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FEATURES OF DEVELOPMENT AND RESEARCH OF NATIONAL RECOMBINANT DRUGS OF COAGULATION FACTORS VII, VIII, IX

DOI: https://doi.org/10.29296/25877305-2019-09-18
Issue: 
9
Year: 
2019

Professor I. Davydkin(1), MD; Professor V. Zorenko(2), MD; A. Shuster(3), Candidate of Biological Sciences; A. Borozinets(3), Candidate of Medical Sciences 1-Samara State Medical University 2-National Medical Research Center of Hematology, Moscow 3-AO «GENERIUM», Vladimir region, Volginsky

Demand for pharmaceuticals of preferential drug provision under the program «7 high-cost nosologies» is satisfied by imports on 63% in Russia. The share of domestic biotechnological products is not more than 10% of the total market of medicines. In order to stimulate the development and production of domestic innovative medicines, the state program of the Russian Federation «development of the pharmaceutical and medical industry» for 2013–2020 was approved. Example of successful implementation of this program is development, research and industrial production of the full range of recombinant blood coagulation factors VII, VIII and IX (Coagil-VII®, Octofactor®, Innonafactor®) for the treatment of all types of hemophilia A, B and hemophilia with inhibitors. Biotechnological features of the production of blood clotting factors, the results of clinical and observational studies and the modern role of recombinant blood clotting factors in the treatment of hemophilia in the Russian Federation are analyzed.

Keywords: 
hemophilia
recombinant blood coagulationfactors VII
VIII
IX
drug provision of Russian patients
program «7 high-cost nosologies»
clinical and observational studies



References: 
  1. Farmatsevticheskij rynok Rossii 2017 [Elektronnyj resurs]: Analit. otchet / M.: DSMGroup. [Farmatsevticheskii rynok Rossii 2017 [Elektronnyi resurs]: Analit. otchet / M.: DSMGroup (in Russ.)] URL: http://www.dsm.ru/docs/analytics/dsm_report 2017.pdf
  2. Martynjuk O.V. Sootnoshenie importnyh i otechestvennyh lekarstvennyh sredstv na farmatsevticheskom rynke Rossijskoj Federatsii: itogi 20-letnego razvitija // Molodoj uchenyj – 2015; 10 (90): 912–6 [Martynyuk O.V. Sootnoshenie importnykh i otechestvennykh lekarstvennykh sredstv na farmatsevticheskom rynke Rossiiskoi Federatsii: itogi 20-letnego razvitiya // Molodoi uchenyi – 2015; 10 (90): 912–6 (in Russ.)].
  3. Federal'nyj registr po nozologii «gemofilija» po vsem sub'ektam Rossijskoj Federatsii po sostojaniju na 17.02.2017 [Elektronnyj resurs]: Federal'nyj registr / M.: Vserossijskoe obschestvo gemofilii [Federal’nyi registr po nozologii «gemofiliya» po vsem sub»ektam Rossiiskoi Federatsii po sostoyaniyu na 17.02.2017 [Elektronnyi resurs]: Federal’nyi registr / M.: Vserossiiskoe obshchestvo gemofilii (in Russ.)] URL: https://docs.google.com/viewer?url=https%3A%2F%2Fhemophilia.ru%2Findex.php%3Fdo%3Ddownload%26id%3D520%26viewonline%3D1
  4. Report on the Annual Global Survey 2017 of World Federation of Hemophilia, 2018; 74.
  5. Prikaz Minpromtorga Rossii ot 23.10.2009 №965 «Ob utverzhdenii Strategii razvitija farmatsevticheskoj promyshlennosti Rossijskoj Federatsii na period do 2020 goda» [Prikaz Minpromtorga Rossii ot 23.10.2009 №965 «Ob utverzhdenii Strategii razvitiya farmatsevticheskoi promyshlennosti Rossiiskoi Federatsii na period do 2020 goda» (in Russ.)]. URL: http://www.consultant.ru/cons/cgi/online
  6. Gosudarstvennaja programma «Razvitie farmatsevticheskoj i meditsinskoj promyshlennosti» na 2013–2020 gody [Elektronnyj resurs]: Osnovnye dannye po gosudarstvennoj programme «Razvitie farmatsevticheskoj i meditsinskoj promyshlennosti» na 2013–2020 gody / M.: Minpromtorg Rossii, 2014 [Gosudarstvennaya programma «Razvitie farmatsevticheskoi i meditsinskoi promyshlennosti» na 2013–2020 gody [Elektronnyi resurs]: Osnovnye dannye po gosudarstvennoi programme «Razvitie farmatsevticheskoi i meditsinskoi promyshlennosti» na 2013–2020 gody / M.: Minpromtorg Rossii, 2014 (in Russ.)] URL: http://minpromtorg.gov.ru/common/upload/files/docs/MinProm_02.06.14.pdf
  7. Ivanov R., Sekareva G., Kravtsova O. i dr. Pravila provedenija issledovanij bioanalogovyh lekarstvennyh sredstv (bioanalogov) // Farmakokinetika i farmakodinamika. – 2014; 1: 21–36 [Ivanov R., Sekarjova G., Kravcova O. et al. Guidelines of research biosimilar drugs // Farmakokinetika i farmakodinamika. – 2014; 1: 21–36 (in Russ.)].
