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<article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" xmlns:ali="http://www.niso.org/schemas/ali/1.0/" article-type="other" dtd-version="1.2" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">Pediatric Hematology/Oncology and Immunopathology</journal-id><journal-title-group><journal-title xml:lang="en">Pediatric Hematology/Oncology and Immunopathology</journal-title><trans-title-group xml:lang="ru"><trans-title>Вопросы гематологии/онкологии и иммунопатологии в педиатрии</trans-title></trans-title-group></journal-title-group><issn publication-format="print">1726-1708</issn><issn publication-format="electronic">2414-9314</issn><publisher><publisher-name xml:lang="en">Fund Doctors, Innovations, Science for Children</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">84</article-id><article-id pub-id-type="doi">10.24287/1726-1708-2017-16-1-83-95</article-id><article-categories><subj-group subj-group-type="toc-heading"><subject>Статьи</subject></subj-group><subj-group subj-group-type="article-type"><subject></subject></subj-group></article-categories><title-group><article-title xml:lang="en">Modern approach to diagnosis of inherited functional platelet disorders</article-title><trans-title-group xml:lang="ru"><trans-title>Диагностика врожденных нарушений функций тромбоцитов: современное состояние вопроса</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Fedorova</surname><given-names>D. V.</given-names></name><name xml:lang="ru"><surname>Фёдорова</surname><given-names>Д. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, pediatrician of Outpatient Consultative Unit</p><p>Russia 117997, Moscow, Samory Mashela st., 1</p><p>+7 (495) 287-6570, ext. 1702</p></bio><email>darya.v.fedorova@gmail.com</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Zharkov</surname><given-names>P. A.</given-names></name><name xml:lang="ru"><surname>Жарков</surname><given-names>П. А.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Plyasunova</surname><given-names>S. A.</given-names></name><name xml:lang="ru"><surname>Плясунова</surname><given-names>С. А.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Seregina</surname><given-names>E. A.</given-names></name><name xml:lang="ru"><surname>Серёгина</surname><given-names>Е. А.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Ignatova</surname><given-names>A. A.</given-names></name><name xml:lang="ru"><surname>Игнатова</surname><given-names>А. А.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><xref ref-type="aff" rid="aff1"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">National Scientific and Practical Centre of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev</institution></aff><aff><institution xml:lang="ru">ФГБУ «Национальный научно-практический центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава России, Москва</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2017-02-10" publication-format="electronic"><day>10</day><month>02</month><year>2017</year></pub-date><volume>16</volume><issue>1</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>83</fpage><lpage>95</lpage><history><date date-type="received" iso-8601-date="2018-08-10"><day>10</day><month>08</month><year>2018</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2017, «D. Rogachev NMRCPHOI»</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2017, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России</copyright-statement><copyright-year>2017</copyright-year><copyright-holder xml:lang="en">«D. Rogachev NMRCPHOI»</copyright-holder><copyright-holder xml:lang="ru">ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России</copyright-holder><ali:free_to_read xmlns:ali="http://www.niso.org/schemas/ali/1.0/"/><license><ali:license_ref xmlns:ali="http://www.niso.org/schemas/ali/1.0/">https://creativecommons.org/licenses/by/4.0</ali:license_ref></license></permissions><self-uri xlink:href="https://hemoncim.com/jour/article/view/84">https://hemoncim.com/jour/article/view/84</self-uri><abstract xml:lang="en"><p>Inherited functional platelet disorders (IFPD) are very heterogeneous group of hemorrhagic diseases that are difficult to diagnose. Prevalence of IFPD in general population is probably underestimated. In most cases IFPD cause mild to moderate bleeding symptoms in everyday life but may result in life-threating bleeding after trauma and surgical interventions. Rational diagnostic approach is critical to prevent these complications. Data on diagnostic algorithms as well as on benefits and disadvantages of specific laboratory methods are presented in this review. Approach to clinical evaluation is also reviewed with particular attention to bleeding assessment tools. The main emphasis is made on light transmission aggregometry, measurement of platelet granules content and release, flow cytometry. Problems of standardization of platelet functional tests are outlined. Diagnostic algorithms are applied according to the up to date recommendations of International Society on Thrombosis and Haemostasis.</p></abstract><trans-abstract xml:lang="ru"><p/></trans-abstract><kwd-group xml:lang="en"><kwd>children</kwd><kwd>bleeding</kwd><kwd>platelets</kwd><kwd>inherited</kwd><kwd>platelet disorders</kwd><kwd>light transmission aggregometry</kwd><kwd>flow cytometry</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>дети</kwd><kwd>геморрагический синдром</kwd><kwd>тромбоциты</kwd><kwd>тромбоцитопатии</kwd><kwd>световая агрегометрия</kwd><kwd>проточная цитометрия</kwd></kwd-group><funding-group/></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><citation-alternatives><mixed-citation xml:lang="en">1. Gremmel T., Frelinger A.L. 3rd, Michelson A.D. Platelet Physiology. Semin Thromb Hemost. 2016;42(3):191-204.</mixed-citation><mixed-citation xml:lang="ru">Gremmel T., Frelinger A.L. 3rd, Michelson A.D. Platelet Physiology. 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