Search

Objective: to evaluate the role of physical activity (at sports games) in improving the quality of life of organ recipients.Materials and methods. We examined 42 adult lung, heart, kidney and liver recipients, and patients undergoing renal replacement therapy (mean age 42.6 ± 12.09 years) - participants of the First Russian Transplant Games. The results were analyzed. Quality of life of the recipients was assessed using the nonspecific SF-36 questionnaire.Results. After solid organ transplantation, the interviewed recipients answered that they try to lead an active lifestyle, to engage in accessible physical activities, and to participate in sports events intended for organ recipients. Assessment of the quality of life according to the SF-36 questionnaire showed that all the participants had high scores in terms of the physical and psychological component, which is associated with regular physical training and sports. Conclusion. Physical exercise and active participation in sports activities are an important component in the socialization and rehabilitation of organ recipients. These two factors also improve the psychological and physical components of the quality of life of the recipients.

Immunosuppressive therapy (IMT) is the cornerstone of treatment after transplantation. The goal of immunosuppression is to prevent acute and chronic rejection while maximizing patient survival and long-term graft function. However, the expected effects of IMT must be balanced against the major adverse effects of these drugs and their toxicity. The purpose of this review is to summarize world experience on current immunosuppressive strategies and to assess their effects on renal function.

Deceased brain-dead donor liver transplantation (LT) is a high-risk intervention. The outcome depends on a large number of modifiable and non-modifiable factors. Objective: to analyze our own experience and identify preoperative and perioperative prognostic factors for poor outcomes in LT. Materials and methods. The study included 301 liver transplants performed between January 2016 and December 2021. Donor and recipient characteristics, intraoperative data, perioperative characteristics including laboratory test data, and the nature and frequency of complications were used for the analysis. Results. The 1-, 3- and 5-year recipient survival rates were 91.8%, 85.1%, and 77.9%, respectively; graft survival rates were 90.4%, 83.7%, and 76.7%, respectively. The most significant predictors of poor outcome of LT on the recipient side were biliary stents (HR 7.203, p < 0.01), acutely decompensated cirrhosis (HR 2.52, p = 0.02); in the postoperative period, non-surgical infectious complications (HR 4.592, p < 0.01) and number of reoperations (HR 4.063, p < 0.01). Donor creatinine level (HR 1.004, p = 0.01, one factor analysis; HR 1.004, p = 0.016, multivariate analysis) was the only reliable prognostic negative factor. Conclusion. LT taking into account established risk factors will improve surgery outcomes and help personalize the therapy for each patient.

Primary cardiac sarcoma is a rapidly progressive, aggressive cardiac tumor that is very rare in the general population. Conservative treatment for this tumor is not very effective. The only effective treatment is radical surgical removal of the malignancy. However, if sarcoma removal cannot be performed, heart transplantation (HT) becomes the only treatment option. The median survival of heart recipients with preoperative cardiac sarcoma is 8.5 months. Globally, such operations are performed in a small number of cases. This paper presents the first experience of HT for a patient with primary cardiac sarcoma in the Russian Federation.

Objective: to monitor the current trends and developments in organ donation and transplantation in the Russian Federation based on data from the year 2021. Materials and methods. Heads of organ transplant centers were surveyed through questionnaires. Data control was done using the information accounting system of the Russian Ministry of Health. We performed a comparative analysis of data obtained over years from various federal subjects of the Russian Federation and transplantation centers. Results. Based on data retrieved from the 2021 Registry, 45 kidney, 29 liver and 17 heart transplantation programs were existing in the Russian Federation as of the year 2021. The kidney transplant waiting list in 2021 included about 10.5% of the 60,000 patients receiving dialysis. Organ donation activity in 2021 was 4.5 per million population, with a 78.4% multi-organ procurement rate and an average of 3.0 organs procured from one effective donor. In 2021, there were 9.5 kidney transplants per million population, 4.2 liver transplants per million population and 2.0 heart transplants per million population. Same year, the number of transplant surgeries performed in the Russian Federation increased by 18.3% compared to the year 2020, reaching the level of 2019. In Moscow, organ donation activity was 23.7 per million population, that of 2019. In 2021, the city of Moscow and the Moscow region accounted for 12 functioning organ transplant centers, performing 57.7% of all kidney transplants and 70.5% of all extrarenal transplants in the country. The number of organ recipients in the Russian Federation has exceeded 140 per million population. Conclusion. In 2021, donor activity and volume of transplant care in Russian regions recovered. This was after the decline in 2020 that resulted from the new coronavirus disease (COVID-19) pandemic. In addition, 7 new transplant programs were established. Further development of regional organ donation and transplantation programs, improvement in their efficiency, increase in the activity of transplant centers and development of inter-regional collaboration are expected in the Russian Federation in 2022.

