Terapevticheskii Arkhiv最新文献

{"title":"[Results of a prospective observational study of imiglucerase biosimilar in adults with type I Gaucher disease].","authors":"E A Lukina, R V Ponomarev, G N Salogub, V A Bezrukikh, E V Saifullina, I L Davydkin, S A Volkova, T V Shelekhova, M V Kosinova, E V Vasiliev, E G Kirillova, O E Danilova, R K Khairetdinov, O A Markova, E V Zuev, A I Borozinets","doi":"10.26442/00403660.2025.02.203194","DOIUrl":"https://doi.org/10.26442/00403660.2025.02.203194","url":null,"abstract":"<p><strong>Aim: </strong>To collect and analyze real-world data on long-term enzyme replacement therapy with Glurazyme® in patients with type I Gaucher disease (GD).</p><p><strong>Materials and methods: </strong>The study included 31 patients with type I GD (21 patients with intact spleen and 10 patients with a history of splenectomy) over 18 years. Efficacy was assessed by the change in hemoglobin level (primary endpoint), platelet count, spleen and liver volumes, prevalence of bone marrow infiltration, and the number of patients with quiet hip disease according to magnetic resonance imaging (secondary endpoints). Safety was assessed by the incidence of treatment-related adverse events and serious adverse events, as well as by the incidence of production of imiglucerase binding and neutralizing antibodies (IgG, IgE). The mean follow-up duration was 54 weeks.</p><p><strong>Results: </strong>The study did not show a statistically significant change in hemoglobin levels, platelet count, or spleen and liver volumes. There was an upward tendency in platelet count in the overall group (<i>p</i>=0.1) and patients after splenectomy (<i>p</i>=0.08). The prevalence of specific bone marrow infiltration and the number of patients with quiet hip disease remained unchanged. During the safety analysis, one adverse reaction, a mild increase in alanine aminotransferase, was reported, which resolved spontaneously by the end of the study. The immunogenicity analysis showed the initial presence of anti-drug antibodies (ADAs) in 5 (16.7%) of the 30 examined patients. At the end of the study, ADAs were detected in only 3 (10%) patients. The detected ADAs had a low titer and no neutralizing activity, and they did not affect the treatment effectiveness.</p><p><strong>Conclusion: </strong>Long-term therapy with the imiglucerase biosimilar was associated with a stable course of GD without progression. It was characterized by a good safety profile and low immunogenicity.</p>","PeriodicalId":22209,"journal":{"name":"Terapevticheskii Arkhiv","volume":"97 2","pages":"169-175"},"PeriodicalIF":0.3,"publicationDate":"2025-03-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144040104","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[The role of neurohormonal regulatory factors in the violation of the motor evacuation function of the duodenum in chronic duodenal insufficiency].","authors":"Y M Vakhrushev, M S Busygina","doi":"10.26442/00403660.2025.02.203121","DOIUrl":"https://doi.org/10.26442/00403660.2025.02.203121","url":null,"abstract":"<p><strong>Aim: </strong>To study the interaction of hormonal factors and indicators of vegetative homeostasis in violation of the motor evacuation function of the duodenum in chronic duodenal insufficiency (CDI).</p><p><strong>Materials and methods: </strong>70 patients with CDI were under observation. The average age of patients was 35.7±11.4 years, women were 32 (45.7%), men - 38 (54.3%). The control group included 31 healthy individuals [average age 39.1±12.3 years; there were 15 women (48.3%), 16 men (51.7%)]. Cardiorhythmographic examination and analysis of heart rate variability with the \"Varicard 2.51\" complex were used to study the ANS. To study the intramural nervous apparatus of the duodenum, a biopsy piece of its wall from the lower horizontal section was examined. Electrochemiluminescence analysis made it possible to determine the content of gastrin, insulin, cortisol, and somatostatin in the blood. To diagnose motor disorders of the gastroduodenal zone, peripheral electrogastroenterography was performed using the gastroenteromonitor GEM-01 \"Gastroscan-GEM\".</p><p><strong>Results: </strong>In patients with CDI, in addition to changes in the clinical and electrophysiological picture, changes in the vegetative status were revealed. This condition correlates with the following electrophysiological signs in CDI - hypomotor duodenum leads to a greater increase in the ratio <i>P_i/P_i</i>_{+ 1} stomach / duodenum after food stimulation (17.43±2.46) in this group of patients (<i>p</i>=0.000). The study of the basal hormone content in CDI showed a significant increase in gastrin, cortisol and a decrease in insulin and somatostatin in comparison with the control group. The relationship between them and vegetative-electrophysiological indicators is revealed.</p><p><strong>Conclusion: </strong>Comprehensive studies of myoelectric activity, the functional state of the autonomic nervous system and hormonal regulatory factors, followed by the analysis of multidimensional statistics data, revealed new pathophysiological patterns in duodenal motility disorders in CDI.