DOI: https://doi.org/10.30841/2307-5112.6.2018.169142

Гемофилия

С. В. Видиборець, Ю. Ю. Дерпак, Ю. Ю. Попович

Аннотация


В статье приведены алгоритмы современной гемостатической терапии гемофилии,основанной на применении как замещающейтерапии факторами свертывания крови, так идругих гемостатических препаратов. Приведены схемы расчета дозы гемостатическихпрепаратов, интервалы введения и продолжительность леченияпри наиболее частовстречающихся проявлениях геморрагического синдрома. Отдельно рассмотрены схемылечения болезни в амбулаторных условиях и в стационаре.

Ключевые слова


гемофилия; гемостатическая терапия; факторы свертывания крови; схемы лечения; геморрагический синдром

Полный текст:

PDF (Українська)

Литература


Averijanov E., Semenjaka V., Rybakov A. Issljedovanije effektivnosti preparata BioKlot A u pazientov s gemofiliej A s gemartrozami krupnyh sustavov [The study of the effectiveness of blood clotting factor VIII, BioClot A in patient with hemophilia A complicated with hemarthrosis in major joints]. Hematologija. Transfusiologija. Vostochnaja Evropa, 2015, no. 3(03), pp. 105–111.

Averianov E.V. Meaning of aspirate analysis for differential diagnostics of chronic blood-induced synovitis of knee-joint at hemophilic patients. Gematologija i perelivanije krovi, 2012, no. 36, pp. 9–13.

Averianov E.V., Semeniaka V.I. A comparative analysis of some blood count parameters at hemophilic patients with different disease severity. Gematologija i perelivanije krovi, 2012, no. 36, pp. 14–19.

Berntorp E. Modern haemophilia care / E. Berntorp, A.D. Shapiro // Lancet. 2012, vol. 379, no. 9824, pp. 1447–1456. https://doi.org/10.1016/S0140-6736(11)61139-2

Bezditko N. Clinical and economic analysis of different strategies for the treatvent of hemophilia A / N. Bezditko, O. Stasyshyn // Hematologija. Transfusiologija. Vostochnaja Evropa, 2017, vol. 3, no. 4, pp. 830–841.

Blat Y. A renaissance for the contact system in blood coagulation? / Y. Blat, D. Seiffert // Thromb. Haemost. 2008, vol. 99, no. 3, pр. 457–460. https://doi.org/10.1160/TH07-11-0692

Bolton-Maggs P.H. The rare coagulation disorders – review with guidelines for management from the United Kingdom Haemophilia Centre Doctors’ Organisation / Bolton-Maggs P.H., Perry D.J., Chalmers E.A., Parapia L.A., Wilde J.T., Williams M.D. // Haemophilia. 2004, vol. 10, no. 5, рp. 593–628. https://doi.org/10.1111/j.1365-2516.2004.00944.x

Council of Europe. Resolution CM/ Res (2017) 43 on principles concerning haemophilia therapies (replacing resolution CM/Res (2015) 3). PDF

Giangrande P.L.F. Kreuth IV: European consensus proposals for treatment of haemophilia with coagulation factor concentrates / P.L.F. Giangrande [et al.] //. Haemophilia, 2017, vol.23, pp. 370–375. https://doi.org/10.1111/hae.13211

Gomez K. Factor XI deficiency / K. Gomez, P. Bolton-Maggs // Haemophilia. 2008, vol. 14, no. 6, pp. 1183–1189. https://doi.org/10.1111/j.1365-2516.2008.01667.x

Hemophilia. Situational analysis of problems in Ukraine. Quantitative research of the international research of the international research Agency IFAK Institute of Gmbh &Co.

Hemophilija: navchal’nyj posibnyk / Moroz G.I., Vydyborets S.V., Gaidukova S.M. [et al.]. – Kyiv: NMAPO, 2011. – 165 p.

