274 research outputs found

    Paleoecological and permafrost studies of Ice Complex in the Laptev Sea area (Bykovsky Peninsula)

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    Chemical composition of seeds and green beans of common bean varieties, breeded in Omsk State Agrarian University under conditions of southern forest-steppe zone of Western Siberia

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    ArticleThe article considers the biochemical composition of green beans and seeds of common beans varieties, breeded in Omsk State Agrarian University named after P.A. Stolypin (OmSAU). The research was conducted in 2014–2016. Varieties of locally breeded beans, in comparison with the standards, have advantages in the content of protein, zinc, iodine, calcium, iron, sugar; green bean technological properties and tenderizing of seeds during cooking, which becomes an indispensable component of the diet

    Familial Mediterranean fever in Russia: Experience of the Federal Rheumatology Center

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    The paper gives the experience of the V.A. Nasonova Research Institute of Rheumatology in identifying and managing patients with familial Mediterranean fever (FMF). Objective: to describe the features of the disease in patients with FMF in Russia and to compare them with the data obtained in the study of Turkish and Armenian populations with a high incidence of this disease. Patients and methods. The investigation enrolled 23 patients with a documented diagnosis of FMF who met the Turkish pediatric criteria (F. Yalcinkaya et al.) and/or the criteria described by A. Livnech et al. and had two identical mutations (homozygosity) or two different mutations (compound heterozygosity) in the MEFV gene. Among the patients there were 9 men and 14 women. The age of the patients at the time of treatment was 4.5 to 36 years. Their age at onset of FMF was 2 months to 15 years (mean age, 3.2Β±2.3 years). Results. The examination established that 18 (78.3%) patients were Armenians, 3 were representatives of the North Caucasus peoples (a Darghin woman, an Avar man, and an Ingush/Kabardian man), a man from mixed (Greek/Georgian) marriage, and a Russian woman whose ancestors were Armenian and Jewish. The most common manifestation of FMF were recurrent episodes of fever (22; 95.7%); abdominal pain with fever ranked second (19; 82.6%), followed by chest pain (11; 47.8%), locomotor apparatus lesion (16; 69.6%), and skin lesions (7; 30.4%). The episodes were accompanied by increasedΒ levels of acutephase markers in 100% of the patients. There were a high proportion of patients, in whom FMF was concurrent with other rheumatic and autoinflammatory diseases (juvenile chronic arthritis, chronic recurrent multifocal osteomyelitis, and acute rheumatic fever) (7; 30.4%). Twenty-two (95.6%) patients received colchicine; the tumor necrosis factor inhibitor etanercept was prescribed in 2 (8.7%) patients with comorbidity; there was a pronounced therapeutic effect. Conclusion. Although the majority of our patients were Armenians, the disease was also identified in those belonging to the peoples of the North Caucasus and Transcaucasia and in one Russian female patient. There has generally been a fairly typical picture of the disease. The considerable frequency of joint damage and the high proportion of patients with comorbid rheumatic diseases have engaged our attention. From a diagnostic point of view, of importance is the elevated level of acutephase markers during a disease attack, which in combination with the clinical and ethnic characteristics of patients allows the diagnosis of FMF to be suspected

    Superbase ionic liquids for effective cellulose processing from dissolution to carbonisation