Objective: to study the effectiveness of correcting the morphofunctional characteristics of the liver in an experimental model of chronic liver disease (CLD), using implanted cell-engineered constructs (CECs).Materials and methods. Experiments were carried out on male Wistar rats (n = 80) aged 6–8 months with an initial weight of 230–250 g. CLD was modeled by inoculating the rats with 60% CCl4 oil solution for 42 days based on a modified scheme. Microgel based on recombinant spidroin rS1/9 was used as a matrix for CECs fabrication. Allogeneic liver cells (LCs) and multipotent bone marrow-derived mesenchymal stem cells (BM-MSCs) from a healthy donor were used as the cellular component of the CECs. The effectiveness of the corrective effect of the implanted CECs was assessed in an experimental CLD model (n = 60) in two groups of rats: Group 1 (control, n = 20, 1 mL of saline solution was injected into the damaged liver parenchyma) and Group 2 (experimental, n = 40, CECs containing allogenic LCs and BM-MSCs in a 5 : 1 ratio in a volume of 1 mL were implanted into the damaged liver parenchyma). For long-term monitoring of the CEC state, the CECs were labeled by additional inclusion in Cytodex-3. The effectiveness of the regulatory effect of CECs on regenerative processes in the liver was evaluated using biochemical, morphological and morphometric techniques, as well as by flow cytometry at 90 days after implantation.Results. In the control group, the mortality rate in CLD was 25%. There was no death in the experimental group with CLD after CEC implantation. The CECs were found to have a corrective effect on the biochemical and morphological parameters of the liver in CLD during 90 days of follow-up, with concomitant preservation of structural cellular homeostasis in the implanted CECs. Conclusion. Implantation of CECs in the liver facilitates effective correction of CLD by activating regenerative processes in the damaged liver, which is due to long-term preservation of structural cellular homeostasis in the CECs.

Over the past 10 years, significant breakthroughs have been achieved in Russian transplantology in the field of regulatory legal framework. During this period, the powers of government authorities in the field of healthcare on organization of transplant care and organ donation have been defined, and sources and mechanisms for target financing of medical activities related to organ donation for transplantation purposes have been identified. The procedure for providing medical care under surgery (human organ and/or tissue transplantation) has been adopted, and a state registry system for donor organs, donors and recipients has been created. Measures on organ donation and transplantation in the Russian Federation have been approved within the «Healthcare Development», a framework of the state program of the Russian Federation. The Shumakov National Medical Research Center of Transplantology and Artificial Organs (Shumakov Center) has also been identified as the core institution that coordinates the activities of the entire transplant industry in the Russian Federation. Transplant medical care is currently being provided by specialist physicians trained in human organ and tissue transplantation, in collaboration with other specialist physicians. The Nomenclature of Specialties of Specialists with Higher Medical and Pharmaceutical Education, approved by the Russian Ministry of Health via Order No. 700n of October 7, 2015, does not contain a separate specialty related to human organ and tissue transplantation activities, and this is quite justified. However, in order to improve the legal regulation of transplantation activities, it is necessary to unify the requirements for specialists providing medical care in human organ/tissue transplantation. This can be achieved by developing uniform approaches to the definition of labor functions in the professional standards of specialist doctors involved in transplantation.

According to global health statistics, respiratory diseases, together with infectious complications and hereditary lung diseases, rank as the third leading cause of death. Today, lung transplantation (LTx) is a well-recognized modality of treatment for end-stage chronic lung disease. However, the number of LTx surgeries performed is much lower than other solid organs. This is due to the high requirements for the potential donor and characteristics of the lung graft, reflecting the efficiency of gas exchange function. Non-compliance with the selection criteria leads to deselection of donors, which, according to various estimates, occurs in 80–85% of cases. One of the ways to increase the number of lung transplant surgeries is to restore them to the level of optimal gas exchange parameters, which can be achieved and objectively assessed during normothermic ex vivo lung perfusion (EVLP). EVLP is becoming increasingly common at leading transplantation centers in Europe and North America. This has significantly increased the number of transplant surgeries as a result of using lungs procured from suboptimal donors and rehabilitated via EVLP. In our pilot study, the developed Russian-made mechanical circulatory support system showed that performing normothermic EVLP for isolated lungs under experimental conditions is feasible. Basic and optimized perfusion protocols have fully shown that they are reliable and efficient.

Kidney injury in cardiac transplant recipients is one of the most severe complications affecting both short- and long-term transplant outcomes. The need for renal replacement therapy (RRT) is determined not only and not so much by the degree of renal dysfunction, as by the need for correction of fluid balance and metabolic disorders. These circumstances are associated with the specificity of extracorporeal renal replacement therapy in donor heart recipients. In this review, we discuss the problems of early versus delayed initiation of RRT, anticoagulation and vascular access, advantages and disadvantages of continuous and intermittent techniques. Special attention is paid to chronic kidney injury and peculiarities of kidney transplantation in heart recipients.

