10 research outputs found

    KTM TOKAMAK OPERATION SCENARIOS SOFTWARE INFRASTRUCTURE

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    One of the largest problems for tokamak devices such as Kazakhstan Tokamak for Material Testing (KTM) is the operation scenarios' development and execution. Operation scenarios may be varied often, so a convenient hardware and software solution is required for scenario management and execution. Dozens of diagnostic and control subsystems with numerous configuration settings may be used in an experiment, so it is required to automate the subsystem configuration process to coordinate changes of the related settings and to prevent errors. Most of the diagnostic and control subsystems software at KTM was unified using an extra software layer, describing the hardware abstraction interface. The experiment sequence was described using a command language.The whole infrastructure was brought together by a universal communication protocol supporting various media, including Ethernet and serial links. The operation sequence execution infrastructure was used at KTM to carry out plasma experiments

    ДиагностичСскиС ΠΈ прогностичСскиС Π»Π°Π±ΠΎΡ€Π°Ρ‚ΠΎΡ€Π½Ρ‹Π΅ ΠΊΡ€ΠΈΡ‚Π΅Ρ€ΠΈΠΈ развития сСпсиса ΠΏΡ€ΠΈ Π³Π½ΠΎΠΉΠ½ΠΎ-Π²ΠΎΡΠΏΠ°Π»ΠΈΡ‚Π΅Π»ΡŒΠ½Ρ‹Ρ… заболСваниях мягких Ρ‚ΠΊΠ°Π½Π΅ΠΉ

