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Background Despite overall striking advances in survival of childhood liver tumors, outcomes remain poor for specific patient segments. We aimed to assess overall survival (OS) of this rare disease and evaluate the generalizability of prognostic variables included in international collaborative systems using, for the first time, harmonized clinical data from two geographically different cohorts (Greece and Moscow). Methods Data for children (0-14 years) with liver tumors were retrieved from two Southern-Eastern European areas (Greece; 2001-2019 and Moscow; 2012-2019). Kaplan-Meier curves were constructed, and OS values were derived from Cox proportional models controlling for study variables. Results A total of 171 newly diagnosed cases (54.4% males) were included. The OS5-year exceeded 80% in patients Conclusions Outcomes of patients with liver tumors registered in two SEE areas were comparable to those reported by major collaborative trials. Ongoing clinical cancer registration could facilitate comparison of outcomes between different study groups in order to shape state of the art of treatment.

To develop a system to define the degree of liver disruption and severity of portal hypertension in children based on the International Classification of Functioning, Disability and Health (ICF).Studied the results of laboratory and instrumental methods 382 children: 267 patients with various liver diseases, including 49 patients who underwent liver transplantation, and 115 children without liver disease.Based on analysis of statistical data obtained were identified 10 indicators, a set of changes which can be used to assess the degree of disruption of the structure of the liver and the severity of portal hypertension: indicators that reflect the severity of fibrosis and cirrhosis of the liver (METAVIR score on a scale at fibroelastometrii, scores are Desmet at morphological study of the liver) and indicators that reflect the severity of portal hypertension (the diameter of the portal vein, splenic vein diameter, the length of the spleen, recanalization of the umbilical vein, esophageal varices, ascites, hydropericardium, hydrothorax). Each of the indicators was assessed on a 5-point system. Number of points reflects the sum of the changes of these parameters. Decrease the number of points on 0-4% (38-40 points) is regarded as a lack of structural failure of the liver and the severity of portal hypertension by 5-24% (30-37 points)--minor violations on 25-49% (20-29 points) -moderation disorders, 50-95% (3-12 points)--severe handicaps, 96-100% (0-2 points)--absolute violation. Studied the dynamics of children with autoimmune hepatitis, Wilson's disease and chronic hepatitis C.The proposed scoring system for assessing the degree of disruption of the structure of the liver and the severity of portal hypertension can be used as an objective criterion of the severity of the pathological process, to estimate the dynamics of defeat against the background of the therapy, determining the prognosis of the disease and as a criterion of the indications for liver transplantation.

Objective : Our aim was to on the basis of determining the degree of violation of the structure and function of the liver establish their relationships and to assess the dynamics of liver disease in its chronic illnesses in children. Methods : With the help of the developed scoring systems were used to assess the degree of liver dysfunction and the degree of disruption of the structure of the liver and the severity of portal hypertension. Results : The results of the diagnostic methods 252 children aged 1 to 17 years (mean age of 11.8±3,5) with Wilson disease (WD), autoimmune hepatitis (AIH), chronic hepatitis C (CHC) were analyzed; 48 patients underwent liver transplantation. In children with WD, AIH and CHC liver function reduced by 41.3±12.9 % to 28.8±12.5% and 19.1±7.8% respectively. Structure of the liver in children with WD, AIH and CHC was disturbed by 25.0±8.1% to 20.4±9.2% and 6.8±4.4% respectively. The function and structure violations of the liver more pronounced in liver cirrhosis. The use of the developed scoring systems to monitor the severity of liver damage in the dynamics and evaluation of the effectiveness of the therapy is demonstrated. The degree of liver dysfunction is directly dependent on the degree of its structure. Abnormal liver function ≥40% and ≥40% of its structure with treatment failure can be used as a criterion of indications for elective liver transplantation with its chronic diseases in children. Conclusion : Developed a point system to determine liver function and a point system to determine disruption of the structure of the liver and the severity of portal hypertension in children can serve as an objective criterion for assessing the severity of liver disease, monitoring their changes in the dynamics with the assessment of the effectiveness of the therapy and making decisions about the need for routine liver transplantation in its chronic illnesses in children.

