Haemophilia
SCOPUS (1995-2023)SCIE-ISI
1351-8216
1365-2516
Anh Quốc
Cơ quản chủ quản: WILEY , Wiley-Blackwell Publishing Ltd
Lĩnh vực:
HematologyMedicine (miscellaneous)Genetics (clinical)
Các bài báo tiêu biểu
von Willebrand disease (VWD): evidence‐based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA)1Summary. von Willebrand disease (VWD) is a commonly encountered inherited bleeding disorder affecting both males and females, causing mucous membrane and skin bleeding symptoms, and bleeding with surgical or other haemostatic challenges. VWD may be disproportionately symptomatic in women of child‐bearing age. It may also occur less frequently as an acquired disorder...
Tập 14 Số 2 - Trang 171-232 - 2008
The rare coagulation disorders – review with guidelines for management from the United Kingdom Haemophilia Centre Doctors' OrganisationSummary. The rare coagulation disorders are heritable abnormalities of haemostasis that may present significant difficulties in diagnosis and management. This review summarizes the current literature for disorders of fibrinogen, and deficiencies of prothrombin, factor V, FV + VIII, FVII, FX, the combined vitamin K‐dependent factors, FXI and FXIII. Based on both coll...
Tập 10 Số 5 - Trang 593-628 - 2004
Inherited antithrombin deficiency: a reviewSummary. Antithrombin (AT) is a potent inactivator of thrombin and factor Xa and the major inhibitor of blood coagulation. Inherited AT deficiencies are uncommon, with prevalences in the general population between 1 in 500 and 1 in 5000. They are either quantitative (type I) or qualitative (type II). Type II is subdivided into the more common, but less thrombogenic,...
Tập 14 Số 6 - Trang 1229-1239 - 2008
The obstetric and gynaecological management of women with inherited bleeding disorders – review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors’ OrganizationSummary. The gynaecological and obstetric management of women with inherited coagulation disorders requires close collaboration between obstetrician/gynaecologists and haematologists. Ideally these women should be managed in a joint disciplinary clinic where expertise and facilities are available to provide comprehensive assessment of the bleeding disorder and a com...
Tập 12 Số 4 - Trang 301-336 - 2006
Rare coagulation deficienciesSummary. Deficiencies of coagulation factors (other than factor VIII and factor IX) that cause a bleeding disorder are inherited as autosomal recessive traits and are generally rare, with prevalences in the general population varying between 1 : 500 000 and 1 : 2 000 000. In the last few years, the number of patients with recessively transmitted coagulation deficien...
Tập 8 Số 3 - Trang 308-321 - 2002
Guideline on the selection and use of therapeutic products to treat haemophilia and other hereditary bleeding disordersSummary. Evidence‐based guidelines are presented on the selection and use of therapeutic products to treat haemophilia and other hereditary bleeding disorders. They include details of therapeutic products available in the UK and they update and replace previous United Kingdom Haemophilia Centre Doctors’ Organisation guidelines.
Tập 14 Số 4 - Trang 671-684 - 2008
Nanofiltration of plasma‐derived biopharmaceutical productsSummary. This review presents the current status on the use and benefits of viral removal filtration systems – known as nanofiltration – in the manufacture of plasma‐derived coagulation factor concentrates and other biopharmaceutical products from human blood origin.Nanofiltration of plasma products has been implemented at a production scale in the e... ... hiện toàn bộ
Tập 9 Số 1 - Trang 24-37 - 2003
More than menorrhagia: a review of the obstetric and gynaecological manifestations of bleeding disordersSummary. In women, menorrhagia may be the most common manifestation of a bleeding disorder, but it is not the only reproductive tract abnormality that women with bleeding disorders experience. Women with bleeding disorders appear to be at an increased risk of developing haemorrhagic ovarian cysts and possibly endometriosis. As they grow older, they may be more likely...
Tập 11 Số 4 - Trang 295-307 - 2005
Sites of initial bleeding episodes, mode of delivery and age of diagnosis in babies with haemophilia diagnosed before the age of 2 years: a report from The Centers for Disease Control and Prevention’s (CDC) Universal Data Collection (UDC) projectSummary. Lack of detailed natural history and outcomes data for neonates and toddlers with haemophilia hampers the provision of optimal management of the disorder. We report an analysis of prospective data collected from 580 neonates and toddlers aged 0–2 years with haemophilia enrolled in the Universal Data Collection (UDC) surveillance project of the Centers for D...
Tập 15 Số 6 - Trang 1281-1290 - 2009
Management of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' OrganizationSummary. von Willebrand disease (VWD) is the commonest inherited bleeding disorder. The aim of therapy for VWD is to correct the two defects of haemostasis in this disorder, impaired primary haemostasis because of defective platelet adhesion and aggregation and impaired coagulation as a result of low levels of factor VIII. The objective of this guideline is to infor...
Tập 10 Số 3 - Trang 218-231 - 2004