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    Objective. Identification of laboratory parameters that are used in routine practice and can serve as diagnostic and prognostic criteria for the development of sepsis and its outcomes in patients with purulent-inflammatory diseases of soft tissues.Materials and methods. The study included 48 patients with purulent-inflammatory diseases of soft tissues. Recorded the occurrence of such clinical events as the development of sepsis or septic shock, intensive therapy, death or recovery and discharge from the hospital. For the diagnosis of sepsis, a SOFA (Sepsis-related organ failure assessment score) Β³ 2 points was used. Patients were divided into subgroups according to the number of points according to the SOFA scale, intensive care and depending on the outcome of the disease: Subgroup 1 – 26 patients with sepsis (SOFA Β³ 2 points) and 22 patients with systemic inflammatory response syndrome (SIRS) and SOFA <2 points; 2nd subgroup – 12 people who underwent intensive therapy and 36 people without it; 3rd subgroup – 7 patients with a fatal outcome and 41 patients with a favorable outcome.Results. In patients with sepsis, albumin concentration was 24,07 g / l in median versus 34,65 g / l in the control group of patients with SOFA <2 points (p <0,01); glucose -7,82 mmol / l and 5,15 mmol / l (p <0,01); sodium concentration of 133 mmol / li 139 mmol / l (p <0,01). The values of the international normalized ratio (INR) amounted to a median of 1,29 and 1,04 (p <0,01); activated partial thromboplastin time -36,20 seconds and 31,50 seconds (p <0,01). In the subgroup of patients for whom intensive therapy was required, the concentration of albumin was 22,34 g / l by median versus 30,10 g / l (p <0,01); urea – 15,50 mmol / l versus 6,00 mmol / l (p <0,05), glucose – 9,61 mmol / l against 5,80 mmol / l (p <0,05), lactate dehydrogenase-644,00 U / l and 426,00 U / l (p <0,05); INR – 1,35 against 1,05 (p <0,05). The aver-age total protein concentration is 47,80 g / l versus 57,90 g / l (p <0,01). The average albumin is 22,34 g / l versus 28,50 g / l (p <0,05). The glucose concentration among patients with a fatal outcome was 12,00 mmol / l in median versus 5,95 mmol / l (p <0,01); urea – 23,22 mmol / l versus 6,00 mmol / l (p <0,01). The incidence of lethal disease was statistically significantly higher in patients with a total protein level of less than 52 g / l 5,96 times (RR = 5,96, 