274 research outputs found
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
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 2014β2016. 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
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
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 (9β:β1), [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 (9β:β1), [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
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 β temple buildings. The story is told by the artist's granddaughter.Π ΡΡΠ°ΡΡΠ΅ ΠΏΡΠ΅Π΄ΡΡΠ°Π²Π»Π΅Π½Π° Π±ΠΈΠΎΠ³ΡΠ°ΡΠΈΡ Π°ΡΡ
ΠΈΡΠ΅ΠΊΡΠΎΡΠ° ΠΈ ΡΠ΅ΡΡΠ°Π²ΡΠ°ΡΠΎΡΠ° ΠΠ½Π°ΡΠΎΠ»ΠΈΡ ΠΠ½Π΄ΡΠ΅Π΅Π²ΠΈΡΠ° ΠΡΠ·ΡΠΌΠΈΠ½Π°, ΠΏΠΎΡΠ²ΡΡΠΈΠ²ΡΠ΅Π³ΠΎ Π²ΡΡ ΠΆΠΈΠ·Π½Ρ ΡΠΎΡ
ΡΠ°Π½Π΅Π½ΠΈΡ Π΄ΡΡ
ΠΎΠ²Π½ΠΎΠ³ΠΎ Π½Π°ΡΠ»Π΅Π΄ΠΈΡ Π³ΠΎΡΠΎΠ΄Π° ΠΠΈΠΆΠ½Π΅Π³ΠΎ Π’Π°Π³ΠΈΠ»Π° β Ρ
ΡΠ°ΠΌΠΎΠ²ΡΠΌ ΠΏΠΎΡΡΡΠΎΠΉΠΊΠ°ΠΌ. ΠΡΡΠΎΡΠΈΡ ΡΠ°ΡΡΠΊΠ°Π·Π°Π½Π° Π²Π½ΡΡΠΊΠΎΠΉ Ρ
ΡΠ΄ΠΎΠΆΠ½ΠΈΠΊΠ°
THE ROLE OF CLOPIDOGREL IN PATIENTS WITH ACUTE CORONARY SYNDROME AFTER THE EMERGENCE OF MORE POWERFUL INHIBITORS OF P2Y12 RECEPTOR
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
