A review of long-term prophylaxis in the rare inherited coagulation factor deficiencies

被引：30

作者：

Todd, T. ^{[1
]}

Perry, D. J. ^{[2
]}

机构：

[1] Royal Devon & Exeter Hosp, Dept Haematol, Exeter EX2 5DW, Devon, England

[2] Addenbrookes Hosp, Dept Haematol, Cambridge CB2 2QQ, England

来源：

HAEMOPHILIA | 2010年 / 16卷 / 04期

关键词：

prophylaxis; rare inherited coagulation factor deficiencies; VON-WILLEBRAND-DISEASE; FACTOR-VII DEFICIENCY; FACTOR-X DEFICIENCY; RECOMBINANT FACTOR-VIIA; FACTOR-V DEFICIENCY; NEONATAL INTRACRANIAL HEMORRHAGE; ACTIVATED FACTOR-VII; FACTOR-XIII; CONGENITAL AFIBRINOGENEMIA; REPLACEMENT THERAPY;

D O I：

10.1111/j.1365-2516.2009.02118.x

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

The rare inherited coagulation factor deficiencies (deficiencies of factors I, II, V, VII, XI, XIII, combined FV + FVII deficiency, combined deficiency of the vitamin K dependent factors and von Willebrand disease type 3) have an aggregate prevalence of approximately 1:100 000. They may cause recurrent life or function threatening haemorrhage. In this article we review the available literature on long-term prophylaxis and, where possible, make recommendations on this important area.

引用

页码：569 / 583

页数：15

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