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    This is the author accepted manuscript. The final version is available from Royal Society of Chemistry via the DOI in this recordA range of superbase derived ionic liquids (SILs) was synthesised and characterised. Their ability to dissolve cellulose and the characteristics of the produced fibres were correlated to their specific structural and solvent properties. 17 ionic liquids (ILs) (including 9 novel) were analysed and six ILs were selected to produce fibres: 1-ethyl-3-methylimidazolium acetate [C2C1im][OAc], 1-ethyl-3-methylimidazolium diethyl phosphate [C2C1im][DEP] and the SILs 1-ethyl-1,8-diazabicyclo[5.4.0]undec-7-enium diethylphosphate [DBUEt][DEP], 1,8-diazabicyclo[5.4.0]undec-7-enium acetate [DBUH][OAc], 1,5-diazabicyclo[4.3.0]non-5-enium acetate [DBNH][OAc] and 1-ethyl-1,5-diazabicyclo[4.3.0]non-5-enium diethylphsophate [DBNEt][DEP]. The mechanical properties of these fibres were investigated. The obtained fibres were then carbonised to explore possible application as carbon fibre precursors. The fibres obtained using a mixture of 1,5-diazabicyclo[4.3.0]non-5-enium based SILs with acetate and hexanoate anions (9 : 1), [DBNH][OAc][Hex], showed a promising combination of strength, stiffness and strain at failure values for applications in textiles and fibre reinforcement in renewable composites. Using Raman spectroscopy it is demonstrated that these fibres exhibit a relatively high degree of structural order, with fewer defects than the other materials. On the other hand, analogous fibres based on imidazolium cation with acetate and hexanoate anions (9 : 1), [C2C1im][OAc][Hex] showed a decline in the quality of the produced fibres compared to the fibres produced from [C2C1im][OAc], [C2C1im][DEP] or [DBNH][OAc][Hex].We would like to thank the EPSRC grant number EP/L017679/01 for financial support (AERO RB1717)

    Architectural and Spiritual Monuments of the City in the Fate of Human

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    The article presents the biography of the architect and restorer, Anatoly Andreyevich Kuzmin, who devoted his whole life to preserving the spiritual heritage of the city of Nizhny Tagil – temple buildings. The story is told by the artist's granddaughter.Π’ ΡΡ‚Π°Ρ‚ΡŒΠ΅ прСдставлСна биография Π°Ρ€Ρ…ΠΈΡ‚Π΅ΠΊΡ‚ΠΎΡ€Π° ΠΈ рСставратора Анатолия АндрССвича ΠšΡƒΠ·ΡŒΠΌΠΈΠ½Π°, ΠΏΠΎΡΠ²ΡΡ‚ΠΈΠ²ΡˆΠ΅Π³ΠΎ всю Тизнь ΡΠΎΡ…Ρ€Π°Π½Π΅Π½ΠΈΡŽ Π΄ΡƒΡ…ΠΎΠ²Π½ΠΎΠ³ΠΎ наслСдия Π³ΠΎΡ€ΠΎΠ΄Π° НиТнСго Π’Π°Π³ΠΈΠ»Π° – Ρ…Ρ€Π°ΠΌΠΎΠ²Ρ‹ΠΌ постройкам. Π˜ΡΡ‚ΠΎΡ€ΠΈΡ рассказана Π²Π½ΡƒΡ‡ΠΊΠΎΠΉ Ρ…ΡƒΠ΄ΠΎΠΆΠ½ΠΈΠΊΠ°

    THE ROLE OF CLOPIDOGREL IN PATIENTS WITH ACUTE CORONARY SYNDROME AFTER THE EMERGENCE OF MORE POWERFUL INHIBITORS OF P2Y12 RECEPTOR

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    The role of P2Y12 receptor blocker clopidogrel after the introduction into clinical practice of new, more powerful and stable operating drugsΒ belonging to this class of antiplatelet agents is discussed. The advantages and disadvantages of each of the currently used antiplatelet drugsΒ that block the receptor P2Y12 are reviewed. On the basis of the analysis concludes that, despite the emergence of new antiplatelet agents clopidogrel,Β appears to be widely used for a long time in the treatment of patients with acute coronary syndrome and / or after coronary stenting.Β This is primarily due to the presence of large evidence base, and confirmation of safety of long-term therapy clopidogrel.</p

