Репозиторий Университета

© 2018 Russian Transplant Society. All Rights Reserved. Aim. To study the efficacy and safety of the use of paritaprevir, ritonavir, ombitasvir and dasabuvir in combination or without ribavirin in liver recipients with recurrence of HCV 1 genotype after transplantation. Materials and methods. The study included 46 patients after orthotopic liver transplantation with recurrence of HCV 1 genotype. 37 patients completed a 24-week course of antiviral therapy, including paritaprevir, ritonavir, ombitasvir and dasabuvir in combination or without ribavirin. The effectiveness of the therapy was calculated as the proportion of patients who achieved aviremia 12 weeks after the end of the course of treatment. The safety of therapy was assessed by the number of adverse events that occurred during the course of antiviral therapy. Results. A sustained virologic response at 12 weeks after the end of the course of antiviral therapy, including paritaprevir, ritonavir, ombitasvir and dasabuvir, reached 100% of the recipients of the liver. Reduction in the intensity of cytolytic and cholestatic syndromes was noted at week 4 of therapy. Adverse events were recorded in 56.7% of the subjects, mostly they were not severe and were stopped on their own. Acute cellular rejection of the transplant developed in 1 patient (2.7%). There have been no cases of irreversible liver transplant dysfunction or death of the recipient. The conclusion. The use of paritaprevir, ritonavir, ombitasvir and dasabuvir is safe and effective in the treatment of relapse of HCV infection of 1 genotype after liver transplantation.

© 2018 Vserossiiskoe Obshchestvo Kardiologov. All rights reserved. Aim. To evaluate the prevalence of arterial hypertension (AH) in heart transplant recipients, and its influence on the risk of adverse events, as the efficacy and safety of antihypertension medications (AHM). Material and methods. To the study, were consequently included all heart transplant recipients operated in the Shumakov Centre during the years 2013 to 2016 and survived 90 days after orthotopic heart transplantation. Results. Totally, 353 recipients included, with AH or AHM intake in anamnesis in 62 (17,6%). Within 90 days post surgery, AH that demanded for medication therapy was found in 151 (42,8%) patients. In posttransplant AH patients there were the following specific parameters in preoperational period: higher body mass index - 25,7±4,1 vs 24,9±4,4 (р=0,026), blood creatinine concentration - 100,6±62,6 vs 68,8±4,8 (р<0,001), donor heart posterior wall thickness - 11,9±0,8 vs 11,3±0,7 (р=0,034), creatinine concentration in 3 month after operation - 131,7±101,6 vs 94,1±46,5 (p<0,001). There was relation revealed, of AH development risk with anamnesis of AH and renal failure, as a necessity for renal replacement therapy within 30 days post surgery and episodes of acute antibody-mediated reaction on transplant. In the recipients taking angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers (ACEi/ARB) before operation, the survival rate free from adverse events was better than in those taking calcium channel blockers (CCB) (plog-rank=0,042). Conclusion. The results of the study point on high prevalence of AH in heart recipients. Presence of AH in anamnesis, renal failure, episodes of humoral, but not cellular, reaction to the transplant, and donor heart hypertrophy do significantly increase the probability of AH development after transplantation. Comparison revealed significant benefit of ACEi/ ARB versus CCB as antihypertension medications in either monotherapy or in combination with diuretics.

© 2018 Russian Transplant Society. All rights reserved. The article offers analysis of the problem status as well as World and Russian experience of donor organ recipients participation in sports and physical training. The connection of physical activity with the quality of life of patients after transplantation is reflected. The problems associated with organ donation in Russia and other countries and the importance of sports activities in promoting this problem are described. The analysis of foreign experience in the organization of sports games for people with transplanted organs, which indicates: the importance of research methods in admission to physical education and sports among recipients, the need for dosing and selection of physical activity, the involvement of a transplant doctor for individualization of rehabilitation programs. The personal experience of foreign athletes with transplanted organs, including the experience of the Russian recipient’s participation in the 21st world games 2017 is presented. Experience of carrying out sports activities for people with transplanted organs in Russia is also given.