Imanaka Y. Identification and characterization of mutations in factor XI gene of non-Jewish factor XI deficient patients / Imanaka Y., McVey J.H, Nichimura T. et al. // Thromb Haemost. 1993, vol. 69, pp. 752–760.

Information: OOO “Rosh Ukraina” soobschaet o registrazii v Ukrajine prinzipial’no novogo, innovazionnogo ghtgfhfnf Gemlibra (emicizumab) dlja lechenija ingibitornoj formy hemofilii A u vzroslyh i detej. Hematologija. Transfusiologija. Vostochnaja Evropa, 2018, vol. 4, no. 4, pp. 447–448.

Kadir R.A. Screening for factor XI deficiency amongst pregnant women of Ashkenasi Jewis origin / R.A. Kadir, C.E. Kingman, C. Chi et al. // Haemophilia. – 2006, vol.12, no. 6, pp. 625–628. https://doi.org/10.1111/j.1365-2516.2006.01347.x

Moroz G., Vydyborets S.V. Nabuta hemofilija – vid diagnostyky do likuvannja [Acquired hemophilia – from diagnosis to treatment]. Ukrains’kij zhurnal gematologii ta transfusiologii. 2012, no. 2(17), pp. 35–44.

Moroz G.I., Vydyborets S.V., Gaidukova S.M. Prophylactic treatment of hemophilia: problems and prospects of introduction in Ukraine. Ukrains’kij zhurnal gematologii ta transfusiologii. 2009, no. 3(9), pp. 35–38.

Novak V.L., Masljak Z.V., Buzerak N.F. [et al.]. Pokaznyky dijal’nosti gematologichnoji sluzby Ukrajiny v 2016 rozi [Indicators of hematological service of Ukraine in 2016 ]. Lviv, 2017. – 42 p.

Srivastava A. Guidelines for the management of hemophilia / A. Srivastava, A.K. Brewer, E.P. Mauser-Bunschoten [et al.] // Haemophilia. 2013, vol.19, no.1, pp. e1–e47. https://doi.org/10.1111/j.1365-2516.2012.02909.x

Stasyshyn A.V. Hemophilia, aggravated by the inhibitor / In: Molecular pathology in proteins // Edit. D.I. Zabolotny. – New York: Nova Science Publishers, Inc. – 2009. – 191 p.

Unifikovanyj klinichnyj protocol ekstrennoj medychnoj dopomogy. Gemofilija. Nakaz MOZ Ukrainy vid 15.01.2014 roku № 34 [Unified clinical protocol of emergency medical care. Hemophilia. Order of Ministry of haelth of Ukraine from 15.01.2014 no.34]. PDF

Uss A. Optimizatsija okazanija gematologicheskoj pomoshi vzroslomu naseleniju Respubliki Belarus’ [Optimization of rendering hematological assistance to the adult population of the Republic of Belarus]. Hematologija. Transfusiologija. Vostochnaja Evropa, 2017, vol. 3, no. 4, pp. 637–646.

Vil’chevskaja E. Hemophilia v Ukrayine. Situazionnyi analiz, ishodja iz neudovletvorennyh potrebnostej patientov // Hematology. Transfusiology. Estern Europa, 2018, vol.4, no.4, pp. 456–465.

Vydyborets S.V. Industrija preparatov plasmy krovi [Industry of blood plasma preparations]. Hematologija. Transfusiologija. Vostochnaja Evropa, 2016, vol. 2, no. 2, pp. 227–255.

Vydyborets S.V., Moroz G.I., Gaidukova S.M., Sergienko O.V. Pharmacoeconomics of treatment of an hemophilia A. Ukrains’kij zhurnal gematologii ta transfusiologii. 2009, no. 6(9), pp. 26–33.




Copyright (c) 2018 Семейная медицина

Creative Commons License
Эта работа лицензирована Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

ISSN 2412-8708 (Online), ISSN 2307-5112 (Print)

Flag Counter