    БСмСйная срСдизСмноморская Π»ΠΈΡ…ΠΎΡ€Π°Π΄ΠΊΠ° Π² России (ΠΎΠΏΡ‹Ρ‚ Ρ€Π°Π±ΠΎΡ‚Ρ‹ Π€Π΅Π΄Π΅Ρ€Π°Π»ΡŒΠ½ΠΎΠ³ΠΎ рСвматологичСского Ρ†Π΅Π½Ρ‚Ρ€Π°)

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    The paper gives the experience of the V.A. Nasonova Research Institute of Rheumatology in identifying and managing patients with familial Mediterranean fever (FMF). Objective: to describe the features of the disease in patients with FMF in Russia and to compare them with the data obtained in the study of Turkish and Armenian populations with a high incidence of this disease. Patients and methods. The investigation enrolled 23 patients with a documented diagnosis of FMF who met the Turkish pediatric criteria (F. Yalcinkaya et al.) and/or the criteria described by A. Livnech et al. and had two identical mutations (homozygosity) or two different mutations (compound heterozygosity) in the MEFV gene. Among the patients there were 9 men and 14 women. The age of the patients at the time of treatment was 4.5 to 36 years. Their age at onset of FMF was 2 months to 15 years (mean age, 3.2Β±2.3 years). Results. The examination established that 18 (78.3%) patients were Armenians, 3 were representatives of the North Caucasus peoples (a Darghin woman, an Avar man, and an Ingush/Kabardian man), a man from mixed (Greek/Georgian) marriage, and a Russian woman whose ancestors were Armenian and Jewish. The most common manifestation of FMF were recurrent episodes of fever (22; 95.7%); abdominal pain with fever ranked second (19; 82.6%), followed by chest pain (11; 47.8%), locomotor apparatus lesion (16; 69.6%), and skin lesions (7; 30.4%). The episodes were accompanied by increasedΒ levels of acutephase markers in 100% of the patients. There were a high proportion of patients, in