Π‘Π΅ΠΌΠ΅ΠΉΠ½Π°Ρ ΡΡΠ΅Π΄ΠΈΠ·Π΅ΠΌΠ½ΠΎΠΌΠΎΡΡΠΊΠ°Ρ Π»ΠΈΡ ΠΎΡΠ°Π΄ΠΊΠ° Π² Π ΠΎΡΡΠΈΠΈ (ΠΎΠΏΡΡ ΡΠ°Π±ΠΎΡΡ Π€Π΅Π΄Π΅ΡΠ°Π»ΡΠ½ΠΎΠ³ΠΎ ΡΠ΅Π²ΠΌΠ°ΡΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠΎΠ³ΠΎ ΡΠ΅Π½ΡΡΠ°)
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 ΠΡΠ΅Π΄ΡΡΠ°Π²Π»Π΅Π½ ΠΎΠΏΡΡ Π€ΠΠΠΠ£ Β«ΠΠ°ΡΡΠ½ΠΎ-ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°ΡΠ΅Π»ΡΡΠΊΠΈΠΉ ΠΈΠ½ΡΡΠΈΡΡΡ ΡΠ΅Π²ΠΌΠ°ΡΠΎΠ»ΠΎΠ³ΠΈΠΈ ΠΈΠΌ. Π.Π. ΠΠ°ΡΠΎΠ½ΠΎΠ²ΠΎΠΉΒ» ΠΏΠΎ Π²ΡΡΠ²Π»Π΅Π½ΠΈΡ ΠΈ Π²Π΅Π΄Π΅Π½ΠΈΡ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Ρ ΡΠ΅ΠΌΠ΅ΠΉΠ½ΠΎΠΉ ΡΡΠ΅Π΄ΠΈΠ·Π΅ΠΌΠ½ΠΎΠΌΠΎΡΡΠΊΠΎΠΉ Π»ΠΈΡ
ΠΎΡΠ°Π΄ΠΊΠΎΠΉ β Π‘Π‘Π (Familial Mediterranean Fever, FMF). Π¦Π΅Π»Ρ ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΡ β ΠΎΠΏΠΈΡΠ°ΡΡ ΠΎΡΠΎΠ±Π΅Π½Π½ΠΎΡΡΠΈ ΡΠ΅ΡΠ΅Π½ΠΈΡ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΡ Ρ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Ρ Π‘Π‘Π Π² Π ΠΎΡΡΠΈΠΈ ΠΈ ΡΠΎΠΏΠΎΡΡΠ°Π²ΠΈΡΡ ΠΈΡ
Ρ Π΄Π°Π½Π½ΡΠΌΠΈ, ΠΏΠΎΠ»ΡΡΠ΅Π½Π½ΡΠΌΠΈ ΠΏΡΠΈ ΠΈΠ·ΡΡΠ΅Π½ΠΈΠΈ ΡΡΡΠ΅ΡΠΊΠΎΠΉ ΠΈ Π°ΡΠΌΡΠ½ΡΠΊΠΎΠΉ ΠΏΠΎΠΏΡΠ»ΡΡΠΈΠΉ, Π² ΠΊΠΎΡΠΎΡΡΡ
Π½Π°Π±Π»ΡΠ΄Π°Π΅ΡΡΡ Π²ΡΡΠΎΠΊΠ°Ρ ΡΠ°ΡΡΠΎΡΠ° ΡΡΠΎΠ³ΠΎ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΡ. ΠΠ°ΡΠΈΠ΅Π½ΡΡ ΠΈ ΠΌΠ΅ΡΠΎΠ΄Ρ. Π ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠ΅ Π²ΠΊΠ»ΡΡΠ΅Π½ΠΎ 23 ΠΏΠ°ΡΠΈΠ΅Π½ΡΠ° Ρ Π΄ΠΎΡΡΠΎΠ²Π΅ΡΠ½ΡΠΌ Π΄ΠΈΠ°Π³Π½ΠΎΠ·ΠΎΠΌ Π‘Π‘Π, ΡΠ΄ΠΎΠ²Π»Π΅ΡΠ²ΠΎΡΡΠ²ΡΠΈΡ