This article presents the analysis of 24384 endoscopic investigations of the upper gastrointestinal tract in population of Leningrad region with symptoms of dyspepsia during the period 2007-2011.The results revealed erosive esophagitis in 5.4%, peptic strictures of the esophagus--0.2%, the columnar-lining esophagus (Barrett's esophagus)--1.1%, and esophageal adenocarcinoma--0.045%. This data shows a high prevalence of esophageal complications of GERD in a local population of Russians.

The article presents an analysis of results of 24 384 endoscopic examinations of the upper gastrointestinal tract in population of the Leningrad oblast with symptoms of gastric dyspepsia during the period from 2007 to 2011. The detection of the columnar-celled metaplasia was 1.1%, adenocarcinoma of the esophagus--0.045%. Esophageal adenocarcinoma occurred in 3.95% of cases of the column-celled esophagus. Barrett's esophagus was revealed in males more often than in women (56.5% and 54.5% respectively). The peak incidence of esophageal adenocarcinoma in males was at the age from 46 to 60 years (36.4% of patients), in females--from 61 to 75 years (27.3% of patients). Intestinal metaplasia was detected in 72.7% of cases with esophageal adenocarcinoma: The diagnosis of long and short segment of column-celled esophagus revealed no significant difference in the development of esophageal adenocarcinoma.

To analyze the results of repeated reconstructions of choleresis after living related liver fragments transplantation.The study included 268 recipients (145 women and 123 men) aged 5 months - 61 years (mean age 16,11 ± 14,62 years) who underwent liver fragments transplantation in the department of liver transplantation of cad. B.V. Petrovskiy Russian Research Surgery Center from 1997 to 2012. Biliary reconstructions were performed at different terms after transplantation in 37 patients (13.81%). Complications followed repeated reconstructions, the initial conditions for biliary anastomosis formation during transplantation, the results of biliary reconstructions after transplantation were analyzed.In most cases despite the prevailing complications there is a combination of various biliary complications requiring biliary reconstruction. It was found that live rfragment used for transplantation, type of primary reconstruction of choler sis, number of bile ducts orifices of graft and biliary anastomoses do not demonstrate statistically significant effect on the incidence of post-transplant biliary reconstructions (p0.05). Poor prognosis in patients with biliary complications required biliary reconstructionsis determined by the development of graft dysfunction. Early reconstruction before development of liver transplant dysfunction is necessary condition of successful treatment. In the case of graft dysfunction liver retransplantation is unique method of treatment.Цель - проанализировать результаты повторных реконструкций желчеотведения после родственных трансплантаций фрагментов печени. Материал и методы. В исследование включены 268 реципиентов (145 женщин и 123 мужчины) в возрасте от 5 мес до 61 года (средний - 16,11±14,62 года), которым с 1997 по 2012 г. в отделении трансплантации печени Российского научного центра хирургии им. акад. Б.В. Петровского были выполнены трансплантации различных фрагментов печени. В различные сроки после трансплантации у 37 (13,81%) из этих пациентов выполнены билиарные реконструкции. Проанализированы спектр осложнений, послуживших показанием к выполнению повторных реконструкций, исходные условия формирования билиарного анастомоза при трансплантации, результаты выполненных после трансплантации реконструкций желчеотведения. Результаты. Чаще всего, несмотря на наличие превалирующего осложнения, имеет место сочетание различных билиарных осложнений, определяющих показания к выполнению билиарной реконструкции. Установлено, что ни вариант используемого при трансплантации фрагмента печени, ни вид первичной реконструкции желчеотведения, ни количество устьев желчных протоков трансплантата и формируемых билиарных анастомозов не демонстрируют статистически значимого влияния на частоту выполняемых после трансплантации билиарных реконструкций (р0,05). Неблагоприятный прогноз у пациентов с билиарными осложнениями, потребовавшими выполнения билиарных реконструкций, определяется развитием дисфункции трансплантата. Условием благополучного лечения пациентов является раннее выполнение реконструкции, до развития дисфункции трансплантата печени. В случаях ее развития единственным возможным методом лечения является своевременное выполнение ретрансплантации печени.