  8. Pravila provedenija issledovanij biologicheskih lekarstvennyh sredstv Evrazijskogo ekonomicheskogo sojuza / Astana, 2016; 714 s. [Elektronnyj istochnik] [Pravila provedeniya issledovanii biologicheskikh lekarstvennykh sredstv Evraziiskogo ekonomicheskogo soyuza / Astana, 2016; 714 s. [Elektronnyi istochnik] (in Russ.)] URL: https://docs.eaeunion.org/docs/ru-ru/01411954/cncd_21112016_89
  9. Guideline on similar biological medicinal products containing biotechnology-derived proteins as active substance: non-clinical and clinical issues. EMEA/CHMP/BMWP/42832/2005 Rev1. 2015. 13c. URL: https://www.ema.europa.eu/en/similar-biological-medicinal-products-containing-biotechnology-derived-proteins-active-substance-non
  10. WFH Online Registry of Clotting Factor Concentrates. WFH eLearning Platform, 2018. URL: https://news.wfh.org/wfh-online-registry-clotting-factor-concentrates
  11. Shrivastava A. i dr. Rukovodstvo po lecheniju gemofilii Vsemirnoj Federatsii Gemofilii / BlekvellPablishing Ltd., 2012; 74 c. [Shrivastava A. et al. Rukovodstvo po lecheniyu gemofilii Vsemirnoi Federatsii Gemofilii / BlekvellPablishing Ltd., 2012; 74 s. (in Russ.)].
  12. Federal'nyj zakon ot 12.04.2010 №61-FZ (red. ot 28.11.2018) «Ob obraschenii lekarstvennyh sredstv» [Federal’nyi zakon ot 12.04.2010 №61-FZ (red. ot 28.11.2018) «Ob obrashchenii lekarstvennykh sredstv» (in Russ.)] URL: http://base.garant.ru/12174909
  13. Guideline on the clinical investigation of recombinant and human plasma-derived factor VIII products. EMA/CHMP/BPWP/144533/2009. – 2011; 20 p.
  14. Guideline on the clinical investigation of recombinant and human plasma-derived factor FIX products. EMA/CHMP/BPWP/144552/2009. – 2011; 20 p.
  15. Zorenko V.Ju., Mishin G.V., Severova T.V. i dr. Farmakokineticheskie svojstva, bezopasnost' i perenosimost' preparata Oktofaktor (rezul'taty I fazy klinicheskogo issledovanija u bol'nyh gemofiliej A) // Vopr. gematol. onkol. i immunopatol. v pediat. – 2013; 12 (2): 30–7 [Zorenko V.Yu., Mishin G.V., Severova T.V. et al. Pharmacokinetics, safety, and tolerability of Octofactor in patients with hemophilia A: Results of phase I clinical study // Vopr. gematol. onkol. i immunopatol. v pediat. – 2013; 12 (2): 30–7 (in Russ.)].
  16. Davydkin I.L., Andreeva T.A., Zorenko V.Ju. i dr. Effektivnost' i bezopasnost' preparata Oktofaktor pri profilakticheskom lechenii bol'nyh s tjazheloj i srednetjazheloj formoj gemofilii A (rezul'taty 1-j chasti klinicheskogo issledovanija II–III fazy) // Vopr. gematol. onkol. i immunopatol. v pediat. – 2013; 12 (3): 29–37 [Davydkin I.L., Andreeva T.A., Zorenko V.Yu., et al. Efficiency and safety of Octofactor in the treatment of patients with severe and moderate hemophilia A: Results of part 1 of phase II–III clinical trial // Vopr. gematol. onkol. i immunopatol. v pediat. – 2013; 12 (3): 29–37 (in Russ.)].
  17. Andreeva T.A., Zorenko V.Ju., Davydkin I.L. i dr. Effektivnost' i bezopasnost' preparata Oktofaktor v lechenii bol'nyh s tjazheloj i srednetjazheloj formoj gemofilii A (rezul'taty 2-j chasti klinicheskogo issledovanija II i III fazy) // Vopr. gematol. onkol. i immunopatol. v pediat. – 2013; 12 (4): 31–7 [Andreeva T.A., Zorenko V.Yu., Davydkin I.L. et al. Efficiency and safety of Octofactor in the treatment of patients with severe and moderate hemophilia A: Results of part 2 of phase II–III clinical trial // Vopr. gematol. onkol. i immunopatol. v pediat. – 2013; 12 (4): 31–7 (in Russ.)].