Kidney injury in heart transplant recipients is of a complex nature and bears the features of all types of cardiorenal interaction impairment. Pre-transplant renal dysfunction, perioperative acute kidney injury, as well as factors associated with graft and immunosuppression, determine the prevalence and severity of kidney pathology in this group of patients. This review examines the pathophysiology of kidney dysfunction in heart failure, the epidemiology, and criteria for acute kidney injury.

Bronchial complications are among the main causes of impairing postoperative period and thansplant failure. Severe bronchial complications are very rare but have a high mortality rate. Light forms decrease transplant function and while progressing can leads to life-threatening conditions without required treatment. Nowadays there is a huge necessity in classification of diagnostic and bronchial complications treatment on different terms after lung transplantation. Methods of observation bronchoscopy and interventional bronchology are allowing us to realize prevention, diagnostic and treatment bronchial complications.

Liver transplantation is the treatment of choice for patients with end-stage liver disease or acute liver failure. However, vascular complications, such as hepatic artery stenosis and/or thrombosis, graft portal vein stenosis and biliodigestive strictures following liver transplantation are still common despite improvements and innovations in surgical techniques. These complications can lead to graft damage or even death, and they are caused by many factors. Although minimally invasive interventional radiology is an optional treatment for such post-liver transplant complications, there is little research on this method of treatment.

Living related liver transplantation has proved to be an effective, safe and radical method for treating end-stage liver diseases. In the last decade, a laparoscopic approach to donor hepatectomy has been gradually introduced into clinical practice. According to world literature, there are presently no uniform standards for performing laparoscopic liver resections in living donors. This literature review considers almost all methods for performing this surgery in living donors. These methods are described in transplant centers around the world.

Liver transplantation is the only effective treatment modality for end-stage liver disease. However, donor organs are not always available. In some cases, the gravity of the patient’s condition makes transplantation impossible. In this regard, the use of artificial liver support systems helps in preparing a patient for transplant surgery. Objective: to conduct a retrospective study aimed at evaluating the efficiency of fractionated plasma separation and adsorption system. Materials and methods. From January 2019 to May 2020, 139 pediatric liver transplants were. We analyzed the data of 5 pediatric patients (2 girls and 3 boys, aged 12 to 17 years) who received fractionated plasma separation and adsorption (FPSA) sessions as a bridge to transplantation. The main clinical indication for FPSA was severe hepatic encephalopathy (grade 3 according to the West Haven Criteria), which was observed at 350–872 μmol/L (average 597 ± 98 μmol/L) serum bilirubin level. The FPSA sessions were conducted on a Prometheus device using AV-600 hemofilters as dialyzers (Fresenius Medical Care, Germany). Results. Depending on the extent of bilirubinemia in patients, it took from one (in one case) to three (in one case) daily FPSA sessions to restore clear consciousness, appetite and physical activity. Average bilirubin levels after treatment cycles decreased from 597 ± 98 to 236 ± 73 μmol/L. All patients successfully underwent liver transplant surgery within two to five days, two patients received a liver fragment from a living related donor. Conclusion. The FPSA system stabilizes the condition of potential recipients with acute liver failure. Further research is required to develop optimal regimens for albumin dialysis.

Many studies have shown that biliary complications after transplantation of the left lateral segment (LLS) of the liver reduce graft and recipient survival. Thus, timely correction of biliary complications, and strictures in particular, improves long-term outcomes in transplantation. Objective: to analyze our own experience in correcting biliary strictures in LLS graft transplantation. Materials and methods. From February 2014 to April 2020, 425 LLS grafts were transplanted in children. 19 (4.5%) patients were diagnosed with biliary strictures at different times after transplantation (from 0.2 to 97 months). Results. Biliary strictures were more often formed a year after transplantation (17.8 ± 23.9 months). In 14 out of the 19 patients, internal-external biliary drainage was successfully performed with phased replacement of the catheter with one that was larger in diameter (from 8.5 Fr to 14 Fr). The catheters were removed in 8 patients after completion of the treatment cycle. Restenosis was not observed during follow-up (13 ± 8.7 months) after the internal-external biliary drainage catheter had been removed. In 5 cases, antegrade passage of a guide wire through the stricture was unsuccessful. As a result, biliary reconstruction was performed in 4 (21.1%) patients and retransplantation was required in 1 (5.3%) patient. Conclusion. An antegrade minimally invasive approach can successfully eliminate biliary strictures in most children after liver LLS graft transplantation. The proposed technique is effective and safe.