ΡΡΡΠ΅ΡΠΊΠΈΠΌ ΠΏΠ΅Π΄ΠΈΠ°ΡΡΠΈΡΠ΅ΡΠΊΠΈΠΌ ΠΊΡΠΈΡΠ΅ΡΠΈΡΠΌ (F. Yalcinkaya ΠΈ ΡΠΎΠ°Π²Ρ.) ΠΈ/ΠΈΠ»ΠΈ ΠΊΡΠΈΡΠ΅ΡΠΈΡΠΌ Π. Livnech ΠΈ ΡΠΎΠ°Π²Ρ. ΠΈ ΠΈΠΌΠ΅Π²ΡΠΈΡ
Π΄Π²Π΅ ΠΎΠ΄ΠΈΠ½Π°ΠΊΠΎΠ²ΡΠ΅ ΠΌΡΡΠ°ΡΠΈΠΈ (Π³ΠΎΠΌΠΎΠ·ΠΈΠ³ΠΎΡΠ½ΠΎΡΡΡ) ΠΈΠ»ΠΈ Π΄Π²Π΅ ΡΠ°Π·Π½ΡΠ΅ ΠΌΡΡΠ°ΡΠΈΠΈ (ΠΊΠΎΠΌΠΏΠ°ΡΠ½Π΄-Π³Π΅ΡΠ΅ΡΠΎΠ·ΠΈΠ³ΠΎΡΠ½ΠΎΡΡΡ) Π³Π΅Π½Π° MEFV. ΠΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² ΠΌΡΠΆΡΠΊΠΎΠ³ΠΎ ΠΏΠΎΠ»Π° Π±ΡΠ»ΠΎ 9, ΠΆΠ΅Π½ΡΠΊΠΎΠ³ΠΎ ΠΏΠΎΠ»Π° β 14. ΠΠΎΠ·ΡΠ°ΡΡ Π±ΠΎΠ»ΡΠ½ΡΡ
Π½Π° ΠΌΠΎΠΌΠ΅Π½Ρ ΠΎΠ±ΡΠ°ΡΠ΅Π½ΠΈΡ ΡΠΎΡΡΠ°Π²ΠΈΠ» ΠΎΡ 4,5 Π΄ΠΎ 36 Π»Π΅Ρ. ΠΠΎΠ·ΡΠ°ΡΡ Π΄Π΅Π±ΡΡΠ° Π‘Π‘Π β ΠΎΡ 2 ΠΌΠ΅Ρ Π΄ΠΎ 15 Π»Π΅Ρ (Π² ΡΡΠ΅Π΄Π½Π΅ΠΌ β 3,2Β±2,3 Π³ΠΎΠ΄Π°). Π Π΅Π·ΡΠ»ΡΡΠ°ΡΡ. ΠΡΠΈ ΠΎΠ±ΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠΈ ΡΡΡΠ°Π½ΠΎΠ²Π»Π΅Π½ΠΎ, ΡΡΠΎ 18 (78,3%) ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Π±ΡΠ»ΠΈ Π°ΡΠΌΡΠ½Π°ΠΌΠΈ, 3 β ΠΏΡΠ΅Π΄ΡΡΠ°Π²ΠΈΡΠ΅Π»ΡΠΌΠΈ Π½Π°ΡΠΎΠ΄ΠΎΠ² Π‘Π΅Π²Π΅ΡΠ½ΠΎΠ³ΠΎ ΠΠ°Π²ΠΊΠ°Π·Π° (1 β Π΄Π°ΡΠ³ΠΈΠ½ΠΊΠ°, 1 β Π°Π²Π°ΡΠ΅Ρ, 1 β ΠΈΠ½Π³ΡΡ/ΠΊΠ°Π±Π°ΡΠ΄ΠΈΠ½Π΅Ρ), 1 β Π³ΡΠ΅ΠΊ/Π³ΡΡΠ·ΠΈΠ½ ΠΈ 1 β ΡΡΡΡΠΊΠ°Ρ, ΠΈΠΌΠ΅Π²ΡΠ°Ρ ΠΏΡΠ΅Π΄ΠΊΠΎΠ² Π°ΡΠΌΡΠ½ ΠΈ Π΅Π²ΡΠ΅Π΅Π². Π‘Π°ΠΌΡΠΌ ΡΠ°ΡΡΡΠΌ ΠΏΡΠΎΡΠ²Π»Π΅Π½ΠΈΠ΅ΠΌ Π‘Π‘Π Π±ΡΠ»ΠΈ ΠΏΠΎΠ²ΡΠΎΡΡΡΡΠΈΠ΅ΡΡ ΡΠΏΠΈΠ·ΠΎΠ΄Ρ Π»ΠΈΡ
