[Evaluation of tissue perfusion by indocyanine green fluorescein angiography in patients with neuroischemic diabetic foot syndrome after endovascular treatment]. / Otsenka tkanevoi perfuzii metodom fluorestsentnoi angiografii s indotsianinom zelenym u patsientov s neiroishemicheskoi formoi sindroma diabeticheskoi stopy posle endovaskulyarnogo lecheniya.

OBJECTIVE: To evaluate the fluorescence angiography (FA) parameters with Indocyanine green (ICG) and their dynamics in diabetic foot patients after endovascular treatment. MATERIAL AND METHODS: A single-center prospective non-blinded study was conducted with the inclusion of 47 diabetic foot patients with Chronic limb-threatening ischemia (CLTI). TcPO2 and FA-ICG were done before and after angioplasty. Zones of interest were selected for FA-ICG: the area of minimum and maximum fluorescence, the area of of the largest part of the foot. Also presented are the parameters of FA ICG: Tstart (sec) - the time of occurrence of min fluorescence (Istart, unit) in the zone of interest after the introduction of ICG; Tmax (sec) - the time to achieve max fluorescence (Imax, unit) after the introduction of ICG; Tmax -Tstart (sec) - the difference in the time of reaching Imax and Istart. RESULTS: The median TcPO2 values indicated the presence of CLTI before revascularization. Technical success of revascularization was achieved in 45 patients. In the postoperative period, statistically significant changes in TcPO2 and Tstart, Tmax, Tmax-Tstar were obtained. A reduction in the time to reach the ICZ to the zones of interest was noted. CONCLUSION: FA-ICG evaluate the visual and quantitative characteristics of perfusion of soft tissues of the foot. Reducing the time to reach the fluorescent substance in the areas of interest makes it possible to assume the restoration of the main blood flow to the foot. Further investigations are warranted to determine threshold values to predict wound healing and indications for revascularization.

[Treatment of thrombotic thrombocytopenic purpura].

The review discusses approaches to treatment of congenital thrombotic thrombocytopenic purpura (TTP) or Upshaw-Schulman syndrome. In congenital TTP, plasma transfusions are sufficient. Such treatment options as plasma exchange, administration of clotting factor VIII concentrate, recombinant ADAMTS13, are also used. Separately discussed issues of management of patients with TTP during pregnancy, and pediatric patients with TTP.

[Treatment of thrombotic thrombocytopenic purpura].

The review discusses approaches to treatment of acquired thrombotic thrombocytopenic purpuгa (aTTP). In patients with aTTP plasma exchanges, glucocorticosteroids allow to stop an acute attack of TTP, and use of rituximab allows to achieve remission. In recent years, caplacizumab has been used. Treatment options such as cyclosporin A, bortezomib, splenectomy, N-acetylcysteine, recombinant ADAMTS13 are also described. Separately discussed issues of management of patients with TTP during pregnancy, and pediatric patients with TTP.

[Extracorporeal cytokine removal in chimeric antigen receptor T-cell therapy associated cytokine release syndrome in patient with acute lymphoblastic leukemia. Case report].

Сytokine release syndrome is the common complication of CAR-T therapy. We report a case of patient with B-cell acute lymphoblastic leukemia developing сytokine release syndrome with shock and multiple organ failure and requiring cytokine removal and hemodiafiltration. Remission of the disease was achieved after CAR-T therapy.

[Empagliflozin and heart failure: position paper of the experts on the results of the online meeting and discussion of the EMPEROR-Preserved Trial].

At an international online expert meeting held on September 16, 2021, the results of the empagliflozin research program EMPA-REG Outcome, EMPEROR-Reduced and EMPEROR-Preserved were reviewed. We analyzed cardiovascular and renal outcomes during the treatment with empagliflozin in patients with chronic heart failure, regardless of the presence of type 2 diabetes mellitus. The positive results of the EMPEROR-Preserved study are updated and their significance for clinical practice is discussed. Several proposals have been adopted that will accelerate the introduction of empagliflozin therapy into practice in patients with heart failure and overcome clinical inertia.

[Diagnosis of thrombotic thrombocytopenic purpura].

Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disease, disease, characterised by microangiopathic hemolytic anaemia, consumption thrombocytopenia, and organ dysfunction. The pathogenesis of TTP is attributed to the deficiency in the activity of the metalloproteinase ADAMTS13, specific von Willebrand factor cleaving protease. TTP is suspected when detecting microangiopathic hemolytic anemia, thrombocytopenia, damage to various organs. Diagnosis of TTP is confirmed by the detection of ADAMTS13 activity in plasma less than 10%. Plasma samples for the study of ADAMTS13 activity should be taken before the start of plasma transfusions or plasma exchange. In patients with severe ADAMTS-13 deficiency autoantibodies anti-ADAMTS13 and inhibitor ADAMTS13 should be investigated. Anti-ADAMTS13 antibodies belonging to IgG not always have inhibitory effects. The inhibitory effect of anti-ADAMTS13 antibodies is confirmed by mixing test. All patients with the first established diagnosis of TTP should be examined for mutations of the ADAMTS13 gene.

[Role of the intensive care in treatment of patients with acute myeloid leukemia].

AIM: Remission induction can be associate, with the life threatening complications and transfer to ICU of de novo acute myeloid leukemia (AML) patients (pts). We evaluate influence of transfer to ICU and life threatening complication on early mortality and long - tram survival of de novo AML pts. MATERIALS AND METHODS: Retrospective study. All de novo AML pts younger than 60 years old admitted in the National Research Center for Hematology from 2013 to 2016 years were enrolled in the study. Patients were divided into 2 groups: pts who were required ICU admission during remission induction (ICU-pts) and pts who did not require ICU admission and received chemotherapy only in hematology ward (non-ICU pts). The reasons for ICU admissions and results of life support were analyzed. Overall survival (OS) were assessed by the Kaplan-Meier method, long rank value p.

[Hybrid vascular interventions on the peripheral arteries in the treatment of critical ischemia in elderly patients.]

Peripheral arterial disease has a huge contribution to the overall mortality rate of the population, especially in the group of patients of old and old age. The likelihood of an adverse outcome is increased against a background with other possible nosological forms. In particular, the combination of peripheral atherosclerosis with diabetes mellitus very often manifests itself in the form of a multilevel lesion of the lower limb arteries. The treatment of such patients is very difficult, since the use of only open or endovascular approaches has its own contraindications and limitations, and cannot be used throughout the course of the lesion. The way out of this situation is the use of a hybrid approach, which consists in the combination of an open surgical reconstruction on the upper floors of the lesion and balloon angioplasty on the arteries of the lower leg. This approach allows you to use the advantages of both methods and achieve good results of treatment. The article presents the experience of treatment using a hybrid approach in elderly and senile patients with multilevel lesion of the lower limb arteries on the background of diabetes mellitus.

Treatment of gastroparesis with botulinum toxin in patient after allogenic bone marrow transplantation.

The case report of botulinum toxin treatment of gastroparesis in a patient following allogenic bone marrow transplantation is described. The causes of gastroparesis and methods of prevention and treatment are discussed. It was noted that pyloric injection of botulinum toxin can improve symptoms and gastric emptying.

[Severe postoperative intracranial hemorrhagic complications in a neurosurgical patient with von Willebrand disease not diagnosed before surgery (a case report and literature review)]. / Tiazhelye posleoperatsionnye intrakranial'nye gemorragicheskie oslozhneniia u neirokhirurgicheskogo bol'nogo s ne diagnostirovannoi do operatsii bolezn'iu Villebranda (klinicheskoe nabliudenie i obzor literatury).

The article describes a rare clinical case of a patient with previously undiagnosed von Willebrand disease and basal meningioma; an intracranial neurosurgical intervention was complicated by delayed intracranial hematomas, both at the resected tumor site and distantly. The diagnosis of von Willebrand disease was established only after special hematology tests and only after surgery. Despite the use of specific therapy, the patient died due to intracranial hemorrhagic complications in the postoperative period. The paper discusses the problem of preoperative diagnosis of asymptomatic hemostasis disorders in neurosurgical patients and potential ways of its solution.

[Impact of various wound dressings on wound bed neoangiogenesis in patients with different forms of diabetic foot syndrome]. / Vliianie razlichnykh sredstv zakrytiia ran na neoangiogenez ranevogo lozha u patsientov s razlichnymi formami sindroma diabeticheskoi stopy.

