Rossiiskii zhurnal gastroenterologii gepatologii koloproktologii最新文献

{"title":"Evaluation of Small Intestinal Permeability in Patients with Overlap Syndrome (Autoimmune Hepatitis/Primary Biliary Cholangitis)","authors":"D. Akberova, A. Odintcova, D. Abdulganieva","doi":"10.22416/1382-4376-2023-33-1-51-58","DOIUrl":"https://doi.org/10.22416/1382-4376-2023-33-1-51-58","url":null,"abstract":"Аim: to evaluate the state of small intestine permeability by the “double sugar test” in patients with overlap syndrome (autoimmune hepatitis / primary biliary cholangitis (AIH / PBC)).Materials and methods. Prospectively, 56 people were included in the study. Of these, 26 were diagnosed with AIH/PBC, 30 were in the control group. The diagnosis was made in accordance with the current recommendations. The average age of patients was 49.7 ± 13.8 years, healthy volunteers — 48.6 ± 9.2 years. The determination of the permeability of the small intestine was carried out by a “double sugar test” (the ratio of lactulose/mannitol in urine), using the method of high-performance liquid chromatography — mass spectrometry.Results. In patients with AIH/PBC, an increase in intestinal permeability was found — 0.20 [0.09; 0.30] (p < 0.001) compared with the control group 0.01 [0.01; 0.02]. We divided patients at the stage of liver damage. An increased small intestinal permeability was revealed: hepatitis stage — 0.19 [0.13; 0.30] (p < 0.001), liver cirrhosis stage — 0.18 [0.09; 0.30] (p < 0.05) compared with the control group. In the early stages of disease (1 month from the onset of the disease) had an increased lactulose/mannitol ratio — 0.13 [0.05; 0.26] (p < 0.001) compared to the control group. In the presence of portal hypertension (PH), small intestinal permeability was increased — 0.18 [0.09; 0.30] (p < 0.001) compared with the control group.Conclusions. An increase in small bowel permeability was found in patients with overlapping syndrome. All patients had increased intestinal permeability (regardless of the presence of extrahepatic manifestations).","PeriodicalId":33798,"journal":{"name":"Rossiiskii zhurnal gastroenterologii gepatologii koloproktologii","volume":"91 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-05-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79594022","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Multimodal Approach to Analgesia in Anorectal Surgery","authors":"T. Garmanova, D. Markaryan, E. Kazachenko, A. Lukianov, M. A. Kazachenko, M. Agapov","doi":"10.22416/1382-4376-2023-33-1-15-23","DOIUrl":"https://doi.org/10.22416/1382-4376-2023-33-1-15-23","url":null,"abstract":"Аim: Evaluation of methods for pain prevention and pain reduction after anorectal surgery at all stages of treatment based on publications found in available databases.Key points. Most anorectal diseases are benign and do not affect life expectancy, meanwhile about 40 % of the population suffer from it. Mainly anorectal disorders affect middle-aged people reducing their life quality. At the same time many articles aimed at studying only postoperative anesthesia. 2 researchers independently searched for articles published in Medline, Scopus, Cochrane, Web of Science, E-library databases using keywords. A total of 54 publications were included. A multimodal approach should include the pain prevention during pre-, intra- and postoperative periods. Acetaminophen is recommended for pain reduction according to the dosing protocols. Acetaminophen combined with other systemic analgesics is advisable for patients with severe pain. The opioids use is justified only for moderate-severe postoperative pain. Metronidazole and lidocaine with nitroglycerin ointment/ nifedipine/сorticosteroids effectiveness has been proven for local postoperative anesthesia. Prophylactic use of antibiotics and bowel preparation do not reduce postoperative pain. Stool softeners are recommended for reducing postoperative pain severity. Additional local anesthesia such as nerve blockade or infiltration anesthesia is recommended for all patients. Modern minimally invasive treatment methods of hemorrhoid are associated with less severe postoperative pain.Conclusion. The recommended scheme of multimodal anesthesia for patients after anorectal surgery is shown on Fig.1. Further studies are needed to evaluate preoperative anesthesia effectiveness, the feasibility of the perioperative flavonoids use, as well as comparisons of various minimally invasive treatment methods of anorectal diseases.","PeriodicalId":33798,"journal":{"name":"Rossiiskii zhurnal gastroenterologii gepatologii koloproktologii","volume":"26 