Aim: to evaluate clinical effi ciency of percutaneous coronary interventions (PCI) at potential recipients of heart with the chronic ischemic heart failure (CHF).Materials and methods. In this retrospective study results of treatment at 76 patients with CHF NYHA III and 36 patients with CHF NYHA IV by scheduled PCI are presented. Duration of observation was from 6 to 160 months. The age of patients at the time of intervention was 61,7 ± 0,62 years (from 33 to 76). 108 male and 4 female. Defi ned life expectancy and echocardiography parameters of the left ventricle (LV) of heart. Results.20 patients were died, 16 of them from cardiovascular events. 18 patients during this period undergo orthotopic heart transplantation (HT). The endovascular revascularization leads to LV volumes reduction, increase of ejection fraction and decrease of pulmonary artery pressure. Progress CHF is followed by negative dynamics of these characteristics and need of HT performance. In 74% of cases the effect of myocardial revascularization allows to provide increase in life expectancy of potential recipients of heart with the chronic ischemic heart failure. Conclusion.Performance of PCI at patients with the chronic ischemic heart failure can delay heart transplantation or become its alternative.

Donor organs shortage leads to extending criteria for deceased liver donation in the whole world.Aim:to compare results of deceased donor liver transplantation (DDLT) depending of donor age over 60 years old.Materials and methods:the study includes 390 DDLT from January 2010 to November 2017. All liver donors separated by age for two groups: I – 60 years and older (n = 26); II – younger than 60 years (n = 364). All donors were standardized by demographic, laboratory fi ndings and inotropic drug requirement. Results:no difference between both groups in severity of ischemia-reperfusion injury, ICU or in-hospital staying (median 2 and 7,5 days respectively) was found. There is also no difference between biliary or vascular complication rate. 5-year actuarial survival rate found no difference between both groups (I: 70%: II: 76%, p = 0,54).Conclusion.Using grafts from donors older than 60 years don’t worsen early and late results of DDLT. Care should be taken to avoid other risk factors (cold ischemia time, warm ischemia time).

The method of mechanical circulation support using non-pulsating fl ow pumps, built on the principle of rotary (centrifugal and axial) pumps, took the leading direction (94%) in the world clinical practice for the treatment of the patients with terminal heart failure. Despite this, the clinic application of these pumps in a number of cases faced with the numbers of negative problems associated with this technology. This is stimulated of a new direction of principles for a control of the rotary pumps, based on the modulation of the speed pumps. The article analyzes the negative factors of the clinical application of non-pulsating fl ow pumps and gives an overview of the methods the optimization of the control pump based on the modulation of the output fl ow.

Aim:to develop an optimal surgical tactic for implantation of “AVK-N” system as a “bridge” to heart transplantation.Materials and methods.17 patients were included. They were operated in the period from 2012 to October 2017 in Federal State Budgetary Institution «V.I. Shumakov National Medical Research Center of Transplantology and Artifi cial Organs». A tiny implantable system «Portable device for assisting cardiac circulation» (AVK-N; Russia) was used for replacing the pumping function of the left ventricle. All patients were examined according to the program of potential recipients for heart transplantation, before the applying of prolonged mechanical circulatory support. Among the operated patients there were 16 (94.1%) men and 1 (5.9%) woman, the average age was 52.64 ± 10.56 (from 33 to 67 years). All patients had congestive heart failure III–IV functional class according to NYHA, refractory to optimal drug therapy. Heart failure was triggered by dilated cardiomyopathy in 12 (70,58%) cases, and by postinfarction systolic dysfunction of the left ventricle in 5 (29,42%). Implantation of «AVK-N» system was performed to potential recipients of the donor heart with terminal stage of CHF with a decrease in LV ejection fraction up to 10%.Results.As a result of this study there were developed several technological aspects facilitating the subsequent heart transplantation.Conclusion.Our experience in optimizing the surgical tactics of the “AVK-N” system implantation as a bridge to heart transplantation, demonstrated the possibility and safety of its active use in both patients with terminal heart failure on the waiting list of heart transplantation and patients having temporary contraindications to HTX.

Diabetes mellitus is a significant social problem. In the Russian Federation, the prevalence of diabetes type 1 is 340.000 people, 21% of them having diabetic nephropathy, as well as other secondary complications leading to disability and high mortality. There are several options for diabetic patients with chronic kidney disease dialysis: kidney transplantation with insulin therapy, simultaneous kidney-pancreas transplant or islet transplant. Good long-term results could be obtained by the whole pancreas and kidney combined transplantation through maximum metabolism recovery comparing to other replacement therapy options. The recipientselection with special attention to the cardiovascular system evaluation is essential to successful transplantation. The proper pancreatic graft assessment needs a wide range of criteria to be analyzed. Integral scores can simplify and systematize the assessment. The choice of preservation technique is important to pancreatic graft ischemia-reperfusion injury minimization. Despite the progress in graft preservation methods, cold storage using University of Wisconsin (UW) solution remains to be the gold standard. The evolution of surgical technique has resulted in utilizing the most physiologic variations of exocrine drainage and venous outflow of the pancreas, so-called portoenteric drainages, which were designed to improve remote metabolic effects and to reduce complications probability.