whom FMF was concurrent with other rheumatic and autoinflammatory diseases (juvenile chronic arthritis, chronic recurrent multifocal osteomyelitis, and acute rheumatic fever) (7; 30.4%). Twenty-two (95.6%) patients received colchicine; the tumor necrosis factor inhibitor etanercept was prescribed in 2 (8.7%) patients with comorbidity; there was a pronounced therapeutic effect. Conclusion. Although the majority of our patients were Armenians, the disease was also identified in those belonging to the peoples of the North Caucasus and Transcaucasia and in one Russian female patient. There has generally been a fairly typical picture of the disease. The considerable frequency of joint damage and the high proportion of patients with comorbid rheumatic diseases have engaged our attention. From a diagnostic point of view, of importance is the elevated level of acutephase markers during a disease attack, which in combination with the clinical and ethnic characteristics of patients allows the diagnosis of FMF to be suspected.Β ΠŸΡ€Π΅Π΄ΡΡ‚Π°Π²Π»Π΅Π½ ΠΎΠΏΡ‹Ρ‚ ЀГБНУ «Научно-ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Ρ‚Π΅Π»ΡŒΡΠΊΠΈΠΉ институт Ρ€Π΅Π²ΠΌΠ°Ρ‚ΠΎΠ»ΠΎΠ³ΠΈΠΈ ΠΈΠΌ. Π’.А. Насоновой» ΠΏΠΎ Π²Ρ‹ΡΠ²Π»Π΅Π½ΠΈΡŽ ΠΈ вСдСнию ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ² с сСмСйной срСдизСмноморской Π»ΠΈΡ…ΠΎΡ€Π°Π΄ΠΊΠΎΠΉ – Π‘Π‘Π› (Familial Mediterranean Fever, FMF). ЦСль исслСдования – ΠΎΠΏΠΈΡΠ°Ρ‚ΡŒ особСнности тСчСния заболСвания Ρƒ ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ² с Π‘Π‘Π› Π² России ΠΈ ΡΠΎΠΏΠΎΡΡ‚Π°Π²ΠΈΡ‚ΡŒ ΠΈΡ… с Π΄Π°Π½Π½Ρ‹ΠΌΠΈ, ΠΏΠΎΠ»ΡƒΡ‡Π΅Π½Π½Ρ‹ΠΌΠΈ ΠΏΡ€ΠΈ ΠΈΠ·ΡƒΡ‡Π΅Π½ΠΈΠΈ Ρ‚ΡƒΡ€Π΅Ρ†ΠΊΠΎΠΉ ΠΈ армянской популяций, Π² ΠΊΠΎΡ‚ΠΎΡ€Ρ‹Ρ… Π½Π°Π±Π»ΡŽΠ΄Π°Π΅Ρ‚ΡΡ высокая частота этого заболСвания. ΠŸΠ°Ρ†ΠΈΠ΅Π½Ρ‚Ρ‹ ΠΈ ΠΌΠ΅Ρ‚ΠΎΠ΄Ρ‹. Π’ исслСдованиС Π²ΠΊΠ»ΡŽΡ‡Π΅Π½ΠΎ 23 ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚Π° с достовСрным Π΄ΠΈΠ°Π³Π½ΠΎΠ·ΠΎΠΌ Π‘Π‘Π›, ΡƒΠ΄ΠΎΠ²Π»Π΅Ρ‚Π²ΠΎΡ€ΡΠ²ΡˆΠΈΡ… Ρ‚ΡƒΡ€Π΅Ρ†ΠΊΠΈΠΌ пСдиатричСским критСриям (F. Yalcinkaya ΠΈ соавт.) ΠΈ/ΠΈΠ»ΠΈ критСриям А. Livnech ΠΈ соавт. ΠΈ ΠΈΠΌΠ΅Π²ΡˆΠΈΡ… Π΄Π²Π΅ ΠΎΠ΄ΠΈΠ½Π°ΠΊΠΎΠ²Ρ‹Π΅ ΠΌΡƒΡ‚Π°Ρ†ΠΈΠΈ (Π³ΠΎΠΌΠΎΠ·ΠΈΠ³ΠΎΡ‚Π½ΠΎΡΡ‚ΡŒ) ΠΈΠ»ΠΈ Π΄Π²Π΅ Ρ€Π°Π·Π½Ρ‹Π΅ ΠΌΡƒΡ‚Π°Ρ†ΠΈΠΈ (ΠΊΠΎΠΌΠΏΠ°ΡƒΠ½Π΄-Π³Π΅Ρ‚Π΅Ρ€ΠΎΠ·ΠΈΠ³ΠΎΡ‚Π½ΠΎΡΡ‚ΡŒ) Π³Π΅Π½Π° MEFV. ΠŸΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ² муТского ΠΏΠΎΠ»Π° Π±Ρ‹Π»ΠΎ 9, ТСнского ΠΏΠΎΠ»Π° – 14. Возраст Π±ΠΎΠ»ΡŒΠ½Ρ‹Ρ… Π½Π° ΠΌΠΎΠΌΠ΅Π½Ρ‚ обращСния составил ΠΎΡ‚ 4,5 Π΄ΠΎ 36 Π»Π΅Ρ‚. Возраст Π΄Π΅Π±ΡŽΡ‚Π° Π‘Π‘Π› – ΠΎΡ‚ 2 мСс Π΄ΠΎ 15 Π»Π΅Ρ‚ (Π² срСднСм – 3,2Β±2,3 Π³ΠΎΠ΄Π°). Π Π΅Π·ΡƒΠ»ΡŒΡ‚Π°Ρ‚Ρ‹. ΠŸΡ€ΠΈ обслСдовании установлСно, Ρ‡Ρ‚ΠΎ 18 (78,3%) ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ² Π±Ρ‹Π»ΠΈ армянами, 3 – прСдставитСлями Π½Π°Ρ€ΠΎΠ΄ΠΎΠ² Π‘Π΅Π²Π΅Ρ€Π½ΠΎΠ³ΠΎ Кавказа (1 – Π΄Π°Ρ€Π³ΠΈΠ½ΠΊΠ°, 1 – Π°Π²Π°Ρ€Π΅Ρ†, 1 – ΠΈΠ½Π³ΡƒΡˆ/ΠΊΠ°Π±Π°Ρ€Π΄ΠΈΠ½Π΅Ρ†), 1 – Π³Ρ€Π΅ΠΊ/Π³Ρ€ΡƒΠ·ΠΈΠ½ ΠΈ 1 – русская, имСвшая ΠΏΡ€Π΅Π΄ΠΊΠΎΠ² армян ΠΈ Π΅Π²Ρ€Π΅Π΅Π². Π‘Π°ΠΌΡ‹ΠΌ частым проявлСниСм Π‘Π‘Π” Π±Ρ‹Π»ΠΈ ΠΏΠΎΠ²Ρ‚ΠΎΡ€ΡΡŽΡ‰ΠΈΠ΅ΡΡ эпизоды Π»ΠΈΡ…ΠΎΡ€Π°Π΄ΠΊΠΈ – 22 (95,7%) ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚Π°, Π²Ρ‚ΠΎΡ€ΠΎΠ΅ мСсто Π·Π°Π½ΠΈΠΌΠ°Π»Π° ΡΠΎΠΏΡ€ΠΎΠ²ΠΎΠΆΠ΄Π°Π²ΡˆΠ°Ρ Π»ΠΈΡ…ΠΎΡ€Π°Π΄ΠΊΡƒ боль Π² ΠΆΠΈΠ²ΠΎΡ‚Π΅ – 19 (82,6%), Π΄Π°Π»Π΅Π΅ слСдовали боль Π² Π³Ρ€ΡƒΠ΄Π½ΠΎΠΉ ΠΊΠ»Π΅Ρ‚ΠΊΠ΅ – 11 (47,8%), ΠΏΠΎΡ€Π°ΠΆΠ΅Π½ΠΈΠ΅ ΠΎΠΏΠΎΡ€Π½ΠΎ-Π΄Π²ΠΈΠ³Π°Ρ‚Π΅Π»ΡŒΠ½ΠΎΠ³ΠΎ Π°ΠΏΠΏΠ°Ρ€Π°Ρ‚Π° – 16 (69,6%), ΠΏΠΎΡ€Π°ΠΆΠ΅Π½ΠΈΠ΅ ΠΊΠΎΠΆΠΈ – 7 (30,4%). Π£ всСх ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ² Π°Ρ‚Π°ΠΊΠΈ ΡΠΎΠΏΡ€ΠΎΠ²ΠΎΠΆΠ΄Π°Π»ΠΈΡΡŒ ΠΏΠΎΠ²Ρ‹ΡˆΠ΅Π½ΠΈΠ΅ΠΌ уровня острофазовых ΠΏΠΎΠΊΠ°Π·Π°Ρ‚Π΅Π»Π΅ΠΉ. ΠžΡ‚ΠΌΠ΅Ρ‡Π΅Π½Π° высокая