95% CI 1,32 – 26,89), glucose more than 11 mmol / l – 7,00 times (OR = 7,00, 95% CI 1,25 – 39,15), urea more than 20 mmol / l – 7,05 times (RR = 7,05, 95% CI 2,00 – 24,85).Conclusion. Routine laboratory indicators as the level of total protein, albumin, glucose, sodium and urea, as well as indicators of the blood coagulation system (INR and APTT), can serve as diagnostic and prognostic criteria for the development of sepsis and its outcomes in patients with purulentinflammatory diseases. soft tissue.ЦСль: выявлСниС Π»Π°Π±ΠΎΡ€Π°Ρ‚ΠΎΡ€Π½Ρ‹Ρ… ΠΏΠΎΠΊΠ°Π·Π°Ρ‚Π΅Π»Π΅ΠΉ, ΠΊΠΎΡ‚ΠΎΡ€Ρ‹Π΅ ΠΈΡΠΏΠΎΠ»ΡŒΠ·ΡƒΡŽΡ‚ΡΡ Π² Ρ€ΡƒΡ‚ΠΈΠ½Π½ΠΎΠΉ ΠΏΡ€Π°ΠΊΡ‚ΠΈΠΊΠ΅ ΠΈ ΠΌΠΎΠ³ΡƒΡ‚ ΡΠ»ΡƒΠΆΠΈΡ‚ΡŒ диагностичСскими ΠΈ прогностичСскими критСриями развития сСпсиса ΠΈ Π΅Π³ΠΎ исходов Ρƒ ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ² с Π³Π½ΠΎΠΉΠ½ΠΎ-Π²ΠΎΡΠΏΠ°Π»ΠΈΡ‚Π΅Π»ΡŒΠ½Ρ‹ΠΌΠΈ заболСваниями мягких Ρ‚ΠΊΠ°Π½Π΅ΠΉ.ΠœΠ°Ρ‚Π΅Ρ€ΠΈΠ°Π»Ρ‹ ΠΈ ΠΌΠ΅Ρ‚ΠΎΠ΄Ρ‹. Π’ исслСдованиС Π²ΠΊΠ»ΡŽΡ‡Π΅Π½ΠΎ 48 ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ² с Π³Π½ΠΎΠΉΠ½ΠΎ-Π²ΠΎΡΠΏΠ°Π»ΠΈΡ‚Π΅Π»ΡŒΠ½Ρ‹ΠΌΠΈ заболСваниями мягких Ρ‚ΠΊΠ°Π½Π΅ΠΉ. ΠŸΡ€ΠΎΠ²ΠΎΠ΄ΠΈΠ»ΠΈ ΡƒΡ‡Π΅Ρ‚ наступлСния Ρ‚Π°ΠΊΠΈΡ… клиничСских событий, ΠΊΠ°ΠΊ Ρ€Π°Π·Π²ΠΈΡ‚ΠΈΠ΅ сСпсиса ΠΈΠ»ΠΈ сСптичСского шока, ΠΏΡ€ΠΎΠ²Π΅Π΄Π΅Π½ΠΈΠ΅ интСнсивной Ρ‚Π΅Ρ€Π°ΠΏΠΈΠΈ, Π»Π΅Ρ‚Π°Π»ΡŒΠ½Ρ‹ΠΉ исход ΠΈΠ»ΠΈ Π²Ρ‹Π·Π΄ΠΎΡ€ΠΎΠ²Π»Π΅Π½ΠΈΠ΅ ΠΈ выписка ΠΈΠ· стационара. Для постановки Π΄ΠΈΠ°Π³Π½ΠΎΠ·Π° «сСпсис» примСняли ΡˆΠΊΠ°Π»Ρƒ SOFA (Sepsis-related organ failure assessment score) Β³Β 2 Π±Π°Π»Π»ΠΎΠ². ΠŸΠ°Ρ†ΠΈΠ΅Π½Ρ‚Ρ‹ Π±Ρ‹Π»ΠΈ распрСдСлСны Π² ΠΏΠΎΠ΄Π³Ρ€ΡƒΠΏΠΏΡ‹ ΠΏΠΎ количСству Π±Π°Π»Π»ΠΎΠ² ΠΏΠΎ шкалС SOFA, провСдСния интСнсивной Ρ‚Π΅Ρ€Π°ΠΏΠΈΠΈ ΠΈ Π² зависимости ΠΎΡ‚ исхода заболСвания: 1-я ΠΏΠΎΠ΄Π³Ρ€ΡƒΠΏΠΏΠ° – 26 Π±ΠΎΠ»ΡŒΠ½Ρ‹Ρ… сСпсисом (SOFAΒ Β³Β 2 Π±Π°Π»Π»ΠΎΠ²) ΠΈ 22 ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚Π° с синдромом систСмного Π²ΠΎΡΠΏΠ°Π»ΠΈΡ‚Π΅Π»ΡŒΠ½ΠΎΠ³ΠΎ ΠΎΡ‚Π²Π΅Ρ‚Π° (Π‘Π‘Π’Π ) ΠΈ SOFA < 2 Π±Π°Π»Π»ΠΎΠ²; 2-я ΠΏΠΎΠ΄Π³Ρ€ΡƒΠΏΠΏΠ° – 12 Ρ‡Π΅Π»ΠΎΠ²Π΅ΠΊ, ΠΊΠΎΡ‚ΠΎΡ€Ρ‹ΠΌ ΠΏΡ€ΠΎΠ²ΠΎΠ΄ΠΈΠ»Π°ΡΡŒ интСнсивная тСрапия, ΠΈ 36 Ρ‡Π΅Π»ΠΎΠ²Π΅ΠΊ Π±Π΅Π· Π½Π΅Π΅; 3-я ΠΏΠΎΠ΄Π³Ρ€ΡƒΠΏΠΏΠ° – 7 Π±ΠΎΠ»ΡŒΠ½Ρ‹Ρ… с Π»Π΅Ρ‚Π°Π»ΡŒΠ½Ρ‹ΠΌ исходом ΠΈ 41 ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ с благоприятным исходом.