Over the last decades mechanical circulation support has become one of the most effective treatment methods for patients with terminal heart failure. Unfortunately, in pediatrics this method is generally used on a short-term basis and includes, first of all, extracorporeal membranous oxygenation and left ventricular bypass by means of the extracorporeal centrifugal pumps. Nevertheless, using of these methods in children allowed considerably reducing mortality on the waiting list for heart transplant. Today, practically the only method of long term mechanical circulatory support in newborns and children at an early age being used is the system of paracorporeal artificial ventricles EXCOR, the usage of which results in larger problems of thrombosis. There is little clinical experience in using of the fully implanted systems developed for adults in older children. Therefore, as shown in the present review, the problem of creating long term circulatory support systems for newborns and younger children on the basis of the implanted pumps continues to be relevant.

Orthotopic liver transplantation is the only way to cure chronic liver failure and certain liver tumors. Deceased donor organs are the prevailing source in most transplantation centers. However, there is an organ shortage because of the increasing number of patients in need of transplantation. Using expanded criteria deceased donors is a routine way to overcome organ shortage. The majority of transplantation centers take older donors depending on the local rules. Successful liver transplantation from 73-year-old deceased donor. Recipient suffered from liver neuroendocrine tumor without signs of extrahepatic spread. Liver function is adequate during 90-day follow-up. Liver transplantation from older deceased donors has good outcome according to world experience. Careful donor-recipient selection is the key to success. The first positive trial in our center enlightens the way for further practice.

Aim. A comparative analysis of the functional efficacy of biomedical cell products (BMCP) for the regeneration of damaged liver based on biopolymer scaffolded porous and hydrogel matrices was performed on the experimental model of acute liver failure. Materials and methods. Matrices allowed for clinical use were employed for BMCP in the form of a sponge made from biopolymer nanostructured composite material (BNCM) based on a highly purified bacterial copolymers of poly (β-hydroxybutyrate-co-β-oxyvalerate) and polyethylene glycol and a hydrogel matrix from biopolymer microheterogeneous collagen-containing hydrogel (BMCH). Cellular component of BMCP was represented by liver cells and multipotent mesenchymal bone marrow stem cells. The functional efficacy of BMCP for the regeneration of damaged liver was evaluated on the experimental model of acute liver failure in Wistar rats (n = 40) via biochemical, morphological, and immunohistochemical methods. Results. When BMCP was implanted to regenerate the damaged liver on the basis of the scaffolded BNCM or hydrogel BMCH matrices, the lethality in rats with acute liver failure was absent; while in control it was 66.6%. Restoration of the activity of cytolytic enzyme levels and protein-synthetic liver function began on day 9 after modeling acute liver failure, in contrast to the control group, where recovery occurred only by days 18–21. Both matrices maintained the viability and functional activity of liver cells up to 90 days with the formation of blood vessels in BMCP. The obtained data confirm that scaffolded BNCM matrix and hydrogel BMCH matrix retain for a long time (up to 90 days) the vital activity of the adherent cells in the BMCP composition, which allows using them to correct acute liver failure. At the same time, hydrogel matrix due to the presence of bioactive components contributes to the creation of the best conditions for adhesion and cell activity which accelerate the regeneration processes in the damaged liver compared to BMCP on scaffolded matrix. Conclusion. A statistically significant difference was found between the functional efficacy of the BMCP studied based on BNCM and BMCH matrices. BMCP based on hydrogel BMCH matrix was more effective for the regeneration of damaged liver.

Introduction. Liver transplantation is a multi-component and complex type of operative treatment. Patients undergoing such a treatment sometimes are getting various complications. One of these complications is a portal hypertension associated with portal vein stenosis.Materials and methods. In 6 years after the left lateral section transplantation from living donor in a pediatric patient the signs of portal hypertension were observed. Stenosis of the portal vein was revealed. Due to this fact percutaneous transhepatic correction of portal vein stenosis was performed.Results. As a result of the correction of portal blood flow in the patient a positive trend was noted. According to the laboratory and instrumental methods of examination the graft had a normal function, portal blood flow was adequate. In order to control the stent patency Doppler ultrasound and MSCT of the abdominal cavity with intravenous bolus contrasting were performed. Due to these examinations the stent function was good, the rate of blood flow in the portal vein due to Doppler data has reached 80 cm/sec, and a decrease of the spleen size was noted.Conclusion. Diagnosis and timely detection of portal vein stenosis in patients after liver transplantation are very important for the preservation of graft function and for the prevention of portal hypertension. In order to do that, ultrasound Doppler fluorimetry examination needs to be performed to each patient after liver transplantation. In cases of violation of the blood flow in the portal vein CT angiography performance is needed. Percutaneous transhepatic stenting of portal vein is a minimally invasive and highly effective method of correction of portal hypertension. Antiplatelet therapy and platelet aggregation control are the prerequisites for successful stent function.