© 2018 Russian Transplant Society. All rights reserved. Aim. To analyse the status and trends in the development of organ donation and organ transplantation in the Russian Federation according to 2017 data. Materials and methods. The survey of heads of transplantation centers was conducted. A comparative analysis of the data obtained in the dynamics of years, between individual subjects of the Russian Federation, the centers of transplantation is performed. Results. According to the register in 2017 in Russia there were only 41 centers for kidney transplantation, 24 liver and 16 hearts. The waiting list for kidney transplantation in 2017 included 5,531 potential recipients, which is approximately 13.8% of the total number of 40,000 patients receiving dialysis. The level of donor activity in 2017 was 3.8 per million of the population, while the share of multiorgan seizures was 66.5%, the average number of organs received from one effective donor was 2.8. In 2017, the level of kidney transplantation was 8.0 per million of the population, the liver transplantation index was 3.0 per million of the population; the rate of heart transplantation is 1.7 per million of the population. In 2017 the number of transplants in Russia increased by 11.3% compared to 2016. There are 11 transplantation centers on the territory of Moscow and the Moscow Region, and half of all kidney transplants and 70% of all liver and heart transplantations are performed. The number of patients with transplanted organs in the Russian Federation is approaching 13,000. Conclusion. In the Russian Federation there is a strong tendency to increase the number of effective donors and to increase the number of organ transplants, and the number of transplant centers is also increasing. In recent years, the country has created prerequisites for the development of organ donation and transplantation: the regulatory and legal framework, public donation funding, material and technical base, etc. In the coming years, positive experience and organizational patterns of organ donation and transplantation from successful regions in Other subjects of the Russian Federation for building effective programs. The leading role in this process should be played by the Academician V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs.

© 2018 Dynasty Publishing House. All rights reserved. The article presents a clinical case of a patient with genetically confirmed Alagille syndrome (ALGS) with marked delay of physical development that did not correspond to the severity of liver damage. Alagille syndrome (ALGS) is a rare hereditary disease with underlying hypoplasia of the intrahepatic bile ducts manifested by cholestasis syndrome in the first weeks of life. Developmental delay is characteristic for cholestatic diseases of the liver, including ALGS, which is conditioned by impaired absorption of fats and fat-soluble vitamins in the intestines. But growth delay and underweight in this syndrome often do not correlate with the severity of cholestatis syndrome, and causes of their development remain unstudied. Cholestatis syndrome was moderate, and clinical signs of liver cirrhosis were absent. Intense skin itching, greatly disturbing not only the baby's wake period but also sleep, along with marked height and weight deficit were indications for liver transplantation at the age of 4 years, after which a fast normalisation of the parameters of physical development was noted. This clinical case study permits to hypothesize that developmental delay in children with ALGS is conditioned by chronic cholestatic liver damage, irrespective of the severity of its clinical presentation.

© 2018 Russian Transplant Society. All rights reserved. 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).

© 2018 Russian Transplant Society. All rights reserved. Aim: To evaluate clinical efficiency 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. Defined 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.

© 2018 Russian Transplant Society. All Rights Reserved. Lung transplantation (LT) for idiopathic pulmonary arterial hypertension (IPAH) now is the only radical treatment of this disease. Aim: to analyze own experience of performing LT in patients with IPAH. Materials and methods. 8 adult IPAH patients, who underwent LT between 2014 and october 2018, were included. In 7 of 8 patients undergoing bilateral lung transplantation on intraoperative venoarterial extracorporeal membrane oxygenation (VA ECMO) with prolongation into the postoperative period. Results. VA ECMO support was prolonged into postoperative period 6 and 7 days respectively in 2 (25,0%) patients and 3 days in 6 (75,0%) patients. Hospital mortality in IPAH patients was 1. Conclusions. Own experience demonstrates that LT is an effective method of treatment in patients with IPAH. Hospital, 1- and 3-year survival rates for the patient collective were 87.5, 75.0 and 75.0% respectively.