AIM: To investigate the impact of various wound dressings on wound bed neoangiogenesis in patients with different forms of diabetic foot syndrome (DFS). SUBJECTS AND METHODS: The clinical (local tissue oxygenation) and immunohistochemical (CD31) markers of foot soft tissue neoangiogenesis were evaluated in patients with DFS receiving negative pressure (NP) therapy and collagen-containing dressings (CCDs) versus standard treatment. 63 patients with neuropathic and neuroischemic (without critical ischemia) forms of DFS were examined after wound debridement. In the postoperative period, 21 patients received NP treatment, CCDs were applied to 21 patients, and 21 patients had standard treatment. RESULTS: During NP therapy, there was statistically significantly intensified local microhemodynamics, as evidenced by transcutaneous oximetry (p < 0.05); the remaining two groups showed no statistically significant differences in transcutaneous oxygen tension during the treatment. Immunohistochemical examination revealed a significant increase in the number of newly formed vessels, as shown by anti-CD31 antibody staining (p < 0.05), in patients who had NP therapy and CCDs (p < 0.05). CONCLUSION: Vacuum (NP) therapy versus standard therapy most effectively affects wound bed neoangiogenesis. This is reflected in the increased local tissue microhemodynamics, as confirmed by immunohistochemical examination.

[Progressive pulmonary hypertension in a patient with type 1 Gaucher disease]. / Progressiruiushchaia legochnaia gipertenziia u patsienta s bolezn'iu Goshe I tipa.

Gaucher disease is the most common form of hereditary enzymopathies combined into a group of lysosomal storage diseases. The basis for the disease is a hereditary deficiency of the activity of acid ß-glucosidase, a lysosomal enzyme involved in the catabolism of lipids, which results in the accumulation of nonutilized cellular metabolism products in the macrophage lysosomes. The main clinical manifestations of type 1 Gaucher disease are cytopenia, hepatomegaly, and splenomegaly, and bone lesion. One of the atypical clinical manifestations of Gaucher disease is damage to the lungs with the development of pulmonary hypertension, which is usually considered within the underlying disease - the development of pneumosclerosis due to macrophage dysfunction. The paper describes a case of progressive pulmonary hypertension in a patient with type 1 Gaucher disease.

[EBV-positive central nervous system lymphoproliferative disease associated with immunosuppression after organ transplantation: Long-term remission without chemotherapy]. / Limfoproliferativnoe EBV-pozitivnoe zabolevanie s porazheniem tsentral'noi nervnoi sistemy, assotsiirovannoe s immunosupressiei posle organnoi transplantatsii: dlitel'naia remissiia posle khimioterapevticheskogo lecheniia.

Primary central nervous system (CNS) lymphomas account for 13-20% of the posttransplant lymphoproliferative disorders (PTLD) and rank among the most aggressive conditions. Reduction of immunosuppressive therapy should be mandatory to treat PTLD, but this is rarely used as the only therapy option. Chemotherapy regimens for PTLD involving the CNS most commonly include high-dose rituximab and high-dose methotrexate and/or cytarabine. The efficiency only of discontinuation of immunosuppressive therapy for PTLD does not exceed 5-10%, but there are no literature data on its efficiency for PTLD involving the CNS. The paper describes a clinical case of achieving long-term remission in a female patient with Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphoma involving the central nervous system, associated with immunosuppression after kidney transplantation from a related donor, in the absence of chemotherapy during immunosuppressive therapy discontinuation and transplantectomy.

[Thrombotic events in patients with hemophilia]. / Tromboticheskie oslozhneniia u bol'nykh gemofiliei.

The paper describes 4 clinical cases of thrombotic events (pulmonary embolism, deep vein thrombophlebitis, acute myocardial infarction, ischemic stroke) that have occurred in patients with hemophilia. It discusses the possible causes of their development and methods for their prevention and treatment. Controlled natural hypocoagulation, in which the dose of an administered deficient factor decreases to such an extent that in order to maintain the safe level of hypocoagulation (plasma factor activity is 15-20%; activated partial thromboplastin time is 1.5-2 times normal values), is proposed as one of the treatment options.

[LUNG ULTRASOUND IN PATIENTS WITH HEMATOLOGICAL MALIGNANCIES AND ACUTE RESPIRATORY FAILURE DUE TO PNEUMONIA.]