In most cases, severe left ventricular systolic dysfunction is a consequence of ischemic heart disease. In clinical condition, current recommendations support myocardial revascularization in patients with ischemic cardiomyopathy by performing coronary artery bypass grafting. Information on safety and effectiveness of percutaneous coronary intervention in patients with ischemic heart failure is limited. The aim of this review is to study the current state of this problem.

«Russian Journal of Transplantology and Artificial Organs»: еnglish version.

Aim. To carry out the analysis of enforcement of regulatory legal acts in transplantological practice, to specify the existing gaps and collisions in them, and to offer approaches for its elimination. Materials and methods. The legislation of the Russian Federation in the fi eld of donation and organ transplantation and(or) tissues (an information law system «Garant»). Archive of addresses to the chief transplantologist of the Russian Ministry of Healthcare concerning application of certain precepts of law in transplantology since 2009 till present. Results. Legislative and subordinate acts relating to the area of donation and organ transplantation and(or) tissues are studied; addresses to transplantologies regarding application of certain law precepts are analyzed. Conclusion. Legislative and subordinate acts in force in the fi eld of donation and organ transplantation and(or) tissues contain gaps and collisions which interfere with the work and development of the industry. With respect thereto, it is reasonable to make amendments eliminating legal defects to a number of regulatory legal acts: to the Federal law No. 323-FZ, to the Act of the Russian Federation No. 4180-I, to the Resolutions of the Government of the Russian Federation No. 291 and No. 294, to the Orders of the Ministry of Healthcare of the Russian Federation No. 567n, No. 307n/4, No. 355n.

Aim.Analysis of relationship between transforming growth factor beta-1 (TGF-β1) level in blood and liver disease severity before and after liver transplantation in early age children with congenital liver diseases.Materials and methods.The study included 135 pediatric patients aged from 2 to 73 months with end-stage liver disease.Results.The level of TGF-β1 in the blood of children with liver failure on average was lower than in healthy children of the same age. The cytokine level depended on the liver disease etiology: in patients with biliary atresia, biliary hypoplasia, Alagille syndrome, Byler disease and other diseases it was lower than that at Caroli disease when it did not differ from the level in healthy children. The level of cytokine in the blood plasma of patients was associated with the severity of hepatic fi brosis: in fi brosis of grade 1 and 4 it was lower than in fi brosis of grade 2 and 3. The liver transplantation from related living donor resulted in increase of TGF-β1 level in the blood plasma of patients regardless of the initial etiology and severity of the liver disease.Conclusion.The blood level of TGF-β1 refl ects liver disease severity in children with congenital liver diseases and may be used as a marker of liver function state before and after liver transplantation.

Aim. To carry out monitoring of the organization and development of the organ donation and transplantation in theRussian Federationaccording to 2015.Materials and methods. Questioning of heads of all the centers of transplantation is carried out. The comparative analysis of the obtained data in dynamics by years, between certain regions of theRussian Federation, the transplantation centers is done.Results. According to the register in2015 inthe Russian Federation 36 centers of renal transplantation, 17 centers of liver transplantation and 10 centers of heart transplantation were functioning. The waiting list of kidney transplantation in 2015 included 4167 potential recipients that make 13% of the total number of the patients (31 500) receiving a dialysis. The rate of donor activity in 2015 made 3.0 pmp. Efficiency of donor programs in 2015 continues to increase: the share of multiorgan retrievals made 57.8%, average number of organs, received from one effective donor, made 2.7. In 2015 the rate of kidney transplantation made 6.5 pmp; the rate of liver transplantation made 2.2 pmp; the rate of heart transplantation made 1.2 pmp. The number of transplantations of liver and heart in theRussian Federationcontinues to increase. The number of transplantations of kidney remains approximately at one level in the range of 950–1050.Moscowcapital region continues to be the center of stability and development of the organ donation and transplantation in the country, in which 10 centers of transplantation are functioning and nearly a half from all kidney transplantations and more than 65% of all liver and heart transplantations are carried out.Conclusion. The potential for further development of the transplantation care in theRussian Federationcontinues to persist. In particular, at the expense of increasing efficiency of regional donation programs, expanding practices of multiorgan recuperation and transplantations of extrarenal organs, through interregional transplant coordination. It is critical to keep the volumes of the state order to deliver transplantological medical care to the population and to implement federal funding to conduct donation programs.

Transplantation of liver left lateral section (LLS) firmly established itself as a radical and effective method of treatment of advanced diffuse and unresectable focal liver diseases in pediatric patients. At the same time surgical community faced the challenge of matching the size of the adult donor’s graft to the volume of the child’s abdomen. Review of the literature presents historical aspects of transplantology, some approaches to measurement of the required liver parenchyma functional mass and methods to prevent complications associated with the usage of large LLS grafts in infants. In addition, the latest data on estimation of intra-abdominal pressure and development of intra-abdominal hypertension syndrome are also presented.