</p>","PeriodicalId":22209,"journal":{"name":"Terapevticheskii Arkhiv","volume":"97 2","pages":"109-114"},"PeriodicalIF":0.3,"publicationDate":"2025-03-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144046983","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Radiation-induced intestinal injury].","authors":"A I Parfenov, L K Indejkina, E A Sabelnikova, A V Leontiev, A A Makarova","doi":"10.26442/00403660.2025.02.203116","DOIUrl":"https://doi.org/10.26442/00403660.2025.02.203116","url":null,"abstract":"<p><p>This article addresses theoretical and clinical aspects of radiation-induced intestinal injuries, which complicate radiation therapy for malignant neoplasms of the abdominal and pelvic organs. Many clinical aspects of this issue remain unknown due to the lack of awareness among doctors and patients. Further study of radiation-induced intestinal injuries and the development of personalized approaches to their prevention and treatment represent a relevant direction in internal medicine.</p>","PeriodicalId":22209,"journal":{"name":"Terapevticheskii Arkhiv","volume":"97 2","pages":"101-108"},"PeriodicalIF":0.3,"publicationDate":"2025-03-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144043086","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Evolution of enterology as a science].","authors":"E A Sabelnikova, A V Kagramanova, O V Knyazev, S V Bykova, I N Ruchkina","doi":"10.26442/00403660.2025.02.203001","DOIUrl":"https://doi.org/10.26442/00403660.2025.02.203001","url":null,"abstract":"<p><p>The article discusses the main milestones in the development of enterology in the clinic of internal diseases. The review of the important stages of the study of diseases of the small and large intestine, its main achievements, discoveries resulting from the scientific and clinical work of outstanding Russian scientists. A deep understanding of pathological processes in diseases of the small intestine is discussed, diagnostic criteria and the possibility of identifying new nosological forms are considered.</p>","PeriodicalId":22209,"journal":{"name":"Terapevticheskii Arkhiv","volume":"97 2","pages":"214-218"},"PeriodicalIF":0.3,"publicationDate":"2025-03-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144033222","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Clinical and anamnestic predictors of poor long-term prognosis in patients with chronic heart failure and implanted cardioverter-defibrillator].","authors":"N B Lebedeva, I V Talibullin, P G Parfenov, O L Barbarash","doi":"10.26442/00403660.2025.01.203045","DOIUrl":"https://doi.org/10.26442/00403660.2025.01.203045","url":null,"abstract":"<p><strong>Aim: </strong>Identification of a complex of clinical and anamnestic predictors of an unfavorable long-term prognosis in patients with heart failure with low left ventricular ejection fraction and an implanted cardioverter-defibrillator (ICD).</p><p><strong>Materials and methods: </strong>In 260 patients with heart failure with low left ventricular ejection fraction and ICD included in the \"Kuzbass Register of Patients with ICD\", data were obtained on the status of alive/dead, causes of death and cardiovascular events during a 4-year follow-up period. The clinical-instrumental and socio-demographic parameters entered into the register before ICD implantation were used to compile a prognostic regression model.</p><p><strong>Results: </strong>A total of 348 cardiovascular events (endpoints) were recorded, of which 54 were deaths. The main cause of death in 48 (88.9%) patients was acute decompensated heart failure. According to the multivariate regression analysis, the factors that increase the risk of an unfavorable long-term outcome included in the prognostic model were: the level of systolic pressure in the pulmonary artery, the thickness of the interventricular septum, social status, the presence of chronic obstructive pulmonary disease, the low ejection fraction of the left ventricle and the absence of a renin-blocker angiotensin-aldosterone system (model sensitivity - 70%, specificity - 75.9%, AUC=0.8).</p><p><strong>Conclusion: </strong>The use of a predictive model in clinical practice will make it possible to personalize approaches to making a decision on the need for ICD implantation and further monitoring of patients in order to improve their survival.</p>","PeriodicalId":22209,"journal":{"name":"Terapevticheskii Arkhiv","volume":"97 1","pages":"21-28"},"PeriodicalIF":0.3,"publicationDate":"2025-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144044732","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[A systematic review on the safety and efficacy of metamizole sodium as a therapy for the treatment of fever in children and adults].","authors":"A G Malyavin, S L Babak, A L Zaplatnikov, V А Bulgakova, M Y Garusova, M D Ilyukhina","doi":"10.26442/00403660.2025.01.203101","DOIUrl":"https://doi.org/10.26442/00403660.2025.01.203101","url":null,"abstract":"<p><strong>Aim: </strong>To systematically analyze existing publications from available scientific databases (PubMed, Cochrane, eLibrary) for the period from 2018 to 2023 on the treatment of fever in children and adults with metamizole sodium (MS).