portal hypertension.

Aim: Clinical case of successful two-stage surgical treatment of a 1-year-old child with congenital heart disease and biliary cirrhosis is represented in this article. At the fi rst day of life laparotomy was performed because of high intestinal obstruction. Kasai procedure and Roux-en-Y choledochojejunostomy were per- formed on 12 th day and at the end of second month of life, respectively. Liver biopsy showed the signs of biliary cirrhosis. At the same time ventricular septal defect and atrial septal defect with pulmonary hyper- tension were diagnosed. The fi rst step of treatment was the surgical septal defects closure. No complications during procedure, cardiopulmonary bypass and post-operative period were registered. There were no nega- tive effects on liver function after cardiac surgery. 11 months later living-donor liver transplantation was performed without any complications. Patient was discharged at 35 th post-transplant day with stable graft function.