In cardiac recipients non-invasive monitoring will identify the risk of acute rejection, as well as reduce the number of routine biopsies. The relation between the development of the transplanted heart rejection and the change of elastic properties of common carotid artery has been proved. Aim: to analyze dynamics of iRIG in treatment of graft rejection in heart recipients.Materials and methods. 59 heart recipients were examined: 21 patients (pts)without rejection, 20 pts – with rejection after 1st biopsy, 15 pts – with rejection after 1st and 2 biopsies, 3 pts – with persistent rejection after all biopsies. iRIG was estimated using empirical equation.Results. Dynamics of iRIG in pts. without rejection according to all biopsies over time was not signifi cantly changed 6280 ± 2143, 6083 ± 2388, 6362 ± 1984 and 6188 ± 3012 cm/sec2 (p = 0.11, p = 0.13, p = 0.17 between the 1st and 2nd, 2nd and 3rd, 3rd and 4th values, respectively). In pts. with rejection iRIG decreased during treatment, but was signifi cantly higher even after successful treatment. In patients with persistent rejection iRIG did not decrease and tended to increase (17 459 ± 9702 cm/sec2 on the results of the 1st biopsy to 21 305 ± 10 448 cm/sec2 on the results of the 4th biopsy).Conclusions. In patients with heart transplant iRIG does not change signifi cantly with the time after transplantation. iRIG increases in all types of rejection, followed by its decrease in the course of therapy; in patients with persistent rejection iRIG remains high. Evaluation of iRIG can be used for non-invasive monitoring of patients after heart transplantations (HTx) and to identify patients at high risk of transplant rejection.

Living donor liver transplantation is a highly effective method to help children with end stage liver diseases. Projected success of operation is largely determined at the stage of selection of potential donor. In our review of the literature is presented historical information, are considered «eastern» and «western» way of development of pediatric living donor liver transplantation, are analyzed the ethical and psychosocial aspects of living donor liver transplantation, and also are set out principles and protocols for evaluation potential donors. In addition, the modern views on volumetry of the potential donor liver and on choice of graft type for transplantation, including for children with low weight are presented.

Dear colleagues!

It is shown that developed method of titrating AB0 antibodies allows defi ning the titer of the investigational antibodies more precisely on 1–3 dilution of serum compared to the prototype method (titration method of antibodies in saline medium on the plane). It is more obvious as it excludes hardly interpretable results due to the possibility of conducting visual assessment of agglutination reaction in the gel card thick column and requires less time for analysis. The results can be saved for comparison with the results of further research. That is not possible under prototype titration method. Aim : our aim is to create a laboratory technique that can accurately, reliably and clearly produce titration of AB0 system antibodies, including in patients with low initial concentration of agglutinins in the blood; a technique more economical in terms of spending serum and that takes less time. Materials and methods : those modes were empirically chosen which allow titration of AB0 system agglutinins using gel technology based micro typing; to titer group antibodies 1640 serum assays of recipients in AB0-incompatible transplantation were analyzed. The result of the use of specially developed method in organ transplantation from incompatible blood donors consists in enhancing accuracy, sensitivity of natural, complete and incomplete AB0 system immune antibodies titration, in its clarity, using of blood micro-doses for earlier detection of sensitizing of the patient, which is especially important in Pediatrics. Conclusion: the developed procedure of AB0-antibodies’ titration using modern gel technology makes possible a more precise monitoring of the titer of antibodies that is necessary to predict the graft rejection risk, to select the Protocol of preoperative preparation and postoperative management of patients, to assess the effectiveness of therapy in patients for whom it is diffi cult to fi nd a compatible blood type donor, and for whom today AB0-incompatible transplantation is a life-saving.