доля ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ², Ρƒ ΠΊΠΎΡ‚ΠΎΡ€Ρ‹Ρ… Π‘Π‘Π› ΡΠΎΡ‡Π΅Ρ‚Π°Π»Π°ΡΡŒ с Π΄Ρ€ΡƒΠ³ΠΈΠΌΠΈ рСвматичСскими ΠΈ Π°ΡƒΡ‚ΠΎΠ²ΠΎΡΠΏΠ°Π»ΠΈΡ‚Π΅Π»ΡŒΠ½Ρ‹ΠΌΠΈ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΠΌΠΈ (ΡŽΠ²Π΅Π½ΠΈΠ»ΡŒΠ½Ρ‹ΠΌ хроничСским Π°Ρ€Ρ‚Ρ€ΠΈΡ‚ΠΎΠΌ, хроничСским Ρ€Π΅Ρ†ΠΈΠ΄ΠΈΠ²ΠΈΡ€ΡƒΡŽΡ‰ΠΈΠΌ ΠΌΡƒΠ»ΡŒΡ‚ΠΈΡ„ΠΎΠΊΠ°Π»ΡŒΠ½Ρ‹ΠΌ остСомиСлитом, острой рСвматичСской Π»ΠΈΡ…ΠΎΡ€Π°Π΄ΠΊΠΎΠΉ) – 7 (30,4%). 22 (95,6%) ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚Π° ΠΏΠΎΠ»ΡƒΡ‡Π°Π»ΠΈ ΠΊΠΎΠ»Ρ…ΠΈΡ†ΠΈΠ½. 2 (8,7%) Π±ΠΎΠ»ΡŒΠ½Ρ‹ΠΌ с ΠΊΠΎΠΌΠΎΡ€Π±ΠΈΠ΄Π½ΠΎΠΉ ΠΏΠ°Ρ‚ΠΎΠ»ΠΎΠ³ΠΈΠ΅ΠΉ Π±Ρ‹Π» Π½Π°Π·Π½Π°Ρ‡Π΅Π½ ΠΈΠ½Π³ΠΈΠ±ΠΈΡ‚ΠΎΡ€ Ρ„Π°ΠΊΡ‚ΠΎΡ€Π° Π½Π΅ΠΊΡ€ΠΎΠ·Π° ΠΎΠΏΡƒΡ…ΠΎΠ»ΠΈ этанСрцСпт, отмСчался Π²Ρ‹Ρ€Π°ΠΆΠ΅Π½Π½Ρ‹ΠΉ тСрапСвтичСский эффСкт. Π’Ρ‹Π²ΠΎΠ΄Ρ‹. Π₯отя Π±ΠΎΠ»ΡŒΡˆΠΈΠ½ΡΡ‚Π²ΠΎ Π½Π°ΡˆΠΈΡ… ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ² Π±Ρ‹Π»ΠΈ армянС, Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΠ΅ выявлСно Ρ‚Π°ΠΊΠΆΠ΅ Ρƒ Π»ΠΈΡ†, относящихся ΠΊ Π½Π°Ρ€ΠΎΠ΄Π°ΠΌ Π‘Π΅Π²Π΅Ρ€Π½ΠΎΠ³ΠΎ Кавказа ΠΈ Π—Π°ΠΊΠ°Π²ΠΊΠ°Π·ΡŒΡ, ΠΈ 1 русской ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΊΠΈ. Π’ Ρ†Π΅Π»ΠΎΠΌ наблюдалась достаточно типичная ΠΊΠ°Ρ€Ρ‚ΠΈΠ½Π° заболСвания. ΠžΠ±Ρ€Π°Ρ‰Π°Π»ΠΈ Π½Π° сСбя Π²Π½ΠΈΠΌΠ°Π½ΠΈΠ΅ Π·Π½Π°Ρ‡ΠΈΡ‚Π΅Π»ΡŒΠ½Π°Ρ частота пораТСния суставов ΠΈ высокая доля ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ² с ΠΊΠΎΠΌΠΎΡ€Π±ΠΈΠ΄Π½ΠΎΠΉ рСвматичСской ΠΏΠ°Ρ‚ΠΎΠ»ΠΎΠ³ΠΈΠ΅ΠΉ. Π’Π°ΠΆΠ½Ρ‹ΠΌ с диагностичСской Ρ‚ΠΎΡ‡ΠΊΠΈ зрСния являСтся ΠΏΠΎΠ²Ρ‹ΡˆΠ΅Π½ΠΈΠ΅ уровня острофазовых ΠΌΠ°Ρ€ΠΊΠ΅Ρ€ΠΎΠ² Π²ΠΎ врСмя Π°Ρ‚Π°ΠΊΠΈ заболСвания, Ρ‡Ρ‚ΠΎ Π² сочСтании с клиничСской ΠΊΠ°Ρ€Ρ‚ΠΈΠ½ΠΎΠΉ ΠΈ этничСской характСристикой ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ² позволяСт Π·Π°ΠΏΠΎΠ΄ΠΎΠ·Ρ€ΠΈΡ‚ΡŒ Π΄ΠΈΠ°Π³Π½ΠΎΠ· Π‘Π‘Π›.Β 