Π Π΅Π·ΡƒΠ»ΡŒΡ‚Π°Ρ‚Ρ‹. Π£ ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ² с сСпсисом концСнтрация Π°Π»ΡŒΠ±ΡƒΠΌΠΈΠ½Π° составила ΠΏΠΎ ΠΌΠ΅Π΄ΠΈΠ°Π½Π΅ 24,07 Π³/Π» ΠΏΡ€ΠΎΡ‚ΠΈΠ² 34,65 Π³/Π» Π² ΠΊΠΎΠ½Ρ‚Ρ€ΠΎΠ»ΡŒΠ½ΠΎΠΉ Π³Ρ€ΡƒΠΏΠΏΠ΅ Π±ΠΎΠ»ΡŒΠ½Ρ‹Ρ… с SOFA < 2 Π±Π°Π»Π»ΠΎΠ² (p< 0,01); Π³Π»ΡŽΠΊΠΎΠ·Ρ‹ – 7,82 ммоль/Π» ΠΈ 5,15 ммоль/Π» (p< 0,01); концСнтрация натрия 133 ммоль/Π» ΠΈ 139 ммоль/Π» (p< 0,01). ЗначСния ΠΌΠ΅ΠΆΠ΄ΡƒΠ½Π°Ρ€ΠΎΠ΄Π½ΠΎΠ³ΠΎ Π½ΠΎΡ€ΠΌΠ°Π»ΠΈΠ·ΠΎΠ²Π°Π½Π½ΠΎΠ³ΠΎ ΠΎΡ‚Π½ΠΎΡˆΠ΅Π½ΠΈΡ (МНО) составили ΠΏΠΎ ΠΌΠ΅Π΄ΠΈΠ°Π½Π΅ 1,29 ΠΈ1,04 (p< 0,01); Π°ΠΊΡ‚ΠΈΠ²ΠΈΡ€ΠΎΠ²Π°Π½Π½ΠΎΠ³ΠΎ частичного тромбопластинового Π²Ρ€Π΅ΠΌΠ΅Π½ΠΈ – 36,20 с ΠΈ 31,50 с (p< 0,01). Π’ ΠΏΠΎΠ΄Π³Ρ€ΡƒΠΏΠΏΠ΅ ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ², ΠΊΠΎΡ‚ΠΎΡ€Ρ‹ΠΌ ΠΏΠΎΡ‚Ρ€Π΅Π±ΠΎΠ²Π°Π»ΠΎΡΡŒ ΠΏΡ€ΠΎΠ²Π΅Π΄Π΅Π½ΠΈΠ΅ интСнсивной Ρ‚Π΅Ρ€Π°ΠΏΠΈΠΈ, ΠΊΠΎΠ½Ρ†Π΅Π½Ρ‚Ρ€Π°Ρ†ΠΈΠΈ Π°Π»ΡŒΠ±ΡƒΠΌΠΈΠ½Π° составили ΠΏΠΎ ΠΌΠ΅Π΄ΠΈΠ°Π½Π΅ 22,34 Π³/Π» ΠΏΡ€ΠΎΡ‚ΠΈΠ² 30,10 Π³/Π» (p< 0,01); ΠΌΠΎΡ‡Π΅Π²ΠΈΠ½Ρ‹ – 15,50 ммоль/Π» ΠΏΡ€ΠΎΡ‚ΠΈΠ² 6,00 ммоль/Π» (p< 0,05), Π³Π»ΡŽΠΊΠΎΠ·Ρ‹β€“ 9,61 ммоль/Π»ΠΏΡ€ΠΎΡ‚ΠΈΠ² 5,80 ммоль/Π»(p< 0,05), Π»Π°ΠΊΡ‚Π°Ρ‚Π΄Π΅Π³ΠΈΠ΄Ρ€ΠΎΠ³Π΅Π½Π°Π·Ρ‹ – 644,00 Π•Π΄/Π» ΠΈ 426,00 Π•Π΄/Π» (p< 0,05); МНО – 1,35 ΠΏΡ€ΠΎΡ‚ΠΈΠ² 1,05 (p< 0,05). БрСдняя концСнтрация ΠΎΠ±Ρ‰Π΅Π³ΠΎ Π±Π΅Π»ΠΊΠ° – 47,80 Π³/Π» ΠΏΡ€ΠΎΡ‚ΠΈΠ² 57,90 Π³/Π» (p < 0,01). Π‘Ρ€Π΅Π΄Π½ΠΈΠ΅ ΠΏΠΎΠΊΠ°Π·Π°Ρ‚Π΅Π»ΠΈ Π°Π»ΡŒΠ±ΡƒΠΌΠΈΠ½Π° – 22,34 Π³/Π» ΠΏΡ€ΠΎΡ‚ΠΈΠ² 28,50 Π³/Π» (p < 0,05). ΠšΠΎΠ½Ρ†Π΅Π½Ρ‚Ρ€Π°Ρ†ΠΈΡ Π³Π»ΡŽΠΊΠΎΠ·Ρ‹ срСди ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ² с Π»Π΅Ρ‚Π°Π»ΡŒΠ½Ρ‹ΠΌ исходом составила ΠΏΠΎ ΠΌΠ΅Π΄ΠΈΠ°Π½Π΅ 12,00 ммоль/Π» ΠΏΡ€ΠΎΡ‚ΠΈΠ² 5,95 ммоль/Π» (p< 0,01); ΠΌΠΎΡ‡Π΅Π²ΠΈΠ½Ρ‹ – 23,22 ммоль/Π» ΠΏΡ€ΠΎΡ‚ΠΈΠ² 6,00 ммоль/Π» (p < 0,01). Частота наступлСния Π»Π΅Ρ‚Π°Π»ΡŒΠ½ΠΎΠ³ΠΎ исхода Π±ΠΎΠ»Π΅Π·Π½ΠΈ Π±Ρ‹Π»Π° статистичСски Π·Π½Π°Ρ‡ΠΈΠΌΠΎ Π²Ρ‹ΡˆΠ΅ Ρƒ ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ² с ΡƒΡ€ΠΎΠ²Π½Π΅ΠΌ ΠΎΠ±Ρ‰Π΅Π³ΠΎ Π±Π΅Π»ΠΊΠ° ΠΌΠ΅Π½Π΅Π΅ 52 Π³/Π» Π² 5,96 Ρ€Π°Π· (ОР = 5,96, 95 % Π”Π˜ 1,32 – 26,89), Π³Π»ΡŽΠΊΠΎΠ·Ρ‹ Π±ΠΎΠ»Π΅Π΅ 11 ммоль/Π» – Π² 7,00 Ρ€Π°Π· (ОР = 7,00, 95 % Π”Π˜ 1,25 – 39,15), ΠΌΠΎΡ‡Π΅Π²ΠΈΠ½Ρ‹ Π±ΠΎΠ»Π΅Π΅ 20 ммоль/Π» – Π² 7,05 Ρ€Π°Π· (ОР = 7,05, 95 % Π”Π˜ 2,00 – 24,85).Π—Π°ΠΊΠ»ΡŽΡ‡Π΅Π½ΠΈΠ΅. Π’Π°ΠΊΠΈΠ΅ Ρ€ΡƒΡ‚ΠΈΠ½Π½Ρ‹Π΅ Π»Π°Π±ΠΎΡ€Π°Ρ‚ΠΎΡ€Π½Ρ‹Π΅ ΠΏΠΎΠΊΠ°Π·Π°Ρ‚Π΅Π»ΠΈ, ΠΊΠ°ΠΊ ΡƒΡ€ΠΎΠ²Π΅Π½ΡŒ ΠΎΠ±Ρ‰Π΅Π³ΠΎ Π±Π΅Π»ΠΊΠ°, Π°Π»ΡŒΠ±ΡƒΠΌΠΈΠ½Π°, Π³Π»ΡŽΠΊΠΎΠ·Ρ‹, натрия ΠΈ ΠΌΠΎΡ‡Π΅Π²ΠΈΠ½Ρ‹, Π° Ρ‚Π°ΠΊΠΆΠ΅ ΠΏΠΎΠΊΠ°Π·Π°Ρ‚Π΅Π»ΠΈ ΡΠ²Π΅Ρ€Ρ‚Ρ‹Π²Π°ΡŽΡ‰Π΅ΠΉ систСмы ΠΊΡ€ΠΎΠ²ΠΈ (МНО ΠΈ АЧВВ) ΠΌΠΎΠ³ΡƒΡ‚ ΡΠ»ΡƒΠΆΠΈΡ‚ΡŒ диагностичСскими ΠΈ прогностичСскими критСриями развития сСпсиса ΠΈ Π΅Π³ΠΎ исходов Ρƒ ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ² с Π³Π½ΠΎΠΉΠ½ΠΎ-Π²ΠΎΡΠΏΠ°Π»ΠΈΡ‚Π΅Π»ΡŒΠ½Ρ‹ΠΌΠΈ заболСваниями мягких Ρ‚ΠΊΠ°Π½Π΅ΠΉ