ΠΎΡΠ°Π΄ΠΊΠΈ β 22 (95,7%) ΠΏΠ°ΡΠΈΠ΅Π½ΡΠ°, Π²ΡΠΎΡΠΎΠ΅ ΠΌΠ΅ΡΡΠΎ Π·Π°Π½ΠΈΠΌΠ°Π»Π° ΡΠΎΠΏΡΠΎΠ²ΠΎΠΆΠ΄Π°Π²ΡΠ°Ρ Π»ΠΈΡ
ΠΎΡΠ°Π΄ΠΊΡ Π±ΠΎΠ»Ρ Π² ΠΆΠΈΠ²ΠΎΡΠ΅ β 19 (82,6%), Π΄Π°Π»Π΅Π΅ ΡΠ»Π΅Π΄ΠΎΠ²Π°Π»ΠΈ Π±ΠΎΠ»Ρ Π² Π³ΡΡΠ΄Π½ΠΎΠΉ ΠΊΠ»Π΅ΡΠΊΠ΅ β 11 (47,8%), ΠΏΠΎΡΠ°ΠΆΠ΅Π½ΠΈΠ΅ ΠΎΠΏΠΎΡΠ½ΠΎ-Π΄Π²ΠΈΠ³Π°ΡΠ΅Π»ΡΠ½ΠΎΠ³ΠΎ Π°ΠΏΠΏΠ°ΡΠ°ΡΠ° β 16 (69,6%), ΠΏΠΎΡΠ°ΠΆΠ΅Π½ΠΈΠ΅ ΠΊΠΎΠΆΠΈ β 7 (30,4%). Π£ Π²ΡΠ΅Ρ
ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Π°ΡΠ°ΠΊΠΈ ΡΠΎΠΏΡΠΎΠ²ΠΎΠΆΠ΄Π°Π»ΠΈΡΡ ΠΏΠΎΠ²ΡΡΠ΅Π½ΠΈΠ΅ΠΌ ΡΡΠΎΠ²Π½Ρ ΠΎΡΡΡΠΎΡΠ°Π·ΠΎΠ²ΡΡ
ΠΏΠΎΠΊΠ°Π·Π°ΡΠ΅Π»Π΅ΠΉ. ΠΡΠΌΠ΅ΡΠ΅Π½Π° Π²ΡΡΠΎΠΊΠ°Ρ Π΄ΠΎΠ»Ρ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ², Ρ ΠΊΠΎΡΠΎΡΡΡ
Π‘Π‘Π ΡΠΎΡΠ΅ΡΠ°Π»Π°ΡΡ Ρ Π΄ΡΡΠ³ΠΈΠΌΠΈ ΡΠ΅Π²ΠΌΠ°ΡΠΈΡΠ΅ΡΠΊΠΈΠΌΠΈ ΠΈ Π°ΡΡΠΎΠ²ΠΎΡΠΏΠ°Π»ΠΈΡΠ΅Π»ΡΠ½ΡΠΌΠΈ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΠΌΠΈ (ΡΠ²Π΅Π½ΠΈΠ»ΡΠ½ΡΠΌ Ρ
ΡΠΎΠ½ΠΈΡΠ΅ΡΠΊΠΈΠΌ Π°ΡΡΡΠΈΡΠΎΠΌ, Ρ
ΡΠΎΠ½ΠΈΡΠ΅ΡΠΊΠΈΠΌ ΡΠ΅ΡΠΈΠ΄ΠΈΠ²ΠΈΡΡΡΡΠΈΠΌ ΠΌΡΠ»ΡΡΠΈΡΠΎΠΊΠ°Π»ΡΠ½ΡΠΌ ΠΎΡΡΠ΅ΠΎΠΌΠΈΠ΅Π»ΠΈΡΠΎΠΌ, ΠΎΡΡΡΠΎΠΉ ΡΠ΅Π²ΠΌΠ°ΡΠΈΡΠ΅ΡΠΊΠΎΠΉ Π»ΠΈΡ
ΠΎΡΠ°Π΄ΠΊΠΎΠΉ) β 7 (30,4%). 22 (95,6%) ΠΏΠ°ΡΠΈΠ΅Π½ΡΠ° ΠΏΠΎΠ»ΡΡΠ°Π»ΠΈ ΠΊΠΎΠ»Ρ
