Abstract:
:Clinical and laboratory data of children with von Willebrand disease (VWD) types have been derived from retrospective studies and small case series. This article reports on the clinical and laboratory data of a large pediatric cohort in one single Argentinian center. The biological and clinical responses to desmopressin and replacement therapies are also described. Over a 15-year period, 194 of 1150 children (16.9%) were diagnosed as having type 1 VWD (80%), type 2 VWD (19%), and type 3 VWD (1%). The distribution of the different type 2 VWD subtypes was type 2A VWD, 43%; type 2B VWD, 32%; type 2M VWD, 19%; and type 2N VWD, 6%. Eighty patients with type 1 VWD and 12 patients with type 2 VWD were prospectively evaluated to desmopressin (DDAVP) response. A complete response was observed in all children with type 1 VWD, whereas 40% of the children with severe type 1 VWD and with type 2 VWD achieved a complete response. All the children who received DDAVP as prophylaxis or treatment for bleeding had good clinical evolution. Considering the restricted availability of specialized hemostasis centers, we believe our clinical and laboratory approach appropriate for the detection of patients with different types of VWD. Further studies are necessary to determine epidemiological aspects of VWD in Argentina to estimate the necessary facilities and trained personnel for the diagnosis and management of patients with VWD.
journal_name
Semin Thromb Hemostjournal_title
Seminars in thrombosis and hemostasisauthors
Bonduel M,Frontroth JP,Hepner M,Sciuccati G,Feliu-Torres A,Pieroni Gdoi
10.1055/s-0031-1281043subject
Has Abstractpub_date
2011-07-01 00:00:00pages
560-7issue
5eissn
0094-6176issn
1098-9064journal_volume
37pub_type
杂志文章abstract::Low-molecular-weight heparins (LMWHs) are now universally accepted as drugs of choice for postsurgical prophylaxis and treatment of deep vein thrombosis (DVT). Currently, these agents are also being developed for the treatment of various cardiovascular conditions. Because of manufacturing differences, each of the LMWH...
journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章,评审
doi:10.1055/s-2004-823007
更新日期:2004-02-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
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doi:10.1055/s-2007-1004383
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章,评审
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章,评审
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更新日期:2020-03-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章,评审
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章,多中心研究
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更新日期:2016-07-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章,评审
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更新日期:2009-09-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章
doi:10.1055/s-0039-1697949
更新日期:2020-06-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章,评审
doi:10.1055/s-2007-1004360
更新日期:1985-01-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章,评审
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更新日期:2010-06-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章,评审
doi:10.1055/s-0031-1300948
更新日期:2012-02-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章
doi:10.1055/s-2007-996036
更新日期:1998-01-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章,评审
doi:10.1055/s-2000-13221
更新日期:2000-01-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章
doi:10.1055/s-2007-999030
更新日期:1996-01-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章
doi:10.1055/s-0038-1676319
更新日期:2019-03-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章,评审
doi:10.1055/s-0029-1234145
更新日期:2009-07-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章,评审
doi:10.1055/s-2007-994914
更新日期:1999-01-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章,评审
doi:10.1055/s-2004-822973
更新日期:2004-02-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章
doi:10.1055/s-0036-1584351
更新日期:2017-02-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章,评审
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更新日期:2013-02-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章,评审
doi:10.1055/s-2007-991540
更新日期:2007-10-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 指南,杂志文章,实务指引
doi:10.1055/s-2007-996107
更新日期:1997-01-01 00:00:00
abstract::There are several genetic and acquired risk factors for venous thromboembolism. Exposure to high altitude (HA), either during air travel, ascension of mountains, or while engaging in sports activities, has been observed to result in a hypercoagulable state, thus predisposing to thromboembolic events. Although several ...
journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章,评审
doi:10.1055/s-0032-1301413
更新日期:2012-03-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章,评审
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更新日期:2006-04-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章,评审
doi:10.1055/s-0037-1601328
更新日期:2018-07-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章,评审
doi:10.1055/s-2005-922223
更新日期:2005-11-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
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更新日期:1991-10-01 00:00:00
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journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章
doi:10.1055/s-2007-1003541
更新日期:1986-04-01 00:00:00
abstract::Each bone marrow megakaryocyte (MK) releases thousands of platelets into the circulation, and the underlying molecular and cellular mechanisms recently have received intense scrutiny. Genetic studies are beginning to clarify the mechanisms by which transcription factors help distinguish MK progenitors from other blood...
journal_title:Seminars in thrombosis and hemostasis
pub_type: 杂志文章,评审
doi:10.1055/s-2004-833474
更新日期:2004-08-01 00:00:00
abstract:: ...
journal_title:Seminars in thrombosis and hemostasis
pub_type: 社论,历史文章
doi:10.1055/s-0038-1677460
更新日期:2019-02-01 00:00:00