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Ôåäåðàëüíîå ãîñóäàðñòâåííîå áþäæåòíîå ó÷ðåæäåíèå íàóêè
Èíñòèòóò òåîðåòè÷åñêîé è ýêñïåðèìåíòàëüíîé áèîôèçèêè
Ðîññèéñêîé àêàäåìèè íàóê


ÎÎÎ "ÈÖ ÊÎÌÊÎÍ"

Àäðåñ ðåäàêöèè è ðåêâèçèòû

192012, Ñàíêò-Ïåòåðáóðã, óë.Áàáóøêèíà, ä.82 ê.2, ëèòåðà À, êâ.378

ISSN 1999-6314


Êëèíè÷åñêàÿ ìåäèöèíà » Òåðàïèÿ • Ãåìàòîëîãèÿ

Òîì: 14
Ñòàòüÿ: « 10 »
Ñòðàíèöû:. 113-134
Îïóáëèêîâàíà â æóðíàëå: 4 ôåâðàëÿ 2013 ã.

English version

Áîëåçíü Âèëëåáðàíäà ó æåíùèí

Êîëîñêîâ À.Â.1,2, Ñòîëèöà À.À.2, Ôèëèïïîâà Î.È.1,3

1ÃÁÓÇ «Ãîðîäñêàÿ áîëüíèöà ¹ 26», Ñàíêò-Ïåòåðáóðã, Ðîññèÿ
2ÔÃÁÓ «Ôåäåðàëüíûé öåíòð ñåðäöà, êðîâè è ýíäîêðèíîëîãèè èìåíè Â.À. Àëìàçîâà»
3ÔÃÁÓ «Ðîññèéñêèé íàó÷íî-èññëåäîâàòåëüñêèé èíñòèòóò ãåìàòîëîãèè è òðàíñôóçèîëîãèè Ôåäåðàëüíîãî ìåäèêî-áèîëîãè÷åñêîãî àãåíòñòâà»


Ðåçþìå
Æåíùèíû ñ íàñëåäñòâåííûìè íàðóøåíèÿìè ñâåðòûâàþùåé ñèñòåìû êðîâè èìåþò ðèñê ãåìîððàãè÷åñêèõ îñëîæíåíèé âñëåäñòâèå ãåìîñòàòè÷åñêèõ íàðóøåíèé âî âðåìÿ ìåíñòðóàöèè è ðîäîâ.  ïîñëåäíåå äåñÿòèëåòèå âî âñåì ìèðå íàáëþäàåòñÿ èíòåðåñ èññëåäîâàòåëåé ê ïðîáëåìàì æåíùèí ñ íàñëåäñòâåííûìè íàðóøåíèÿìè ãåìîñòàçà. Ýòî ïðèâåëî ê çíà÷èòåëüíîìó óëó÷øåíèþ îñâåäîìëåííîñòè âðà÷åé - êëèíèöèñòîâ â îáëàñòè ïàòîëîãèè ñèñòåìû ãåìîñòàçà ó æåíùèí. Ìåíîððàãèÿ ÿâëÿåòñÿ ñàìûì ðàñïðîñòðàíåííûì ñèìïòîìîì ãåìîððàãè÷åñêîãî äèàòåçà ó æåíùèí ñ íàñëåäñòâåííûì íàðóøåíèåì ñâåðòûâàíèÿ êðîâè è ìîæåò áûòü ïåðâûì èëè åäèíñòâåííûì ñèìïòîìîì. Ðîäû äëÿ ýòèõ æåíùèí òàêæå ïðåäñòàâëÿþò ãåìîñòàòè÷åñêóþ ïðîáëåìó. Ïðîáëåìû ãåìîñòàçà òðåáóþò ñïåöèàëèçèðîâàííîãî è èíäèâèäóàëüíîãî ïîäõîäà ê âåäåíèþ áåðåìåííîñòè. Áîëåçíü Âèëëåáðàíäà ÿâëÿåòñÿ íàèáîëåå ðàñïðîñòðàíåííûì íàñëåäñòâåííûì íàðóøåíèåì ñâåðòûâàþùåé ñèñòåìû êðîâè ñ ÷àñòîòîé âñòðå÷àåìîñòè 1 – 2 % â îáùåé ïîïóëÿöèè. Çàáîëåâàíèå âûçâàíî äåôèöèòîì èëè äèñôóíêöèåé ôàêòîðà Âèëëåáðàíäà, ïëàçìåííîãî áåëêà, êîòîðûé îïîñðåäóåò íà÷àëüíóþ àäãåçèþ òðîìáîöèòà â ìåñòàõ ïîâðåæäåíèÿ ñîñóäà, à òàêæå ñâÿçûâàåò â öèðêóëÿöèè è ñòàáèëèçèðóåò ôàêòîð VIII. Áîëåçíü Âèëëåáðàíäà íå ÿâëÿåòñÿ òèïè÷íûì ïðèìåðîì äèñôóíêöèè ïëàçìåííîãî çâåíà ñâåðòûâàþùåé ñèñòåìû êðîâè, ïîñêîëüêó äåôåêò ôàêòîðà Âèëëåáðàíäà âûçûâàåò, ïðåæäå âñåãî, íàðóøåíèå àãðåãàöèîííîé ôóíêöèè òðîìáîöèòîâ.


Êëþ÷åâûå ñëîâà
áîëåçíü Âèëëåáðàíäà, ìåíîððàãèÿ, áåðåìåííîñòü, âûêèäûø, ðîäû



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îòêðûòü ñòàòüþ â íîâîì îêíå

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