GEMOFILIYA A BILAN OG'RIGAN BEMORLARDA EMIZUMABNI QO'LLASH NATIJALARI – SINOVLI YAKKA MARKAZLI TADQIQOT
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emizizumab, gemofiliya A, VIII omil, VIII omil ingibitori, bir bosqichli ivish usuli, xromogen usul##article.abstract##
Ma'lumki, VIII koagulatsiya omili yetishmovchiligidan kelib chiqadigan irsiy qon ketishi buzilishi bo'lgan og'ir gemofiliya A bilan og'rigan bemorlarda ko'pincha nazoratsiz qon ketishi kuzatiladi, bu esa koagulatsiya omillari bilan profilaktik almashtirish terapiyasi qo'llanilishiga qaramay sodir bo'ladi. Bunday holatlar gemofiliya A ning ingibitor shakliga xosdir [1,2]. Ingibitor - bu koagulatsiya omillarining prokoagulyant faolligini maxsus ravishda neytrallashtiradigan, qon ketishini boshqarishni qiyinlashtiradigan poliklonal yuqori affinlikli IgG antitelosidir. Ingibitorlarning paydo bo'lishi prognozning yomonlashishi bilan bog'liq: qon ketishining yuqori chastotasi, artropatiyaning tez rivojlanishi va hayot sifatining pasayishi. Og'irroq holatlarda bu bolalar o'limiga olib kelishi mumkin [3,6]. Yuqorida aytib o'tilgan holatlarning oldini olish, bemorlarning hayot sifatini yaxshilash va asoratlarning oldini olish uchun Bolalar gematologiyasi, onkologiyasi va immunologiyasi ilmiy-amaliy tibbiyot markazi VIII omil ingibitorlari yetishmovchiligidan kelib chiqadigan irsiy qon ketishi buzilishi bo'lgan 15 ta bolaga Hemlibra (emicizumab) ni buyurdi.
Библиографические ссылки
Румянцев А.Г., Масчан А.А., Вдовин В.В., Свирин П.В. Федеральные клинические рекомендации по диагностике и лечению детей с гемофилией А, гемофилией В и болезнью Виллебранда. М.; 2015. [Rumyantsev A.G., Maschan A.A., Vdovin V.V., Svirin P.V. Federal clinical guidelines for the diagnosis and treatment of children with hemophilia A, hemophilia B and von Willebrand disease. Moscow; 2015. (In Russ.)].
Srivastava A., Santagostino E., Dougall A., Kitchen S., Sutherland M., Pipe S.W., et al. WFH Guidelines for the Management of Hemophilia, 3rd edition. Haemophilia 2020; 26 Suppl 6: 1–158.
Barg A.A., Budnik I., Avishai E., Brutman‐Barazani T., Bashari D., Misgav M., et al. Emicizumab prophylaxis: Prospective longitudinal real‐ world follow‐up and monitoring. Haemophilia 2021; 27 (3): 383–91.
Долгов В.В., Свирин П.В., Вавилова Т.В. Лабораторная диагностика нарушений гемостаза. Учебно-методическое пособие. М; 2019. [Dolgov V.V., Svirin P.V., Vavilova T.V. Laboratory diagnosis of hemostasis disorders. Educational guide. Moscow; 2019. (In Russ.)].
Takeyama M., Nogami K., Shima M. A new parameter in the thrombin generation assay, mean velocity to peak thrombin, reflects factor VIII activity in patients with haemophilia A. Haemophilia 2016; 22 (5): e474–7.
Ketteler C., Hoffmann I., Davidson S., Tiede A., Richter N. Monitoring of different factor VIII replacement products using a factor VIII one‐stage clotting assay on cobas t 511/711 analysers. Haemophilia 2021; 27 (6): e704–12.
Gray E., Kitchen S., Bowyer A., Chowdary P., Jenkins P.V., Murphy P., et al. Laboratory measurement of factor replacement therapies in the treatment of congenital haemophilia: A United Kingdom Haemophilia Centre Doctors’ Organisation guideline. Haemophilia 2020; 26 (1): 6–16.
Жаркова П.А. Есть ли место заместительной терапии гемофилии А у детей в настоящем и будущем? Российский журнал детской гематологии и онкологии 2022; 9 (3): 56–64. DOI: 10.21682/2311-1267- 2022-9-3-56-64
Bowyer A.E., Lowe A.E., Tiefenbacher S. Laboratory issues in gene therapy and emicizumab. Haemophilia 2021; 27 (S3): 142–7.
Kizilocak H., Marquez‐Casas E., Malvar J., Carmona R., Young G. Determining the approximate factor VIII level of patients with severe haemophilia A on emicizumab using in vivo global haemostasis assays. Haemophilia 2021; 27 (5): 730–5
Jonsson F., Mercier F., Prins N.H., Schmitt C., Retout S. Exposure-response modeling of emicizumab for the prophylaxis of bleed counts in hemophilia A patients. 27th Meeting of the Population Approach Group in Europe (PAGE). 2018.
Adamkewicz J.I., Chen D.C., PazPriel I. Effects and Interferences of Emicizumab, a Humanised Bispecific Antibody Mimicking Activated Factor VIII Cofactor Function, on Coagulation Assays. Thromb Haemost 2019; 119 (7): 1084–93