© 2018 National Research University Higher School of Economics. All Rights Reserved. The aim: to analyze the waiting list for heart transplantation from 2010 to 2017 and to characterize recipients with chronic heart failure III–IV NYHA Class. Methods. The study comprised 997 patients (139 [14%] females and 858 [86%] males) included in the waiting list for heart transplantation the period from January 2010 to December 2017. The average age of patients on the waiting list was 49.0 ± 12.0 (from 10 to 78 years). Before making a decision on inclusion in the waiting list, all patients underwent clinical and instrumental examination, including general clinical studies, echocardiography, measurement of central hemodynamic parameters using a Swan–Gans catheter, computer and/or magnetic resonance imaging of the chest, abdominal and brain. Results. Heart transplantation was performed on 728 patients (99 females – 13.6% and 629 males – 86.4%) including 18 children aged 12 to 17 years (14.18 ± 2.07 years). Mortality in the waiting list in 2010 was 16.1%, compared with 3.2% in 2017.

© 2018 Russian Transplant Society. All Rights Reserved. Aim. To study the effect of ACE inhibitors (ACEI) in heart recipients on the prognosis and myocardial remodeling. Materials and methods. Three hundred and eighty-six patients who received orthotopic heart transplantation (HT) were consequently enrolled to the study from February 2009 to November 2016. Results. Thirty days after the HT, ACEIs were assigned to 141 recipients. Arterial hypertension was diagnosed in all cardiac recipients who received ACEI and among 48 patients (19.5%) from non-ACEI group. Patients receiving ACEI had significantly better event-free survival than control group (p = 0.045) during the follow-up for 1361,6 ± 36,9 days. Left ventricle (LV) end-diastolic dimension did not change over the time in both groups, whereas LV posterior wall thickness in non-ACEI group significantly increased from 1.35 ± 0.03 cm to 1,23 ± 0.05 cm (p < 0.05). Conclusion. Cardiac recipients who received ACE inhibitors had better survival and less transplant left ventricle progression, that could reflect beneficial effects of renin-aldosterone-angiotensin system inhibition after heart transplantation.

© 2018 Russian Transplant Society. All rights reserved. Tricuspid valve insufficiency is one of the most common variants of valve pathology of transplanted heart. The review is sanctified to the analysis of modern looks to on causes, potentially influencing on development of tricuspid valve insufficiency of transplanted heart, and also methods sent to declines risk his development.

© 2018 Russian Transplant Society. All rights reserved. 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.

© 2018 Stavropol State Medical University. All rights reserved. The review presents data on the relationship of intestinal dysbiosis with irritable bowel syndrome (IBS) and the possibility of modulation of the intestinal microbiota (MB) in IBS therapy. It is shown that imbalance of MB caused by the influence of diet, antibiotics or other exogenous factors leads to gastrointestinal disorders, in particular, to the development of IBS. The data are presented that the modulation of MB with the help of prebiotics, antibiotics, probiotics and fecal microbiota transplantation has a positive therapeutic effect in patients with IBS.

Первичные миодистрофии это гетерогенная группа наследственных нервно-мышечных заболеваний, характеризующихся прогрессирующей слабостью и дегенерацией скелетных мышц. В общей когорте взрослых больных с синдромом дилатационной кардиомиопатии (ДКМП) доля пациентов с первичными миопатиями невелика (в нашем регистре из 220 больных с синдромом ДКМП 3,6%), но они отличаются молодым возрастом и нередко тяжелым прогрессирующим течением заболевания. Генофенотипические корреляции, принципы стратификации риска и ведения таких больных четко не определены, данные об успешной трансплантации сердца у них и ее месте в их лечении немногочисленны. Цель проанализировать особенности течения кардиомиопатии в рамках генетически верифицированных и неуточненных первичных миопатий и роль трансплантации сердца в ее лечении. Материал и методы. В исследование включены 9 больных, 5 мужчин и 4 женщины, средний возраст 31,4+13,8 года (от 16 до 63) с сочетанием кардиомиопатии и скелетной миодистрофии. Обследование пациентов включало общий осмотр, определение уровня КФК в крови, антител к различным антигенам сердца методом непрямого ИФА, ДНК кардиотропных вирусов в крови и миокарде методом ПЦР, ЭхоКГ, суточное мониторирование ЭКГ по Холтеру; дополнительно проведены МСКТ сердца, МРТ, игольчатая электронейромиография, консультация невролога, эндомиокардиальная биопсия правого желудочка, исследование эксплантированного сердца, биопсия скелетной мышцы, аутопсия. Медико-генетическое консультирование было проведено всем больным. Генетическое исследование включало поиск мутаций в 57 генах методами прямого секвенирования по Сенгеру и полупроводникового секвенирования на платформе PGM IonTorrent. Результаты. Мутации в генах LMNA, EMD и DES были выявлены у 7 из 9 пациентов. Симптомы скелетной миопатии в большинстве случаев предшествовали появлению первых проявлений КМП. Практически у всех пациентов первыми симптомами поражения сердца были нарушения ритма и проводимости. У 2/3 больных при ЭхоКГ выявлена развернутая картина ДКМП. Значимое повышение титра антител к различным антигенам сердца выявлено у 5 больных. 3 больных умерли, еще 3 успешно выполнена трансплантация сердца. Живы без трансплантации только 3 женщины, 2 из них имплантированы устройства (электрокардиостимулятор и CRT-D). Обсуждение. Патогенные мутации верифицированы у 77,8% больных в 3 генах: LMNA, EMD и DES; трансплантация сердца успешно выполнена в каждой подгруппе. Присоединение миокардита является несомненным фактором ухудшения прогноза при различных миопатиях. Выводы. Кардиомиопатии в рамках миопатий имеют неуклонно прогрессирующее течение и плохой прогноз. К 9 мес суммарной конечной точки «смерть + трансплантация» достигли 66,7% больных, летальность составила 33,3%. Женский пол оказался благоприятным прогностическим признаком.