From 1997 to 2003 transplantation of liver's right lobe from alive related donor was performed in 64 patients (28 men, 36 women) aged from 6 to 61 years (mean age was 22.6 +/- 3.2 years). Body weight of recipients ranged from 18 to 92 Kg (52.7 +/- 2.8 Kg on average). Indications for surgery were following: cirrhosis of liver due to Wilson's disease (34), primary sclerotic cholangitis (6), viral cirrhosis of liver (6), primary biliary cirrhosis (4), Bayler's disease (3), Caroly's disease (3), Budd-Chiary syndrome (3), secondary biliary cirrhosis (2), cirrhosis of liver due to deficient of alpha(1)-antitrypsin (1), hepatocellular carcinoma (1), total nodular transformation of liver (1). Intensive care before transplantation was needed for 68.75% potential recipients. Donors of liver's right lobe were relatives of patients similar to blood group. Age of donors ranged from 18 to 49 years, mean age was 37.9 +/- 1.4 years. Donors underwent right-sided hemihepatectomy, there were no complications. Lethal outcome in recipients was seen in 2 (3.1%) cases and was not associated with function of transplant. Lethal outcome in long-term period after transplantation was seen in 4 (6.5%) cases. All the others patients survived and followed from 1 to 73 months (23.8 +/- 2.4 months on average). Quality of life was good. Transplantation of liver's right lobe from related donor is the independent line in orthotopic transplantation of liver and ensure reliable results.

The use of relative donors in the transplantation of the liver has shown a good performance as an alternative line in its orthotopical grafting. Shortage of donor organs actively stimulates the development of relative transplantation. The main problem of relative hepatic transplantation is the limited capacity of obtaining a required mass of a hepatic graft for obese recipients. To settle this problem, the Russian Surgery Research Center, Russian Academy of Medical Sciences, has developed an original safe procedure for obtaining the right lobe of the liver from an alive relative donor and for implanting it in a recipient. In November 1997 to October 2001, transplantation of the right hepatic lobe from an alive relative donor was made in 23 recipients (10 males and 13 females aged 9 to 55 (mean 22.3 +/- 3.1) years. Their body weight was 24 to 80 (mean 51.4 +/- 3.0) kg. Indications for surgery were as follows: hepatic cirrhosis (HC) at the end stage of the Wilson-Konovalov disease (n = 10), primary sclerotic cholangitis (n = 4), HC of viral etiology (n = 3), Bailer's disease (n = 2), primary biliary HC (n = 2), HC in the presence of alpha 1-antitrypsin deficiency (n = 1), and secondary biliary HC (n = 1). The donors of the right lobe of the liver were recipients' mothers in 9 cases, their fathers in 6 cases, sisters in 2 cases, sons in 1 cases, their daughter, brother, aunt, cousin in 1 case each. The donors' age ranged from 19 to 49 (mean 37.9 +/- 1.4) years. The donors underwent right hemihepatectomy, complications were absent in them. There were early mortality among the recipients. Two patients died in the late postoperative period. The remaining 21 recipients were survivors and followed up for 1 to 48 (mean 14.9 +/- 2.9) months. Their life quality was good. The use of the right lobe of the liver from an alive relative donor is the optimum alternative to transplantation of the cadaverous liver and partially compensates the shortage of donor organs for children, adolescents, and adults.

The unique in Russia experience of 35 liver transplantations since 1990 has revealed benefits of the method and quite encouraging results with 81% survival of the recipients. But the extreme shortage of cadaveric donor organs followed by more than 60 per cent mortality in waiting list and absence of any real facilities to improve the situation dictate the living related donation to become a considerable alternative. Totally 12 living related liver transplant procedures were performed with no morbidity in donors. In 7 donors the routine surgical technique of harvesting were used to get left lateral segment in five and whole left lobe in two cases. The unique twin-to-twin liver left lobe donation with no consequent immunosuppression was observed in this series. New special technique of living donor surgery was successfully developed for liver right lobe harvesting. The living related liver right lobe replacement was used in 5 cases and appeared to become a real compensation of donor organs shortage for elder children with body weight more than 20 kg and adults.