ΠΈΡΠΈΠ½. 2 (8,7%) Π±ΠΎΠ»ΡΠ½ΡΠΌ Ρ ΠΊΠΎΠΌΠΎΡΠ±ΠΈΠ΄Π½ΠΎΠΉ ΠΏΠ°ΡΠΎΠ»ΠΎΠ³ΠΈΠ΅ΠΉ Π±ΡΠ» Π½Π°Π·Π½Π°ΡΠ΅Π½ ΠΈΠ½Π³ΠΈΠ±ΠΈΡΠΎΡ ΡΠ°ΠΊΡΠΎΡΠ° Π½Π΅ΠΊΡΠΎΠ·Π° ΠΎΠΏΡΡ
ΠΎΠ»ΠΈ ΡΡΠ°Π½Π΅ΡΡΠ΅ΠΏΡ, ΠΎΡΠΌΠ΅ΡΠ°Π»ΡΡ Π²ΡΡΠ°ΠΆΠ΅Π½Π½ΡΠΉ ΡΠ΅ΡΠ°ΠΏΠ΅Π²ΡΠΈΡΠ΅ΡΠΊΠΈΠΉ ΡΡΡΠ΅ΠΊΡ. ΠΡΠ²ΠΎΠ΄Ρ. Π₯ΠΎΡΡ Π±ΠΎΠ»ΡΡΠΈΠ½ΡΡΠ²ΠΎ Π½Π°ΡΠΈΡ
ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Π±ΡΠ»ΠΈ Π°ΡΠΌΡΠ½Π΅, Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΠ΅ Π²ΡΡΠ²Π»Π΅Π½ΠΎ ΡΠ°ΠΊΠΆΠ΅ Ρ Π»ΠΈΡ, ΠΎΡΠ½ΠΎΡΡΡΠΈΡ
ΡΡ ΠΊ Π½Π°ΡΠΎΠ΄Π°ΠΌ Π‘Π΅Π²Π΅ΡΠ½ΠΎΠ³ΠΎ ΠΠ°Π²ΠΊΠ°Π·Π° ΠΈ ΠΠ°ΠΊΠ°Π²ΠΊΠ°Π·ΡΡ, ΠΈ 1 ΡΡΡΡΠΊΠΎΠΉ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΊΠΈ. Π ΡΠ΅Π»ΠΎΠΌ Π½Π°Π±Π»ΡΠ΄Π°Π»Π°ΡΡ Π΄ΠΎΡΡΠ°ΡΠΎΡΠ½ΠΎ ΡΠΈΠΏΠΈΡΠ½Π°Ρ ΠΊΠ°ΡΡΠΈΠ½Π° Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΡ. ΠΠ±ΡΠ°ΡΠ°Π»ΠΈ Π½Π° ΡΠ΅Π±Ρ Π²Π½ΠΈΠΌΠ°Π½ΠΈΠ΅ Π·Π½Π°ΡΠΈΡΠ΅Π»ΡΠ½Π°Ρ ΡΠ°ΡΡΠΎΡΠ° ΠΏΠΎΡΠ°ΠΆΠ΅Π½ΠΈΡ ΡΡΡΡΠ°Π²ΠΎΠ² ΠΈ Π²ΡΡΠΎΠΊΠ°Ρ Π΄ΠΎΠ»Ρ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Ρ ΠΊΠΎΠΌΠΎΡΠ±ΠΈΠ΄Π½ΠΎΠΉ ΡΠ΅Π²ΠΌΠ°ΡΠΈΡΠ΅ΡΠΊΠΎΠΉ ΠΏΠ°ΡΠΎΠ»ΠΎΠ³ΠΈΠ΅ΠΉ. ΠΠ°ΠΆΠ½ΡΠΌ Ρ Π΄ΠΈΠ°Π³Π½ΠΎΡΡΠΈΡΠ΅ΡΠΊΠΎΠΉ ΡΠΎΡΠΊΠΈ Π·ΡΠ΅Π½ΠΈΡ ΡΠ²Π»ΡΠ΅ΡΡΡ ΠΏΠΎΠ²ΡΡΠ΅Π½ΠΈΠ΅ ΡΡΠΎΠ²Π½Ρ ΠΎΡΡΡΠΎΡΠ°Π·ΠΎΠ²ΡΡ