  18. Zorenko V.Ju., Mishin G.V., Severova T.V. i dr. Farmakokineticheskie svojstva, bezopasnost' i perenosimost' preparata Innonafaktor (rezul'taty I fazy klinicheskogo issledovanija u bol'nyh s tjazheloj i srednetjazheloj formoj gemofilii B) // Vopr. gematol. onkol. i immunopatol. v pediat. – 2014; 13 (4): 39–49 [Zorenko V.Yu., Mishin G.V., Severova T.V. et al. Pharmacokinetics, safety, and tolerability of Innonafactor: Results of phase I clinical study in patients with severe and moderate hemophilia B // Vopr. gematol. onkol. i immunopatol. v pediat. – 2014; 13 (4): 39–49 (in Russ.)].
  19. Andreeva T.A., Zorenko V.Ju., Davydkin I.L. i dr. Effektivnost' i bezopasnost' preparata Innonafaktor pri profilakticheskom lechenii bol'nyh s tjazheloj i srednetjazheloj formoj gemofilii V (rezul'taty 1-j chasti klinicheskogo issledovanija II–III fazy) // Vopr. gematol. onkol. i immunopatol. v pediat. – 2015; 14 (1): 65–75 [Andreeva T.A., Zorenko V.Yu., Davydkin I.L. et al. Efficiency and safety of Innonafactor in prevention of bleeding episodes in patients with severe and moderate hemophilia B: Results of part 1 of phase II–III clinical trial // Vopr. gematol. onkol. i immunopatol. v pediat. – 2015; 14 (1): 65–75 (in Russ.)].
  20. Davydkin I.L., Zorenko V.Ju., Andreeva T.A. i dr. Effektivnost' i bezopasnost' preparata Innonafaktor v lechenii bol'nyh s tjazheloj i srednetjazheloj formoj gemofilii B (rezul'taty 2-j chasti klinicheskogo issledovanija II–III fazy) // Vopr. gematol. onkol. i immunopatol. v pediat. – 2015; 14 (2): 50–8 [Davydkin I.L., Zorenko V.Yu., Andreeva T.A. et al. Efficiency and safety of Innonafactor in prevention of bleeding episodes in patients with severe and moderate hemophilia B: Results of part 2 of phase II–III clinical trial // Vopr. gematol. onkol. i immunopatol. v pediat. – 2015; 14 (2): 50–8 (in Russ.)].
  21. Zorenko V.Ju., Poljanskaja T.Ju. i dr. Opyt primenenija preparata Koagil-VII pri ortopedicheskih operatsijah u bol'nyh s ingibitornoj gemofiliej A // Vopr. gematol. onkol. i immunopatol. v pediat. – 2011; 10 (3): 35–40 [Zorenko V.Yu., Polyanskaya T.Yu., Galstyan G.M. et al. Experience gained in the administration of Soagil- VII in orthopedic surgeries in patients with hemophilia A and inhibitors // Vopr. gematol. onkol. i immunopatol. v pediat. – 2011; 10 (3): 35–40 (in Russ.)].
  22. Zorenko V.Ju., Galstjan G.M., Poljanskaja T.Ju. i dr. Sravnenie gemostaticheskogo dejstvija preparatov rekombinantnogo aktivirovannogo faktora svertyvanija krovi VII // Vopr. gematol. onkol. i immunopatol. v pediat. – 2011; 10 (4): 16–22 [Zorenko V.Yu., Galstyan G.M., Polyanskaya T.Yu. Comparison of the hemostatic effects of recombinant activated factor VII agents // Vopr. gematol. onkol. i immunopatol. v pediat. – 2011; 10 (4): 16–22 (in Russ.)].
  23. Vdovin V.V., Andreeva T.A., Davydkin I.L. i dr. Effektivnost' i bezopasnost' preparata Oktofaktor pri profilakticheskom lechenii podrostkov s tjazheloj gemofiliej A // Rossijskij zhurnal detskoj gematologii i onkologii. – 2017; 4 (1): 61–70 [Vdovin V.V., Andreeva T.A., Davydkin I.L. et al. The efficacy and safety of the drug Octofactor in prophylactic treatment of adolescents with severe hemophilia A // Russian Journal of Pediatric Hematology and Oncology. – 2017; 4 (1): 61–70 (in Russ.)]. https://doi.org/10.17650/2311-1267-2017-4-1-61-70.