    Implementation of remote participation at Tokamak device experiments

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    The paper describes the software implementation of remote participation at Tokamak device experiment, particularly hardware remote control and monitoring implementation. This is an alternative to the traditional implementations based on such software kits as MDS+ and PK IVK used at Russian and foreign Tokamak devices: T-10, T-15, JET, JT-60, FTU. Traditional implementations are outdated and require deep modification for effective usage at new devices, so the new implementation is rather actual. The implementation is based on the use of new unified communication protocol for Tokamak subsystems - T-ICS. The protocol takes into account the individual peculiarities of subsystem hardware platform and provides the means of message encryption required for communication using Ethernet-based networks. The comparison of traditional approaches and the described implementation was also described. The implementation of the approach proposed was shown using a TRACE MODE SCADA for the experiment automation system hardware control at one of the foreign Tokamak devices. The use of the new implementation combined with SCADA allows significant automation of the applications development, simplifies hardware control and monitoring command language. At the present the software implementation was tested on the models of the active foreign Tokamak subsystems and it is currently used for model subsystem control. Because of the high potential of the mechanism is going to be integrated into control system of modified T-15 Russian Tokamak

    Implementation of remote participation at Tokamak device experiments

    No full text
    The paper describes the software implementation of remote participation at Tokamak device experiment, particularly hardware remote control and monitoring implementation. This is an alternative to the traditional implementations based on such software kits as MDS+ and PK IVK used at Russian and foreign Tokamak devices: T-10, T-15, JET, JT-60, FTU. Traditional implementations are outdated and require deep modification for effective usage at new devices, so the new implementation is rather actual. The implementation is based on the use of new unified communication protocol for Tokamak subsystems - T-ICS. The protocol takes into account the individual peculiarities of subsystem hardware platform and provides the means of message encryption required for communication using Ethernet-based networks. The comparison of traditional approaches and the described implementation was also described. The implementation of the approach proposed was shown using a TRACE MODE SCADA for the experiment automation system hardware control at one of the foreign Tokamak devices. The use of the new implementation combined with SCADA allows significant automation of the applications development, simplifies hardware control and monitoring command language. At the present the software implementation was tested on the models of the active foreign Tokamak subsystems and it is currently used for model subsystem control. Because of the high potential of the mechanism is going to be integrated into control system of modified T-15 Russian Tokamak

    Diagnostic and prognostic laboratory criteria for the development of sepsis in purulent-inflammatory diseases of soft tissues