PURPOSE: To compare the accuracy of bedside lung ultrasound (LUS) and chest computed tomography (CT) for the de- tection of lung lesions in patients with hematological malignancies and acute respiratory failure (ARF). MATERIALS AND METHODS: 39 patients with hematological malignancies and ARF were enrolled in prospective study. The investigation of the patients included LUS, chest C, extravascular lung water index (EVLW) by transpulmonary ther- modilution, and bronchoalveolar lavage (BAL). RESULTS: There was correlation between the total number of B-lines and E VLW index (r = 0,40; p <0,05). The sensitivity, specificity of LUS in the total number of B-lines were 78% and 70%, respectively (and A UC 0,7). There were correla- tions between A-lines and volume of hyperaerated lung regions (r = 0,40; p <0,05) and normally ventilated (r = 0,60; p = 0,001) regions, between A-lines and the total lung volume (r = 0,50; p = 0,001), between A-lines and volume of poorlyventilated lung regions (r = -0,40; p = 0,001), A-lines and weight of normally ventilated lung regions (r = 0,50; p = 0,001), A-lines and weight of poorly ventilated regions (r = -0,35; p <0,05), total count of B-lines and volume of poorly ventilated lung regions (r = 0,4; p = 0,001), between total count of B-lines and weight poorly ventilated lung regions (r = 0,4; p = 0,001). There were associations between USfeathers and etiology ofpneumonia. A-lines were often detected in patients with gram-negative bacterial pneumonia and fungal pneumonia more than in patients with pneu- mocystis pneumonia. B-lines were detected often in patients with Pneumocystis pneumonia. Sensitivity ofLUS pleural effusion assessment was 95%, specificity was 90%. CONCLUSION: LUS is high sensitivity and specificity method to detect lung lesions in patients with ARF.

Rates and predictors of hypoglycaemia in 27 585 people from 24 countries with insulin-treated type 1 and type 2 diabetes: the global HAT study.

AIMS: To determine the global extent of hypoglycaemia experienced by patients with diabetes using insulin, as there is a lack of data on the prevalence of hypoglycaemia in developed and developing countries. METHODS: This non-interventional, multicentre, 6-month retrospective and 4-week prospective study using self-assessment questionnaire and patient diaries included 27 585 patients, aged ≥18 years, with type 1 diabetes (T1D; n = 8022) or type 2 diabetes (T2D; n = 19 563) treated with insulin for >12 months, at 2004 sites in 24 countries worldwide. The primary endpoint was the proportion of patients experiencing at least one hypoglycaemic event during the observational period. RESULTS: During the prospective period, 83.0% of patients with T1D and 46.5% of patients with T2D reported hypoglycaemia. Rates of any, nocturnal and severe hypoglycaemia were 73.3 [95% confidence interval (CI) 72.6-74.0], 11.3 (95% CI 11.0-11.6) and 4.9 (95% CI 4.7-5.1) events/patient-year for T1D and 19.3 (95% CI 19.1-19.6), 3.7 (95% CI 3.6-3.8) and 2.5 events/patient-year (95% CI 2.4-2.5) for T2D, respectively. The highest rates of any hypoglycaemia were observed in Latin America for T1D and Russia for T2D. Glycated haemoglobin level was not a significant predictor of hypoglycaemia. CONCLUSIONS: We report hypoglycaemia rates in a global population, including those in countries without previous data. Overall hypoglycaemia rates were high, with large variations between geographical regions. Further investigation into these differences may help to optimize therapy and reduce the risk of hypoglycaemia.

[The Study of Influence of Different Methods of Local Treatment on Wound Healing in Patients with Diabetic Foot Ulcers].

Aim: To evaluate the influence of different methods of local treatment on tissue repair in patients with diabetic foot ulcers. Materials and Methods: We evaluated such clinical characteristics as wound size and local perfusion after using negative pressure wound therapy (NPWT), local collagen, and standard care in patients with diabetic foot ulcers. We observed 63 patients with neuropathic and neuroischemic forms of diabetic foot (without critical ischemia) after surgical debridement. After that 21 patients received NPWT, 21 local collagen treatment and 21 ­ standard care. Results: After using NPWT wound area and depth decreased in 19,8% and 42,8% (p<0.05), in group of collagen dressings in 26,4 and 30,4% (p<0.05). In control group those parameters were 17,0 и 16.6% respectively (p<0.05). There was found the significant increase of local perfusion according to oxygen monitoring in group of NPWT (p<0.05). Conclusion: The received data showed that the intensity of lower limb tissue repair processes increases more significant after using NPWT and collagen dressings in comparison to standard care which is found according to wound size and tissue perfusion alterations.

[The specific features of pain syndrome in patients with hemophilia]. / Osobennosti bolevogo sindroma u patsientov s gemofiliei.