Первичные миодистрофии это гетерогенная группа наследственных нервно-мышечных заболеваний, характеризующихся прогрессирующей слабостью и дегенерацией скелетных мышц. В общей когорте взрослых больных с синдромом дилатационной кардиомиопатии (ДКМП) доля пациентов с первичными миопатиями невелика (в нашем регистре из 220 больных с синдромом ДКМП 3,6%), но они отличаются молодым возрастом и нередко тяжелым прогрессирующим течением заболевания. Генофенотипические корреляции, принципы стратификации риска и ведения таких больных четко не определены, данные об успешной трансплантации сердца у них и ее месте в их лечении немногочисленны. Цель проанализировать особенности течения кардиомиопатии в рамках генетически верифицированных и неуточненных первичных миопатий и роль трансплантации сердца в ее лечении. Материал и методы. В исследование включены 9 больных, 5 мужчин и 4 женщины, средний возраст 31,4+13,8 года (от 16 до 63) с сочетанием кардиомиопатии и скелетной миодистрофии. Обследование пациентов включало общий осмотр, определение уровня КФК в крови, антител к различным антигенам сердца методом непрямого ИФА, ДНК кардиотропных вирусов в крови и миокарде методом ПЦР, ЭхоКГ, суточное мониторирование ЭКГ по Холтеру; дополнительно проведены МСКТ сердца, МРТ, игольчатая электронейромиография, консультация невролога, эндомиокардиальная биопсия правого желудочка, исследование эксплантированного сердца, биопсия скелетной мышцы, аутопсия. Медико-генетическое консультирование было проведено всем больным. Генетическое исследование включало поиск мутаций в 57 генах методами прямого секвенирования по Сенгеру и полупроводникового секвенирования на платформе PGM IonTorrent. Результаты. Мутации в генах LMNA, EMD и DES были выявлены у 7 из 9 пациентов. Симптомы скелетной миопатии в большинстве случаев предшествовали появлению первых проявлений КМП. Практически у всех пациентов первыми симптомами поражения сердца были нарушения ритма и проводимости. У 2/3 больных при ЭхоКГ выявлена развернутая картина ДКМП. Значимое повышение титра антител к различным антигенам сердца выявлено у 5 больных. 3 больных умерли, еще 3 успешно выполнена трансплантация сердца. Живы без трансплантации только 3 женщины, 2 из них имплантированы устройства (электрокардиостимулятор и CRT-D). Обсуждение. Патогенные мутации верифицированы у 77,8% больных в 3 генах: LMNA, EMD и DES; трансплантация сердца успешно выполнена в каждой подгруппе. Присоединение миокардита является несомненным фактором ухудшения прогноза при различных миопатиях. Выводы. Кардиомиопатии в рамках миопатий имеют неуклонно прогрессирующее течение и плохой прогноз. К 9 мес суммарной конечной точки «смерть + трансплантация» достигли 66,7% больных, летальность составила 33,3%. Женский пол оказался благоприятным прогностическим признаком.