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-05-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89785262","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Wilson – Konovalov Disease: Clinical Cases with Different Manifestations and Outcomes","authors":"O. Samodova, G. Smirnova, E. Krieger","doi":"10.22416/1382-4376-2023-33-1-77-83","DOIUrl":"https://doi.org/10.22416/1382-4376-2023-33-1-77-83","url":null,"abstract":"Аim: to describe clinical cases of Wilson – Konovalov disease in pediatric patients.Key points. The first clinical case demonstrates the manifestation of Wilson – Konovalov disease with unexplained mild elevation of aminotransferases at the age of 6 years. Despite the persistent hyperenzymemia, no additional laboratory tests were prescribed. At the age of 10, the patient showed signs of liver failure and neurological symptoms. Laboratory and instrumental examination enabled to diagnose Wilson – Konovalov disease at the stage of liver cirrhosis. The diagnosis was confirmed at the Federal medical center. The diagnosis was delayed and made 4 years after the hyperenzymemia was first revealed. The relief of clinical signs was observed after the orthotopic transplantation of the right lobe of the liver from a living related donor. The patient was under surveillance for 10 years after the transplantation.The second clinical case shows another variant of the course of the Wilson – Konovalov disease manifested as an acute hepatitis. To clarify the etiology of the disease, the patient was hospitalized. In 2 weeks, the patient developed symptoms of acute liver failure, progressive hemorrhagic syndrome, acute hepatic encephalopathy. Death from fulminant hepatitis occurred in 3 weeks after the disease onset after the disease onset.Conclusion. Healthcare workers should be aware of clinical signs of Wilson – Konovalov disease. Screening for the disease is recommended for children with an unexplained increase of liver transaminases, acute liver failure, chronic hepatitis and liver cirrhosis.","PeriodicalId":33798,"journal":{"name":"Rossiiskii zhurnal gastroenterologii gepatologii koloproktologii","volume":"16 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-05-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84951728","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Changes in the Structural and Functional Albumin Properties in Patients with Decompensated Liver Cirrhosis","authors":"A. Turkina, M. Maevskaya, M. Zharkova, V. Ivashkin","doi":"10.22416/1382-4376-2023-33-1-40-50","DOIUrl":"https://doi.org/10.22416/1382-4376-2023-33-1-40-50","url":null,"abstract":"Аim: to conduct a comparative analysis of serum albumin's structural and functional properties in decompensated cirrhotic patients by means of spin prob EPR spectroscopy.Materials and methods. The main study group included 70 patients with decompensated liver cirrhosis and ascites. The control group consisted of 12 healthy volunteers, comparable in gender and age, without liver diseases. To assess the structural and functional ability, serum albumin was analyzed by EPR spectroscopy.Results. Albumin levels within reference intervals were found in 37 patients (59.8 %). The native albumin index decrease in cirrhotic patients as the disease progressed with the lowest values in the Child – Pugh C group (p < 0.001). The binding efficiency of albumin decreased in accordance with the severity of cirrhosis with minimal albumin binding capacity in the Child – Pugh C (Me = 25.43 %; n = 30; p < 0.001). The transport activity of RTQ albumin decreased in patients with decompensated cirrhosis, the lowest transport ability was observed in the Child – Pugh C group (Me = 26.09 %). In patients with decompensated disease the detoxification potential was significantly reduced: Child – Pugh B — Me = 44.03 %; Child – Pugh C — Me =17.16 %. Despite the normal values of serum albumin in 72.5% of patients with cirrhosis B and in 26.7% in the cirrhosis C group, only 12.3% in the cirrhosis B group had normal albumin function and in cirrhosis C nо patients had normal albumin function.Conclusion. There were not only serum concentration depletion in cirrhotic patients, but also albumin physiological non-oncotic properties were violated. The severity of these changes increased with the progression of cirrhosis. Our data allow us to raise the question of the need to use the EPR test to determine indications for albumin replacement therapy in patients with cirrhosis and the presence of ascites, even at normal values of its serum concentration.","PeriodicalId":33798,"journal":{"name":"Rossiiskii zhurnal gastroenterologii gepatologii koloproktologii","volume":"156 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73647872","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Modern Approaches to the Diagnosis and treatment of Clostridioides difficile (C. difficile)-associated Disease in Adults (literature Review and Expert Council Resolution)","authors":"V. Ivashkin, I. Maev, D. Andreev, O. Goloshchapov, A. Derinov, O. Zolnikova, K. Ivashkin, O. Kiseleva, A. Kiryukhin, O. Lyashenko, E. Poluektova, A. S. Tertychnyy, A. S. Trukhmanov, A. Ulyanin, Arkadiy A. Sheptulin, O. Shifrin, Goloshchapov Andreev D.N., Derinov O.V., Zolnikova O.Yu, Kiseleva O.Yu, В.