ΠΌΠ°ΡΠΊΠ΅ΡΠΎΠ² Π²ΠΎ Π²ΡΠ΅ΠΌΡ Π°ΡΠ°ΠΊΠΈ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΡ, ΡΡΠΎ Π² ΡΠΎΡΠ΅ΡΠ°Π½ΠΈΠΈ Ρ ΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΎΠΉ ΠΊΠ°ΡΡΠΈΠ½ΠΎΠΉ ΠΈ ΡΡΠ½ΠΈΡΠ΅ΡΠΊΠΎΠΉ Ρ
Π°ΡΠ°ΠΊΡΠ΅ΡΠΈΡΡΠΈΠΊΠΎΠΉ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² ΠΏΠΎΠ·Π²ΠΎΠ»ΡΠ΅Ρ Π·Π°ΠΏΠΎΠ΄ΠΎΠ·ΡΠΈΡΡ Π΄ΠΈΠ°Π³Π½ΠΎΠ· Π‘Π‘Π.Β
Π‘ΠΎΠ²ΡΠ΅ΠΌΠ΅Π½Π½ΡΠ΅ ΠΏΠΎΠ΄Ρ ΠΎΠ΄Ρ ΠΊ Π΄ΠΈΠ°Π³Π½ΠΎΡΡΠΈΠΊΠ΅ , Π»Π΅ΡΠ΅Π½ΠΈΡ ΠΈ ΠΌΠΎΠ½ΠΈΡΠΎΡΠΈΠ½Π³Ρ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎ Π² Ρ ΠΊΡΠΈΠΎΠΏΠΈΡΠΈΠ½-Π°ΡΡΠΎΡΠΈΠΈΡΠΎΠ²Π°Π½Π½ΡΠΌΠΈ ΠΏΠ΅ΡΠΈΠΎΠ΄ΠΈΡΠ΅ΡΠΊΠΈΠΌΠΈ ΡΠΈΠ½Π΄ΡΠΎΠΌΠ°ΠΌΠΈ (CAPS)
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.ΠΡΠΈΠΎΠΏΠΈΡΠΈΠ½-Π°ΡΡΠΎΡΠΈΠΈΡΠΎΠ²Π°Π½Π½ΡΠ΅ ΠΏΠ΅ΡΠΈΠΎΠ΄ΠΈΡΠ΅ΡΠΊΠΈΠ΅ ΡΠΈΠ½Π΄ΡΠΎΠΌΡ (Cryopyrin-Associated Periodic Syndromes, CAPS) ΡΠ²Π»ΡΡΡΡΡ Π½Π°ΠΈΠ±ΠΎΠ»Π΅Π΅ ΡΡΠΊΠΈΠΌΠΈ ΠΏΡΠ΅Π΄ΡΡΠ°Π²ΠΈΡΠ΅Π»ΡΠΌΠΈ ΠΌΠΎΠ½ΠΎΠ³Π΅Π½Π½ΡΡ
Π°ΡΡΠΎΠ²ΠΎΡΠΏΠ°Π»ΠΈΡΠ΅Π»ΡΠ½ΡΡ
Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΠΉ (ΠΠΠ). ΠΠ½ΠΈ Ρ
Π°ΡΠ°ΠΊΡΠ΅ΡΠΈΠ·ΡΡΡΡΡ ΡΠΎΡΠ΅ΡΠ°Π½ΠΈΠ΅ΠΌ Π»ΠΈΡ
ΠΎΡΠ°Π΄ΠΊΠΈ, ΡΡΠΏΠΈ ΠΈ Π΄ΡΡΠ³ΠΈΡ
ΡΠΈΠΌΠΏΡΠΎΠΌΠΎΠ², ΡΠΎΠΏΡΠΎΠ²ΠΎΠΆΠ΄Π°ΡΡΠΈΡ
ΡΡ ΡΠΎΡΠΌΠΈΡΠΎΠ²Π°Π½ΠΈΠ΅ΠΌ ΠΏΠΎΡΠ΅Π½ΡΠΈΠ°Π»ΡΠ½ΠΎ ΠΈΠ½Π²Π°Π»ΠΈΠ΄ΠΈΠ·ΠΈΡΡΡΡΠΈΡ
ΠΈΠ»ΠΈ ΠΆΠΈΠ·Π½Π΅ΡΠ³ΡΠΎΠΆΠ°ΡΡΠΈΡ