  24. Andreeva T.A., Zorenko V.Ju., Davydkin I.L. i dr. Effektivnost' i bezopasnost' preparata Oktofaktor pri profilakticheskom lechenii u patsientov s tjazheloj gemofiliej A // Rossijskij zhurnal detskoj gematologii i onkologii. – 2018; 5 (3): 60–73 [Andreeva T.A., Zorenko V.Y., Davydkin I.L. et al. Efficacy and safety of the drug Octofactor in prophylactic treatment in patients with severe haemophilia A // Russian Journal of Pediatric Hematology and Oncology. – 2018; 5 (3): 60–73 (in Russ.)]. https://doi.org/10.17650/2311-1267-2018-5-3-60-73.
  25. Andreeva T.A., Davydkin I.L., Mamaev A.N. i dr. Rezul'taty mnogotsentrovogo, prospektivnogo, otkrytogo, nekontroliruemogo issledovanija effektivnosti i bezopasnosti preparata Innonafaktor u patsientov v vozraste 12 let i starshe s tjazheloj i srednetjazheloj gemofiliej V // Rossijskij zhurnal detskoj gematologii i onkologii. – 2017; 4 (4): 20–32 [Andreeva T.A., Davydkin I.L., Mamaev A.N. et al. Results of a multicenter, prospective, open, uncontrolled study of the efficacy and safety of the drug Innonafactor in patients aged 12 years and older with severe and moderate hemophilia B // Russian Journal of Pediatric Hematology and Oncology. – 2017; 4 (4): 20–32 (in Russ.)]. https://doi.org/10.17650/2311-1267-2017-4-4-20-32.
  26. Vol'skaja E.A. Osnovy nadlezhaschej praktiki neinterventsionnyh issledovanij lekarstvennyh preparatov // Kachestvennaja klinicheskaja praktika. – 2011; 1: 19–24 [Vol’skaya E.A. Osnovy nadlezhashchei praktiki neinterventsionnykh issledovanii lekarstvennykh preparatov // Kachestvennaya klinicheskaya praktika. – 2011; 1: 19–24 (in Russ.)].
  27. Zozulja N.I., Jastrubinetskaja O.I., Beljaeva S.S. i dr. Rezul'taty nabljudatel'nogo issledovanija effektivnosti i bezopasnosti preparata Oktofaktor // Rossijskij zhurnal detskoj gematologii i onkologii. – 2019; 6 (2): 30–47 [Zozulya N.I., Yastrubinetskaya O.I., Belyaeva S.S., et al. The results of the use of Octofactor in patients with moderate and severe hemophilia A (data from a prospective, multicenter, open-label, observational study) // Russian Journal of Pediatric Hematology and Oncology. – 2019; 6 (2): 30–47 (in Russ.)]. https://doi.org/10.21682/2311-1267-2019-6-2-30-47.
  28. Kuznik B.I., Sturov V.G., Levshin N.Ju. i dr. Gemorragicheskie i tromboticheskie zabolevanija i sindromy u detej i podrostkov: Patogenez, klinika, diagnostika, terapija i profilaktika / Novosibirsk: Nauka, 2018; 524 s. [Kuznik B.I., Sturov V.G., Levshin N.Yu. et al. Gemorragicheskie i tromboticheskie zabolevaniya i sindromy u detei i podrostkov: Patogenez, klinika, diagnostika, terapiya i profilaktika / Novosibirsk: Nauka, 2018; 524 s. (in Russ.)]
  29. Kudlaj D.A. Immunometabolicheskie aspekty patogeneza politravmy. Dis. …. d-ra med. nauk. Novosibirsk: GOU VPO «Novosibirskij gosudarstvennyj meditsinskij universitet», 2007 [Kudlai D.A. Immunometabolicheskie aspekty patogeneza politravmy. Dis. …. d-ra med. nauk. Novosibirsk: GOU VPO «Novosibirskii gosudarstvennyi meditsinskii universitet», 2007 (in Russ.)].
  30. Trekova N.A., Gus'kov D.A., Goncharova A.V. i dr. Effektivnost' otechestvennogo rekombinantnogo aktivirovannogo faktora VII dlja lechenija krovotechenij i normalizatsii gemostaza pri operatsijah na grudnom otdele aorty // Vestn. anesteziol. i reanimatol. – 2019; 16 (3): 25–31 [Trekova N.A., Guskov D.A., Goncharova A.V. et al. The efficacy of recombinant activated factor vii (coagil vii) for treatment haemorrhage and haemostasis disordes during thoracic aorta operations // Messenger of anesthesiology and resuscitation. – 2019; 16 (3): 25–32 (in Russ.)]. https://doi.org/10.21292/2078-5658-2019-16-3-25-31
  31. Fedorova T.A., Shmakov R.G., Rogachevskij O.V. i dr. Menedzhment krovi patsientki v akusherskoj praktike pri vrastanii platsenty // Med. sovet. – 2019; 7: 134–41 [Fedorova T.A., Shmakov R.G., Rogachevskiy O.V. et al. Patient’s blood management in obstetric practice with placenta accrete // Med. sovet. – 2019; 7: 134–41 (in Russ.)].