</p><p><strong>Materials and methods: </strong>A systematic review of scientific publications on the efficacy and safety of MS therapy for fever in adults and children compared to non-steroidal anti-inflammatory drugs was conducted. Six randomized clinical trials involving 884 patients (101 adults and 783 children) were included in the analysis.</p><p><strong>Results: </strong>In all studies, MS has been demonstrated to be effective in reducing fever in both children and adults when used for short-term therapy. MS is superior to paracetamol, ibuprofen, acetylsalicylic acid and not inferior to nimesulide and propacetamol in terms of effectiveness in reducing body temperature between 1.5 and 6.0 hours after oral intake. It has been shown that treatment of fever with MS is a safer strategy compared to other non-steroidal anti-inflammatory drugs, as it has no clinically significant risks of gastric mucosal irritation, development of gastrointestinal bleeding and erosions. It has been established that therapeutic doses of MS do not lead to an increase in blood pressure, and in some cases contribute to hypotensive effect, especially in fever in adult comorbid patients with arterial hypertension.</p><p><strong>Conclusion: </strong>MS is an effective treatment for fever in children and adults.</p>","PeriodicalId":22209,"journal":{"name":"Terapevticheskii Arkhiv","volume":"97 1","pages":"71-79"},"PeriodicalIF":0.3,"publicationDate":"2025-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144033223","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[New pathogenic mutation in LMNA gene: Clinical case of familial cardiomyopathy].","authors":"S Y Kashtanova, E М Rimskaya, A N Meshkov, N А Mironova, I K Dzhumaniiazova, E A Zelenova, V V Daniel', M V Ivanov, D A Kashtanova, V S Yudin, A A Keskinov, S I Mitrofanov, A I Akinshina, Y N Vanyushina, S А Kraevoy, S M Yudin, S P Golitsyn","doi":"10.26442/00403660.2025.01.203030","DOIUrl":"https://doi.org/10.26442/00403660.2025.01.203030","url":null,"abstract":"<p><p>We present a clinical case of familial <i>LMNA</i>-associated cardiomyopathy, confirmed by whole genome sequencing. The typical for lamin-associated cardiomyopathy indicates pathogenic nature of the mutation in the first exon of <i>LMNA</i> gene, previously considered a mutation of unknown clinical significance. The presented clinical case demonstrates a radical change in patient treatment strategies in the context of the widespread introduction of molecular genetic research methods into practice.</p>","PeriodicalId":22209,"journal":{"name":"Terapevticheskii Arkhiv","volume":"97 1","pages":"65-70"},"PeriodicalIF":0.3,"publicationDate":"2025-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144032368","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Efficacy of iptacopan monotherapy for suboptimal response to eculizumab in patients with paroxysmal nocturnal hemoglobinuria].","authors":"O U Klimova, I K Golubovskaya, Y N Kuznetsov, M V Marchenko, A D Kulagin","doi":"10.26442/00403660.2025.01.203146","DOIUrl":"https://doi.org/10.26442/00403660.2025.01.203146","url":null,"abstract":"<p><strong>Aim: </strong>To evaluate the hematological response within 4 weeks of iptacopan monotherapy in patients with paroxysmal nocturnal hemoglobinuria and suboptimal response to long-term eculizumab therapy.</p><p><strong>Materials and methods: </strong>The analysis included 8 patients, median age 32 years, with persistent anemia on long-term therapy with eculizumab. The hematological response was assessed when switching to oral monotherapy with iptacopan 200 mg twice daily for 4 weeks.</p><p><strong>Results: </strong>After 4 weeks of iptacopan therapy, an increase in hemoglobin levels by more than 20 g/l was achieved in 7/8 (88%), and a complete response in 6/8 (75%) patients. The median increase in hemoglobin levels was 32.5 g/l from the initial 85.5 g/l (74-100) to 121.5 g/l (80-141); <i>p</i>=0.00013. Independence from red blood cell transfusions was achieved in 100% of cases. Achieving a hematological response to iptacopan therapy was accompanied by a decrease in the level of absolute reticulocyte count, bilirubin and lactate dehydrogenase, as well as a negative result of the anti-C3d direct antiglobulin test.</p><p><strong>Conclusion: </strong>The oral complement factor B inhibitor iptacopan is an effective treatment option for paroxysmal nocturnal hemoglobinuria in patients with a suboptimal response to complement inhibitor C5 due to more effective control of extravascular C3-mediated hemolysis.