    Π‘ΠΎΠ²Ρ€Π΅ΠΌΠ΅Π½Π½Ρ‹Π΅ ΠΏΠΎΠ΄Ρ…ΠΎΠ΄Ρ‹ ΠΊ диагностикС , Π»Π΅Ρ‡Π΅Π½ΠΈΡŽ ΠΈ ΠΌΠΎΠ½ΠΈΡ‚ΠΎΡ€ΠΈΠ½Π³Ρƒ ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎ Π² с ΠΊΡ€ΠΈΠΎΠΏΠΈΡ€ΠΈΠ½-ассоциированными пСриодичСскими синдромами (CAPS)

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    Cryopyrin-associated periodic syndromes (CAPS) are the most prominent representatives of monogenic autoinflammatory diseases (AIDs). TheyΒ are characterized by a concurrence of fever, rash, and other symptoms that are accompanied by disabling or life-threatening complications. BasedΒ on the current guidelines for the management of patients with AIDs and on their own experience, the authors have elaborated a diagnostic algorithmΒ for CAPS, which can optimize their differential diagnosis with systemic juvenile arthritis and other AIDs and the early use of effective therapyΒ and management tactics. Interleukin-1 inhibitors are target agents, by which one can monitor activity and prevent complications. The givenΒ algorithm will be able to improve the timely diagnosis of CAPS and to optimize treat-to-target treatment and management in patients.ΠšΡ€ΠΈΠΎΠΏΠΈΡ€ΠΈΠ½-ассоциированныС пСриодичСскиС синдромы (Cryopyrin-Associated Periodic Syndromes, CAPS) ΡΠ²Π»ΡΡŽΡ‚ΡΡ Π½Π°ΠΈΠ±ΠΎΠ»Π΅Π΅ яркими прСдставитСлями ΠΌΠΎΠ½ΠΎΠ³Π΅Π½Π½Ρ‹Ρ… Π°ΡƒΡ‚ΠΎΠ²ΠΎΡΠΏΠ°Π»ΠΈΡ‚Π΅Π»ΡŒΠ½Ρ‹Ρ… Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΠΉ (АВЗ). Они Ρ…Π°Ρ€Π°ΠΊΡ‚Π΅Ρ€ΠΈΠ·ΡƒΡŽΡ‚ΡΡ сочСтаниСм Π»ΠΈΡ…ΠΎΡ€Π°Π΄ΠΊΠΈ, сыпи ΠΈ Π΄Ρ€ΡƒΠ³ΠΈΡ… симптомов, ΡΠΎΠΏΡ€ΠΎΠ²ΠΎΠΆΠ΄Π°ΡŽΡ‰ΠΈΡ…ΡΡ Ρ„ΠΎΡ€ΠΌΠΈΡ€ΠΎΠ²Π°Π½ΠΈΠ΅ΠΌ ΠΏΠΎΡ‚Π΅Π½Ρ†ΠΈΠ°Π»ΡŒΠ½ΠΎ ΠΈΠ½Π²Π°Π»ΠΈΠ΄ΠΈΠ·ΠΈΡ€ΡƒΡŽΡ‰ΠΈΡ… ΠΈΠ»ΠΈ ΠΆΠΈΠ·Π½Π΅ΡƒΠ³Ρ€ΠΎΠΆΠ°ΡŽΡ‰ΠΈΡ… ослоТнСний. На основС соврСмСнных Ρ€Π΅ΠΊΠΎΠΌΠ΅Π½Π΄Π°Ρ†ΠΈΠΉ ΠΏΠΎ вСдСнию ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ² с АВЗ, Π° Ρ‚Π°ΠΊΠΆΠ΅ нашСго собствСнного ΠΎΠΏΡ‹Ρ‚Π° Ρ€Π°Π·Ρ€Π°Π±ΠΎΡ‚Π°Π½ Π°Π»Π³ΠΎΡ€ΠΈΡ‚ΠΌ диагностики CAPS, ΠΏΠΎΠ·Π²ΠΎΠ»ΡΡŽΡ‰ΠΈΠΉ ΠΎΠΏΡ‚ΠΈΠΌΠΈΠ·ΠΈΡ€ΠΎΠ²Π°Ρ‚ΡŒ ΠΈΡ… Π΄ΠΈΡ„Ρ„Π΅Ρ€Π΅Π½Ρ†ΠΈΠ°Π»ΡŒΠ½ΡƒΡŽ диагностику с систСмным ΡŽΠ²Π΅Π½ΠΈΠ»ΡŒΠ½Ρ‹ΠΌ Π°Ρ€Ρ‚Ρ€ΠΈΡ‚ΠΎΠΌ ΠΈ Π΄Ρ€ΡƒΠ³ΠΈΠΌΠΈ АВЗ, Ρ€Π°Π½Π½Π΅Π΅ Π½Π°Π·Π½Π°Ρ‡Π΅Π½ΠΈΠ΅ эффСктивной Ρ‚Π΅Ρ€Π°ΠΏΠΈΠΈ, Π° Ρ‚Π°ΠΊΠΆΠ΅ Ρ‚Π°ΠΊΡ‚ΠΈΠΊΡƒ вСдСния ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ². Π˜Π½Π³ΠΈΠ±ΠΈΡ‚ΠΎΡ€Ρ‹ ΠΈΠ½Ρ‚Π΅Ρ€Π»Π΅ΠΉΠΊΠΈΠ½Π° 1 ΡΠ²Π»ΡΡŽΡ‚ΡΡ Ρ‚Π°Ρ€Π³Π΅Ρ‚Π½Ρ‹ΠΌΠΈ срСдствами, ΡΒ ΠΏΠΎΠΌΠΎΡ‰ΡŒΡŽ ΠΊΠΎΡ‚ΠΎΡ€Ρ‹Ρ… ΠΌΠΎΠΆΠ½ΠΎ ΠΊΠΎΠ½Ρ‚Ρ€ΠΎΠ»ΠΈΡ€ΠΎΠ²Π°Ρ‚ΡŒ Π°ΠΊΡ‚ΠΈΠ²Π½ΠΎΡΡ‚ΡŒ ΠΈ ΠΏΡ€Π΅Π΄ΡƒΠΏΡ€Π΅ΠΆΠ΄Π°Ρ‚ΡŒ Ρ€Π°Π·Π²ΠΈΡ‚ΠΈΠ΅ ослоТнСний. ΠŸΡ€Π΅Π΄ΡΡ‚Π°Π²Π»Π΅Π½Π½Ρ‹ΠΉ Π°Π»Π³ΠΎΡ€ΠΈΡ‚ΠΌ ΠΏΠΎΠ·Π²ΠΎΠ»ΠΈΡ‚Β ΡƒΠ»ΡƒΡ‡ΡˆΠΈΡ‚ΡŒ ΡΠ²ΠΎΠ΅Π²Ρ€Π΅ΠΌΠ΅Π½Π½ΡƒΡŽ диагностику CAPS, ΠΎΠΏΡ‚ΠΈΠΌΠΈΠ·ΠΈΡ€ΠΎΠ²Π°Ρ‚ΡŒ Π»Π΅Ρ‡Π΅Π½ΠΈΠ΅ ΠΈ Π²Π΅Π΄Π΅Π½ΠΈΠ΅ ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ² Π² Ρ€Π°ΠΌΠΊΠ°Ρ… стратСгии Β«Treat to targetΒ»
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