Т. Ивашкин, И. В. Маев, Д. Н. Андреев, О.В. Голощапов, А.А. Деринов, О. Ю. Зольникова, К.В. Ивашкин, О.Ю. Киселёва, А.П. Кирюхин, О.С. Ляшенко, Е.А. Полуэктова, А. С. Тертычный, А. С. Трухманов, А.И. Ульянин, А.А. Шептулин, О.С. Шифрин","doi":"10.22416/1382-4376-2023-33-2-19-33","DOIUrl":"https://doi.org/10.22416/1382-4376-2023-33-2-19-33","url":null,"abstract":"Aim: to review the modern approaches to the diagnosis and treatment of C. difficile-associated disease in adults and present the resolution of the Expert Council held on March 25, 2023 in Moscow.General provisions. C. difficile is the most important nosocomial pathogen which spores are also commonly found in the environment. Microbiota impairment, primarily due to the use of antibacterial drugs, is a key stage in the development of C. difficile-associated disease. A search for an infection should be carried out only in patients with diarrhea, and it is advisable to use at least 2 laboratory methods. The drug of choice for first-line treatment is vancomycin. If drug treatment is ineffective or the patient has recurrent clostridial infection, fecal microbiota transplantation should be considered. The probiotic strain Saccharomyces boulardii CNCM I-745 has a direct inhibitory effect on C. difficile toxin A, promotes normalization of the intestinal microbiota composition, and decreases the inflammatory reaction in colonic mucosa colonized with a toxigenic strain of C. difficile.Conclusions. Addition of the probiotic strain Saccharomyces boulardii CNCM I-745 to antibacterial therapy promotes both primary and secondary prevention of C. difficile-associated disease.","PeriodicalId":33798,"journal":{"name":"Rossiiskii zhurnal gastroenterologii gepatologii koloproktologii","volume":"36 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79075793","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Duodenal Eosinophilia in Functional Dyspepsia","authors":"Alexandra S.Silaeva, E. Bueverova, Y. Shulpekova","doi":"10.22416/1382-4376-2023-33-2-87-94","DOIUrl":"https://doi.org/10.22416/1382-4376-2023-33-2-87-94","url":null,"abstract":"Aim: to present observation of a patient diagnosed with functional dyspepsia based on current guidelines, and having increased eosinophil counts in the biopsy specimen of duodenal mucosa. To consider possible causes of duodenal eosinophilia in the light of present-day concepts.Highlights. Patient K., 40 years old, complained of dyspeptic phenomena, the first appearance of which she had noted at the age of 18. The patient noted poor tolerance to canned and fermented foods, which provoked an increase in dyspepsia and sometimes caused watery diarrhea. The examination excluded “symptoms of concern”. Successful antihelicobacter eradication therapy was carried out. Morphological examination of the stomach showed phenomena of mild chronic inflammation without intestinal metaplasia or glandular atrophy. A biopsy of the mucosa of the descending part of the duodenum showed a moderate increase in the levels of mononuclears and eosinophils in its lamina propria without penetration into the epithelium of the villi or formation of clusters. The patient suffers from pollinosis; sensitization to birch pollen was diagnosed by a skin prick test. However, she has no oral allergy symptoms, which does not allow linking duodenal eosinophilia to food allergy. Based on current guidelines, the patient was diagnosed with functional dyspepsia. In addition to dietary restrictions, treatment courses with a proton pump inhibitor, itopride, and S-methylmethionine sulfonium chloride, which has an antihistamine effect, were recommended for periods of worsening dyspepsia.Conclusion. The clinical significance of duodenal eosinophilia and local histamine production in patients with a clinical diagnosis of functional dyspepsia deserves special attention. Triggering factors provoking the worsening of symptoms should be analyzed; in particular, a food diary and exclusion of food allergies are recommended. Histamine-neutralizing drugs may play a role in the treatment of FD with duodenal eosinophilia in the future.","PeriodicalId":33798,"journal":{"name":"Rossiiskii zhurnal gastroenterologii gepatologii koloproktologii","volume":"7 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83154142","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Eating Habits, Anxiety and Depression in Patients with Irritable Bowel Syndrome: Clinical and Laboratory Comparisons","authors":"O. Gaus, M. Livzan, О.