</p>","PeriodicalId":22209,"journal":{"name":"Terapevticheskii Arkhiv","volume":"97 1","pages":"46-53"},"PeriodicalIF":0.3,"publicationDate":"2025-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144011742","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[The effect of inflammatory bowel diseases on the risk of atherosclerosis: assessment according to ultrasound imaging and sphygmometry].","authors":"G O Isaev, O I Trushina, M A Isaikina, A A Bestavashvili, M V Yurazh, P Y Kopylov, M H Mnatsakanyan, V V Fomin","doi":"10.26442/00403660.2025.01.203028","DOIUrl":"https://doi.org/10.26442/00403660.2025.01.203028","url":null,"abstract":"<p><strong>Aim: </strong>To evaluate the effect of inflammatory bowel disease (IBD) on the risk of atherosclerosis in patients without known cardiovascular disease.</p><p><strong>Materials and methods: </strong>The study included 115 patients divided into three groups: 37 patients with Crohn's disease (CD), 44 with ulcerative colitis (UC), and 34 in the control group without known IBD and other risk factors. Doppler ultrasound of the brachiocephalic arteries and sphygmometry were used for diagnosis. The main indicators were the thickness of the intima-media complex (TIMC) and vascular stiffness, measured by the cardio-ankle vascular index (CAVI) and the ankle-brachial pressure index (ABI).</p><p><strong>Results: </strong>IBD patients showed an increase in TIMC compared to controls. TIMC of the common carotid arteries on the right: in patients with CD - 0.07 cm (<i>p</i>=0.001), with UC - 0.08 cm (<i>p</i>=0.019), in the control group - 0.06 cm. TIMC of the common carotid arteries on the left: in patients with CD - 0.07 cm (<i>p</i>=0.001), with UC - 0.07 cm (<i>p</i>=0.012), in the control group - 0.06 cm. The sphygmometry indicators (CAVI and ABI) did not differ significantly between the groups. The mean CAVI on the right was 6.8±0.98 for the CD group, 6.6±0.79 for the UC group, and 6.82±0.76 for the control group (<i>p</i>=0.692).</p><p><strong>Conclusion: </strong>IBD can contribute to the thickening of the vascular walls, thus increasing the risk of atherosclerosis, as shown by TIMC. Vascular stiffness indicators (CAVI, ABI) did not differ significantly between the groups.</p>","PeriodicalId":22209,"journal":{"name":"Terapevticheskii Arkhiv","volume":"97 1","pages":"29-34"},"PeriodicalIF":0.3,"publicationDate":"2025-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144054969","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Experience with the use of undenatured type II collagen in patients with stage III knee osteoarthritis: a multicenter, prospective, double-blind, placebo-controlled, randomized trial].","authors":"L I Alekseeva, N G Kashevarova, E A Taskina, E A Strebkova, T A Korotkova, E P Sharapova, N M Savushkina, A M Lila, N A Shostak, I I Nesterovich, V A Dedkova, V B Vasilyuk, N V Egorova, M A Leontyeva, S P Yakupova, I B Vinogradova, V N Sorotskaya, L I Shirokova, A V Rudakova","doi":"10.26442/00403660.2025.01.203145","DOIUrl":"https://doi.org/10.26442/00403660.2025.01.203145","url":null,"abstract":"<p><strong>Introduction: </strong>Current strategies for treating osteoarthritis (OA) are based on a multimodal approach that includes pharmacological and non-pharmacological methods. Undenatured chicken collagen type II (NK2) is considered safe and effective for maintaining joint health and can be used alone and combined with drugs for OA treatment.</p><p><strong>Aim: </strong>To further evaluate the efficacy of the combination of Artneo containing NK2 in patients with stage III knee OA compared with placebo in a multicenter, prospective, double-blind, placebo-controlled, randomized trial.</p><p><strong>Materials and methods: </strong>The study included 212 patients from 12 centers in the Russian Federation, of whom 50 had radiological stage III knee OA: 41 (82.0%) females and 9 (18.0%) males aged 44 to 75. Using the interactive web response system (IWRS), the study population was randomized into two groups: Group 1 (Artneo group) included 28 patients receiving the drug 1 capsule once a day for 180 days, Group 2 (placebo group) included 22 patients receiving placebo with drug-matched presentation and the same regimen as in Group 1. The effectiveness of therapy was assessed by the change of pain using the Visual Analog Scale, the WOMAC index, KOOS, the EQ-5D quality of life questionnaire, and the need for non-steroidal anti-inflammatory drugs. All patients had a complete blood count, urinalysis, blood chemistry, and ultrasonic examination of the target knee joint.</p><p><strong>Results: </strong>A prospective, double-blind, placebo-controlled, randomized trial demonstrated that Artneo containing NK2 was significantly superior to placebo in all studied parameters, improved all clinical manifestations of OA: reduced pain and stiffness, improved joint function and quality of life, and had a good safety profile.</p><p><strong>Conclusion: </strong>Artneo showed high efficacy and safety in the general population of patients, including those with stage III knee OA.</p>","PeriodicalId":22209,"journal":{"name":"Terapevticheskii Arkhiv","volume":"97 1","pages":"54-64"},"PeriodicalIF":0.3,"publicationDate":"2025-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144027279","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}