ΠΎΡΠ»ΠΎΠΆΠ½Π΅Π½ΠΈΠΉ. ΠΠ° ΠΎΡΠ½ΠΎΠ²Π΅ ΡΠΎΠ²ΡΠ΅ΠΌΠ΅Π½Π½ΡΡ
ΡΠ΅ΠΊΠΎΠΌΠ΅Π½Π΄Π°ΡΠΈΠΉ ΠΏΠΎ Π²Π΅Π΄Π΅Π½ΠΈΡ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Ρ ΠΠΠ, Π° ΡΠ°ΠΊΠΆΠ΅ Π½Π°ΡΠ΅Π³ΠΎ ΡΠΎΠ±ΡΡΠ²Π΅Π½Π½ΠΎΠ³ΠΎ ΠΎΠΏΡΡΠ° ΡΠ°Π·ΡΠ°Π±ΠΎΡΠ°Π½ Π°Π»Π³ΠΎΡΠΈΡΠΌ Π΄ΠΈΠ°Π³Π½ΠΎΡΡΠΈΠΊΠΈ CAPS, ΠΏΠΎΠ·Π²ΠΎΠ»ΡΡΡΠΈΠΉ ΠΎΠΏΡΠΈΠΌΠΈΠ·ΠΈΡΠΎΠ²Π°ΡΡ ΠΈΡ
Π΄ΠΈΡΡΠ΅ΡΠ΅Π½ΡΠΈΠ°Π»ΡΠ½ΡΡ Π΄ΠΈΠ°Π³Π½ΠΎΡΡΠΈΠΊΡ Ρ ΡΠΈΡΡΠ΅ΠΌΠ½ΡΠΌ ΡΠ²Π΅Π½ΠΈΠ»ΡΠ½ΡΠΌ Π°ΡΡΡΠΈΡΠΎΠΌ ΠΈ Π΄ΡΡΠ³ΠΈΠΌΠΈ ΠΠΠ, ΡΠ°Π½Π½Π΅Π΅ Π½Π°Π·Π½Π°ΡΠ΅Π½ΠΈΠ΅ ΡΡΡΠ΅ΠΊΡΠΈΠ²Π½ΠΎΠΉ ΡΠ΅ΡΠ°ΠΏΠΈΠΈ, Π° ΡΠ°ΠΊΠΆΠ΅ ΡΠ°ΠΊΡΠΈΠΊΡ Π²Π΅Π΄Π΅Π½ΠΈΡ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ². ΠΠ½Π³ΠΈΠ±ΠΈΡΠΎΡΡ ΠΈΠ½ΡΠ΅ΡΠ»Π΅ΠΉΠΊΠΈΠ½Π° 1 ΡΠ²Π»ΡΡΡΡΡ ΡΠ°ΡΠ³Π΅ΡΠ½ΡΠΌΠΈ ΡΡΠ΅Π΄ΡΡΠ²Π°ΠΌΠΈ, ΡΒ ΠΏΠΎΠΌΠΎΡΡΡ ΠΊΠΎΡΠΎΡΡΡ
ΠΌΠΎΠΆΠ½ΠΎ ΠΊΠΎΠ½ΡΡΠΎΠ»ΠΈΡΠΎΠ²Π°ΡΡ Π°ΠΊΡΠΈΠ²Π½ΠΎΡΡΡ ΠΈ ΠΏΡΠ΅Π΄ΡΠΏΡΠ΅ΠΆΠ΄Π°ΡΡ ΡΠ°Π·Π²ΠΈΡΠΈΠ΅ ΠΎΡΠ»ΠΎΠΆΠ½Π΅Π½ΠΈΠΉ. ΠΡΠ΅Π΄ΡΡΠ°Π²Π»Π΅Π½Π½ΡΠΉ Π°Π»Π³ΠΎΡΠΈΡΠΌ ΠΏΠΎΠ·Π²ΠΎΠ»ΠΈΡΒ ΡΠ»ΡΡΡΠΈΡΡ ΡΠ²ΠΎΠ΅Π²ΡΠ΅ΠΌΠ΅Π½Π½ΡΡ Π΄ΠΈΠ°Π³Π½ΠΎΡΡΠΈΠΊΡ CAPS, ΠΎΠΏΡΠΈΠΌΠΈΠ·ΠΈΡΠΎΠ²Π°ΡΡ Π»Π΅ΡΠ΅Π½ΠΈΠ΅ ΠΈ Π²Π΅Π΄Π΅Π½ΠΈΠ΅ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Π² ΡΠ°ΠΌΠΊΠ°Ρ
ΡΡΡΠ°ΡΠ΅Π³ΠΈΠΈ Β«Treat to targetΒ»
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