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Федеральное государственное бюджетное учреждение науки
Институт теоретической и экспериментальной биофизики
Российской академии наук


ООО "ИЦ КОМКОН"

ФГБУН "Институт токсикологии" ФМБА России

Адрес редакции и реквизиты

192012, Санкт-Петербург, ул.Бабушкина, д.82 к.2, литера А, кв.378

Свидетельство о регистрации электронного периодического издания ЭЛ № ФС 77-37726 от 13.10.2009
Выдано - Роскомнадзор

ISSN 1999-6314


Клиническая медицина » Терапия • Гематология

Том: 14
Статья: « 10 »
Страницы:. 113-134
Опубликована в журнале: 4 февраля 2013 г.
English version

Болезнь Виллебранда у женщин

Колосков А.В.1,2, Столица А.А.2, Филиппова О.И.1,3

1ГБУЗ «Городская больница № 26», Санкт-Петербург, Россия
2ФГБУ «Федеральный центр сердца, крови и эндокринологии имени В.А. Алмазова»
3ФГБУ «Российский научно-исследовательский институт гематологии и трансфузиологии Федерального медико-биологического агентства»


Резюме
Женщины с наследственными нарушениями свертывающей системы крови имеют риск геморрагических осложнений вследствие гемостатических нарушений во время менструации и родов. В последнее десятилетие во всем мире наблюдается интерес исследователей к проблемам женщин с наследственными нарушениями гемостаза. Это привело к значительному улучшению осведомленности врачей - клиницистов в области патологии системы гемостаза у женщин. Меноррагия является самым распространенным симптомом геморрагического диатеза у женщин с наследственным нарушением свертывания крови и может быть первым или единственным симптомом. Роды для этих женщин также представляют гемостатическую проблему. Проблемы гемостаза требуют специализированного и индивидуального подхода к ведению беременности. Болезнь Виллебранда является наиболее распространенным наследственным нарушением свертывающей системы крови с частотой встречаемости 1 – 2 % в общей популяции. Заболевание вызвано дефицитом или дисфункцией фактора Виллебранда, плазменного белка, который опосредует начальную адгезию тромбоцита в местах повреждения сосуда, а также связывает в циркуляции и стабилизирует фактор VIII. Болезнь Виллебранда не является типичным примером дисфункции плазменного звена свертывающей системы крови, поскольку дефект фактора Виллебранда вызывает, прежде всего, нарушение агрегационной функции тромбоцитов.


Ключевые слова
болезнь Виллебранда, меноррагия, беременность, выкидыш, роды


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