AIM: to study the nature of pain in hemophilic arthropathy and its prevention ways used by patients with hemophilia. SUBJECTS AND METHODS: A prospective, multicenter, cross-sectional study was conducted to interview 136 patients with hemophilia A or B. The survey included 32 points, including questions about drug and nondrug pain treatment, as well as questionnaires to assess the severity of pain and its impact on daily life (Brief Pain Inventory) and those to identify a neuropathic pain component (PainDetect questionnaire). RESULTS: 83 (75%) patients with hemophilia experienced acute pain associated with bleeding into the large joints; 44 (39%) patients had chronic pain that had lasted longer than six months; 33% assessed the moderate pain experienced in the past 24 hours as severe (more than 5-8 of the 10 scores). In addition to the above, only 32% of the respondents indicated that they had painless intervals within the last 24 hours. 75% of the hemophilia patients mentioned to have limited daily activities. 74% reported their partial or complete disability when pain occurred. 77% of the respondents pointed out that when having pain, they had experienced great difficulty walking. 55% of the hemophilia patients had sleep disorders because of pain. When acute pain occurred, only 91 (81%) respondents injected a factor VIII or IX preparation, 37% of the respondents used narcotic analgesics; 51% received different nonsteroidal anti-inflammatory drugs; 13% took paracetamol. Some patients indicated that they used alcohol and illegal narcotic drugs to relieve pain. Attention is drawn to the fact that the hemophilia patients very frequently took painkillers: 60% of the respondents used analgesics every month; of them 49% had them every week, 11% every day. Most patients (n=83 (74%)) stated that they treated pain (prescribed drugs) themselves; 49 (44%) patients held they were dissatisfied with their pain treatment. When the question as to how the analgesic you used could "remove' pain was asked, only 12 (10%) patients answered that this could fully relieve the pain; 31 (26%) patients told that the pain was not reduced even by half. The study has shown that a neuropathic pain component is very common in hemophilia patients (31%). CONCLUSION: Pain in patients with hemophilia is a serious problem that negatively affects their quality of life, including their working capacity. The prevention and treatment of pain in hemophilia patients should involve the following: organization of educational activities and pain services; adequate treatment of acute pain; detection and prevention of a neuropathic pain component.

[Comparative evaluation of the intensity of reparative processes in the lower extremity soft tissues of diabetic patients receiving local negative pressure wound treatment and standard wound care]. / Sravnitel'naya otsenka intensivnosti reparativnykh protsessov v myagkikh tkanyakh nizhnikh konechnostei u patsientov s sakharnym diabetom na fone primeneniya lokal'nogo otritsatel'nogo davleniya i standartnoi terapii.

AIM: To study the intensity of soft tissue repair in patients with diabetic foot syndrome (DFS) during local negative pressure wound treatment versus standard wound care. SUBJECTS AND METHODS: The investigators estimated the clinical (wound sizes, local tissue oxygenation), histological (light microscopy), and immunohistochemical (CD31, CD68, MMP-9, and TIMP-1) markers for reparative processes in patients with DFS during vacuum therapy versus standard wound care. Forty-two patients with the neuropathic and neuroischemic (without critical ischemia) forms of DFS were examined after debridement. In the perioperative period, 21 patients received negative pressure wound therapy and 21 had standard wound care. RESULTS: During vacuum therapy, the area and depth of wound defects decreased by 19.8±7.8 and 42.8±5.6%, respectively (p=0.002) (as compared to the baseline data). In the control group, these indicators were 17.0±19.4 and 16.6±21.6% (p=0.002). There was a significant intensification of local microhemodynamics according to transcutaneous oximetry readings in the negative pressure wound treatment group. After 9±2 days of treatment, histological examination of granulation tissue revealed a significant reduction in edema, cessation of inflammatory infiltration, and formation of mature granulation tissue in Group 1. Immunohistological examination indicated a more obvious increase in the count of macrophages (CD68 staining) and a significant increment in the number of newly formed vessels, as evidenced by anti-CD31 antibody staining. During the treatment, there was a decline of the expression of MMP-9 and an increase in that of TIMP-1, as compared to those in the control group. CONCLUSION: The findings are indicative of the enhanced intensity of reparative processes in patients with DFS during vacuum therapy versus standard wound care, resulting in more rapidly decreased wound sizes, increased local microhemodynamics, reduced inflammation, and accelerated wound transition from the inflammatory to the proliferative phase.

[Laparoscopic splenectomy in immune thrombocytopenic purpura in pregnant women]. / Laparoskopicheskaya splenektomiya pri immunnoi trombotsitopenicheskoi purpure u beremennykh.

The paper describes 4 cases of laparoscopic splenectomy in pregnant women with immune thrombocytopenic purpura. No complications of surgery were noted in all the patients. The postoperative period was marked by sustained clinical and hematological remission that made it possible to discontinue prednisolone therapy and to ensure an uncomplicated course of pregnancy and labor.