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    Objective. Identification of laboratory parameters that are used in routine practice and can serve as diagnostic and prognostic criteria for the development of sepsis and its outcomes in patients with purulent-inflammatory diseases of soft tissues.Materials and methods. The study included 48 patients with purulent-inflammatory diseases of soft tissues. Recorded the occurrence of such clinical events as the development of sepsis or septic shock, intensive therapy, death or recovery and discharge from the hospital. For the diagnosis of sepsis, a SOFA (Sepsis-related organ failure assessment score) Β³ 2 points was used. Patients were divided into subgroups according to the number of points according to the SOFA scale, intensive care and depending on the outcome of the disease: Subgroup 1 – 26 patients with sepsis (SOFA Β³ 2 points) and 22 patients with systemic inflammatory response syndrome (SIRS) and SOFA <2 points; 2nd subgroup – 12 people who underwent intensive therapy and 36 people without it; 3rd subgroup – 7 patients with a fatal outcome and 41 patients with a favorable outcome.Results. In patients with sepsis, albumin concentration was 24,07 g / l in median versus 34,65 g / l in the control group of patients with SOFA <2 points (p <0,01); glucose -7,82 mmol / l and 5,15 mmol / l (p <0,01); sodium concentration of 133 mmol / li 139 mmol / l (p <0,01). The values of the international normalized ratio (INR) amounted to a median of 1,29 and 1,04 (p <0,01); activated partial thromboplastin time -36,20 seconds and 31,50 seconds (p <0,01). In the subgroup of patients for whom intensive therapy was required, the concentration of albumin was 22,34 g / l by median versus 30,10 g / l (p <0,01); urea – 15,50 mmol / l versus 6,00 mmol / l (p <0,05), glucose – 9,61 mmol / l against 5,80 mmol / l (p <0,05), lactate dehydrogenase-644,00 U / l and 426,00 U / l (p <0,05); INR – 1,35 against 1,05 (p <0,05). The aver-age total protein concentration is 47,80 g / l versus 57,90 g / l (p <0,01). The average albumin is 22,34 g / l versus 28,50 g / l (p <0,05). The glucose concentration among patients with a fatal outcome was 12,00 mmol / l in median versus 5,95 mmol / l (p <0,01); urea – 23,22 mmol / l versus 6,00 mmol / l (p <0,01). The incidence of lethal disease was statistically significantly higher in patients with a total protein level of less than 52 g / l 5,96 times (RR = 5,96, 95% CI 1,32 – 26,89), glucose more than 11 mmol / l – 7,00 times (OR = 7,00, 95% CI 1,25 – 39,15), urea more than 20 mmol / l – 7,05 times (RR = 7,05, 95% CI 2,00 – 24,85).Conclusion. Routine laboratory indicators as the level of total protein, albumin, glucose, sodium and urea, as well as indicators of the blood coagulation system (INR and APTT), can serve as diagnostic and prognostic criteria for the development of sepsis and its outcomes in patients with purulentinflammatory diseases. soft tissue

    POLYMORPHISM OF THROMBOPHILIA GENES AND THEIR ROLE IN DEVELOPMENT OF DIFFERENT DISEASE PHENOTYPES AND THROMBOTIC COMPLICATIONS IN HEMOPHILIA PATIENTS

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    ObjectiveΒ was to study genetic markers of thrombophilia in patients with hemophilia, which can affect the course of the disease and contribute to thrombotic complications.Β Material and methods.Β The study included 96 patients with severe hemophilia: 75 (78.1Β %) – hemophilia A, 16 (16.7Β %) – hemophilia B, 5 (5.2Β %) – hemophilia with inhibitor form. All patients were with severe hemophilic arthropathyand and underwent knee or hip replacement. The average age of patients was 39.6 years. All patients were examined for markers of thrombophilia.Β Results. Ninety three patients had either a heterozygous or homozygous form of thrombophilia marker polymorphism. One of thrombophilia markers was present in 15 (15.6Β %) patients and in 78 (81.3Β %) there was a combination of several markers. In patients with hemophilia B homozygous mutations in the MTHFR gene (A1298C and C677T) were more than 2 times more frequent than in patients with hemophilia A.Β Conclusion.Β The frequency of occurrence of polymorphism of FV (G1691A), MTHFR (C677T) and PAI-1 in the studied group of patients with hemophilia is higher than in the general Russian population

    Defects

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