В. Гаус, М.А. Ливзан","doi":"10.22416/1382-4376-2023-33-2-34-44","DOIUrl":"https://doi.org/10.22416/1382-4376-2023-33-2-34-44","url":null,"abstract":"Aim: to assess the level of stress hormones (cortisol in saliva), neurotransmitters (serotonin in blood serum, dopamine in blood plasma) in relation to eating habits, anxiety and depression levels in patients with IBS.Materials and methods. An open cohort prospective study was conducted with the inclusion of 263 patients with an established diagnosis of IBS, among them 189 (71.9 %) women and 74 (28.1 %) men. The average age of patients with IBS was 29 [25; 35] years. The control group included 40 healthy volunteers. All individuals included in the study were assessed for diet and eating habits using the WHO CINDI program questionnaire, “Information on Nutrition and Eating Behavior”, the severity of anxiety and depression according to the HADS questionnaire, the level of specific anxiety in relation to gastrointestinal symptoms according to the VSI questionnaire, quality of life according to the IBS-QoL questionnaire. In addition, the enzyme immunoassay method was used to assess the levels of cortisol in the morning and evening portions of saliva, serotonin in the blood serum and dopamine in the blood plasma.Results. Among patients with IBS there is a statistically significantly higher level of cortisol in the morning and evening portions of saliva (U = 19.5, p < 0.001 and U = 111.5, p < 0.001, respectively), serotonin in blood serum (U = 269.0, p = 0.042) and lower plasma dopamine levels (U = 93.5, p = 0.0002) compared with controls. The mean salivary cortisol level among patients with IBS was 45.39 [29.86; 70.10] ng/ml in the morning and 19.21 [13.98; 23.50] ng/ml in the evening, while in the group of healthy individuals it was 19.0 [16.5; 21.7] and 9.7 [8.5; 10.5] ng/ml, respectively. The average content of serotonin in blood serum in patients with IBS was 188.78 [150.41; 230.32] ng/ml, among healthy individuals — 142.80 [130.52; 154.15] ng/ml. The average content of dopamine in blood plasma in patients with IBS was 28.83 [20.08; 41.54] ng/ml, in healthy individuals — 58.20 [48.15; 66.62] ng/ml.Conclusion. In patients with IBS the secretion of the stress hormone (cortisol) and neurotransmitters (serotonin, dopamine) is closely related to the nature of nutrition, the level of anxiety and depression, and is also associated with the clinical variant and severity of the course of the disease.","PeriodicalId":33798,"journal":{"name":"Rossiiskii zhurnal gastroenterologii gepatologii koloproktologii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82934138","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cystic Form of Duodenal Dystrophy (Clinical Case)","authors":"D. E. Okonskaya, Khachik H. Ayvazyan, Yuliia A. Stepanova, A. Zhao, E. A. Sokolova, Д.Е. Оконская, Х.А. Айвазян, Ю. А. Степанова, А.В. Чжао, Евгения Александровна Соколова","doi":"10.22416/1382-4376-2023-33-2-95-104","DOIUrl":"https://doi.org/10.22416/1382-4376-2023-33-2-95-104","url":null,"abstract":"Aim: the reason for the publication was the rare occurrence, as well as the non-specificity of symptoms of the cystic form of duodenal dystrophy. The listed features of this disease lead to difficulties in its differential diagnosis and the choice of the optimal method of treatment.General statements. The report is devoted to the description of a case of successful treatment of a cystic form of duodenal dystrophy — a chronic inflammation of the pancreatic tissue, ectopic in the wall of the duodenum. A 47-year-old patient was admitted to the clinic with complaints of persistent abdominal pain, periodic vomiting, general weakness, weight loss of 20 kg in three months. With the help of computed tomography, the diagnosis was established, the tumor process was rejected, and chronic pancreatitis was detected in the orthotopic pancreas. Due to the presence of changes in the main pancreas, the patient underwent pancreatoduodenal resection. The features of the operation were pronounced infiltrative changes and pronounced vitreous tissue edema, which