Transforming growth factor β1 (TGF-β1) plays a key role in the development of the immune response, as well as in the process of liver regeneration. Measuring the level of TGF-β1 may have important clinical implications in liver transplantation, because cytokine concentration in the tissue and in blood plasma varies with different liver diseases. Aim. To analyze the dynamics of TGF-β1 levels in children-recipients with liver transplant from related donors, including from incompatible blood groups. Materials and methods. The study involved 127 children aged 3 to 72 months (median – 8, average age – 12 ± 14 months), including 57 boys and 70 girls, with liver cirrhosis, developed as the result of congenital and hereditary diseases of the hepatobiliary system. All patients underwent transplantation of the left lateral liver sector from living related donors: 98 patients were transplanted fragment of a liver from identical or AB0-compatible donors and 29 – from incompatible donors. The concentration of TGF-β1 was determined by enzyme immunoassay method in blood plasma samples. Results. Average level of TGF-β1 in blood plasma of children with liver cirrhosis, developed as the result of congenital and hereditary diseases of the hepatobiliary system was 5,2 ± 5,5 ng/ml. A month after liver transplantation from a related donor level of TGF-β1 in blood plasma of recipients increased to 8,1 ± 9,6 ng/ml. One year after transplantation, the average level of TGF-β1 in the recipients of liver fragment was 7,7 ± 8,4 ng/ml, and signifi cantly (p = 0,00) differed from the level prior to transplantation. No association between TGF-β1 level in a month and a year after transplantation and the compatibility of the recipient with AB0 donor was found. A correlation (r = –0,23, p < 0,05) between level of TGF-β1 prior to transplantation and the development of graft dysfunction was observed: in recipients with graft dysfunction (16 cases) cytokine level prior to surgery was lower (p = 0,047) than in other recipients. Conclusion . Liver transplantation leads to a signifi cant increase in the level of TGF-β1 in the blood plasma of children and that level is not different in recipients after transplantation of a liver fragment from AB0-compatible and AB0-incompatible donors and in recipients with anti-group antibodies before and/or after transplantation, and without them.

Glycogen storage diseases I, III and IV types are congenital disorders, which are commonly associated with severe liver diseases. Liver transplantation has been proposed as a treatment of choise for these disorders. While liver transplantation corrects the primary hepatic enzyme defect, the extrahepatic manifestations of glycogenoses often complicate the posttransplant management. Upon review of the English-language literature, 42 children under 18 years old were discovered to have undergone liver transplantation for complications associated with glycogenoses (18 patients with Ia type, 6 – with Ib type, one patient – with III type, 17 – with IV type). This artic- le represents the pediatric liver transplantation for complications associated with glycogenosis Ia type, analyzed posttransplant period in this recipient.

Monitoring of a condition and prospects of organ donation and transplantation development in Russia in the form of the National Registry is carried out under the auspices of the Pro fi le commission on transplantology of Minis- try of Health of Russia and the Russian Transplant Society. According to the registry in 2012 the indicator of do- nor activity decreased, but the indicator of transplant activity remained at the level of the last years. Decrease the number of deceased donors managed to be compensated by means of increase of ef fi ciency of donor programs: by increase of the number of donors after brain death and multi-organ explantation, by increase in average of the organs received from one deceased donor. In 2012 the number of transplantations of heart and liver increased. The main funding mechanism for organs transplantation in Russia is the state task to the transplant centers (fede- ral fi nancing), its role increases. For increase of stability of donor providing it is necessary to continue to develop legal base in the organ donation and transplantation sphere.

Aim. To study the clinica-morphological features of syndromatic and nonsyndromatic paucity of intrahepatic bile ducts in pediatric liver transplant recipients. Methods and results. The clinical records were analyzed and histological studies of native livers of 20 children, who had suffered from paucity of intrahepatic bile ducts and to whom liver transplantation were made, were completed. The obtained data indicate higher levels of AST in patients with nonsyndromatic paucity of intrahepaticbile ducts (p = 0,023). Ductopenia was the more frequent indication of syndromatic form of paucity of intrahepatic bile ducts (p = 0,01), while ductular proliferations, which form «ductular structure», were discovered more often in nonsyndromatic paucity of intrahepaticbile ducts (p = 0,03). The extent of in fl ammatory-destructive changes was more expressed in nonsyndromatic pauci- ty of intrahepatic bile ducts (p = 0,01). Fibrosis or cirrhosis was formed more often in nonsyndromatic paucity of intrahepatic bile ducts (p = 0,008). Conclusion . Our results indicate more severe clinical and morphological manifestations in nonsyndromatic paucity of intrahepatic bile ducts. These fi ndings may suggest about heavier liver condition in patient with nonsyndromatic form of paucity of intrahepatic bile ducts.

Aim of the work. С linical and morphological research of the features of biliary atresia at children-recipients of liver, to whom a portoenterostomy was made in comparison with patients to whom such surgery wasn't carried out. Materials and methods. Clinical records and histological research of liver of 49 children suffering a bili- ary atresia are analyzed. Two groups were created: the 1st group – 21 children without portoenterostomy before liver transplantation and 2nd group – 28 children with portoenterostomy before liver transplantation. Results. Pathomorphology of liver the characteristics in structure of proliferation bile ducts are established: hypostasis and dystrophic changes of cholangiocytes at patients of the fi rst group (p = 0,035) and sclerosis of bile ducts with disappearance of an epithelium at patients of the second group (p = 0,01). In fi rst group of children activity of in fl ammatory process in liver were signi fi cantly higher than in second group (p = 0,02). Conclusion. Our experi- ence has demonstrated that portoenterostomy doesn’t prevent fi brosis of intrahepatic bile ducts and development of fi brosis, and then a development of cirrhosis.