The choice of anesthesia for a high risk operation, orthotopic transplantation of the liver (OTL), is discussed. The authors propose a protocol of anesthesia for OTL. For induction anesthesia, intravenous drugs should be preferred; the liver-free stage of the operation is carried out under anesthesia with a closed isoflurane contour, and the initial metabolic disorders of patients are corrected. When the bloodflow is let in the transplanted organ, intravenous drugs (ketamine, phentanyl, and benzodiazepines) should be administered in order to reduce the vasodilating effect of isoflurane and vasopressors for preventing relative hypovolemia. At the final stage of anesthesia, isoflurane is used. Before transporting the patient into intensive care ward, phentanyl in a dose of 1.5-3 micrograms/kg was injected, because of rapid elimination of isoflurane and awakening of the patient. This protocol maintained the hemodynamics and the major metabolic parameters at the optimal level.

Orthotopic liver transplantation is the only method of choice for many severe liver diseases with poor prognosis. The development of liver transplantation programmes is based on medical science achievements and high technology in surgery, anesthesiology and perfusiology. The experience of the Surgery Research Center, Russian Academy of Medical Sciences, made it possible to obtain positive results of liver transplantation and posttransplantation management. This in turn provided longer survival for 80% of recipients. At the same time the mortality of potential recipients was 60.4%, which is due to the greater shortage of donor organs and which is the main problem in the development of organ grafting in Russia. The fact that there is a high proportion of children who are recipients for the donor's liver requires that liver transplantation from living related donors should be developed.

Experience with 32 curative major and extended liver resections performed in 30 patients with different disseminated focal diseases. The scope of resections was 50 to 85% of the liver parenchyma. Owing to the developed criteria for resectability and surgical techniques of operations, there was no hepatic failure due to the small mass of the liver stump. There were no intraoperative deaths. Postoperative complications were observed in 15.6% of cases. Early postoperative mortality was 6.25%, the deaths were caused by fulminant pleuropneumonia and acute cerebrovascular circulatory disorder. Late mortality (at months 2.5 to 28 (mean 17.6 +/- 4.0 months) was 18.75% and it was due to the generalization of cancer diseases. After extensive hepatic resections, survival was observed in 73.3% of cases with the maximum time of 63 months. The authors' own guidelines for extensive and maximum large resections of the liver are presented in the paper.

From 1990 to 1996, 178 patients with severe diffuse and advanced focal liver diseases with grave prognosis were observed for indications to orthotopic liver transplantation (OLT). According to the revealed indications and contraindications, 95 patients were enrolled for the procedure. Eighteen patients underwent 19 OLTs, including 1 urgent retransplantation and 3 living related graftings. Surgical techniques were conventional; with these, intraoperative mortality and biliary complications could be avoided. Long-term survival (6 to 68 months) was achieved in 8 recipients, including in those after liver retransplantation. Double or triple suppressive regimens were used. These included corticosteroids, cyclosporin A, azathioprine, antithymocytic globulin. The life quality of recipients promoted restoration of their working capacities. Two recipients gave birth to healthy babies.

Retrospective analysis of surgical treatment of 153 patients with traumatic damage to the pancreas is discussed. The injury was closed in 67.3% and open in 32.7% of cases. All cases were grouped in 4 degrees of severity: 1st degree--contusion of the gland without damage to the capsule (22.2%); 2nd degree--rupture of the gland without injury to the pancreatic duct (34.6%); 3rd degree--rupture of the gland with damage to the duct; 4th degree--combined pancreatoduodenal injury (15.7%). The therapeutic tactics was determined by the degree of injury inflicted to the pancreas. In 1st degree operation on the pancreas was not needed. In 2nd and 3rd degrees "abdominization" with drainage was the operation of choice. Distal resection was expedient only in crushing of the organ. The tactics in pancreatoduodenal injuries depended on the character of the duodenal wound. Thirty-nine (25.5%) patients died, 24 (61.5%) of them died in the first two postoperative days from shock. Pancreatitis was the prevailing postoperative complication, it occurred in 41 (31.8%) patients. All patients with injuries to the pancreas should be managed as cases of potential pancreatitis.