made it difficult to mobilize the hepatic flexure of the colon and duodenum.Conclusion. Pancreatoduodenal resection is the optimal surgical intervention for the combination of cystic form of duodenal dystrophy with sub-/decompensated duodenal stenosis.","PeriodicalId":33798,"journal":{"name":"Rossiiskii zhurnal gastroenterologii gepatologii koloproktologii","volume":"232 1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72751535","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Phenotypes of Non-Alcoholic Fatty Liver Disease in Different Regions of the Russian Federation, Diagnostic and Therapeutic Approach in Clinical Practice","authors":"V. Ivashkin, M. Zharkova, Natalya V. Korochanskaya, I. Khlynov, Y. Uspensky, В.Т. Ивашкин, М. С. Жаркова, Н.В. Корочанская, И.Б. Хлынов, Ю.П. Успенский","doi":"10.22416/1382-4376-2023-33-2-7-18","DOIUrl":"https://doi.org/10.22416/1382-4376-2023-33-2-7-18","url":null,"abstract":"Aim: to present data on the prevalence of various phenotypes of non-alcoholic fatty liver disease (NAFLD), the features of its diagnosis and treatment in various regions of the Russian Federation following a meeting of chief gastroenterologists.Key points. Non-alcoholic fatty liver disease (NAFLD) holds the leading position among non-infectious liver diseases in the Russian Federation, its prevalence is 37.3 % and does not tend to decrease.To get information on various aspects of NAFLD diagnosis and treatment, opinion of 18 chief external regional gastroenterologists and leading specialists in the field of liver diseases was studied by analyzing the responses to questions specially formulated for them. Information on the situation in the Ural, Privolzhsky, Southern, Central and North-western Federal Districts was obtained. Regional statistics on the prevalence of NAFLD and its certain phenotypes (steatosis, steatohepatitis, cirrhosis), diagnostic instruments and treatment approaches which are used in clinical practice, as well as on the most common associated conditions was presented.Conclusions. The collected information allows to improve both administrative and treatment and diagnostic-related activities in managing patients suffering from this disease.","PeriodicalId":33798,"journal":{"name":"Rossiiskii zhurnal gastroenterologii gepatologii koloproktologii","volume":"23 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75285177","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pancreatic Perivascular Epithelial Cell Tumor (PEComa) with Liver Metastasis: a Case Report and Literature Review","authors":"Y. Stepanova, Kh. A. Ayvazyan, D. Kalinin, O. Zhavoronkova, V. S. Shirokov, A. Zhao, Ю. А. Степанова, Х.А. Айвазян, Д. В. Калинин, О.И. Жаворонкова, Владимир Степанович Широков, А.В. Чжао","doi":"10.22416/1382-4376-2023-33-2-105-119","DOIUrl":"https://doi.org/10.22416/1382-4376-2023-33-2-105-119","url":null,"abstract":"Aim: to present a clinical case of pancreatic PEComa with liver metastases with an emphasis on morphological features, radiology diagnostic methods and features of treatment tactics, allowing practitioners to get an idea about this rare mesenchymal tumors composed of “perivascular epithelioid cells”, and a review of literature data on pancreatic PEComa, including 32 author's cases.General statements. A 22-year-old woman who was surgically treated for pancreatic head PEComa with infiltration of the duct of Wirsung, common bile duct, duodenal wall, focal invasion into the blood and lymphatic vessels, and perineural space infiltration. Synchronously, metastases were detected in both liver lobes, for which she took Everolimus for 6 years under the control of radiology methods with dose adjustment and frequency of administration. At the A.V. Vishnevsky National Medical Research Center of Surgery, the patient underwent right-sided hemihepatectomy and atypical resection of II-III liver segments.Conclusion. To determine clearer criteria for the diagnosis and differential diagnosis of PEComa, to identify criteria for the malignancy of these tumors, to develop treatment tactics and further dynamic monitoring, a set of statistical data of significant group and randomized clinical trials are needed. Pancreatic PEComas in this group are extremely rare and often have a benign course. The presented clinical case demonstrates the most malignant form of this tumor with localization in the pancreas and liver metastases.","PeriodicalId":33798,"journal":{"name":"Rossiiskii zhurnal gastroenterologii gepatologii koloproktologii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86429478","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}