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Vascular haemostasis

NS Key, Anne De Paepe UGent, Fransiska Malfait UGent and CL Shovlin (2010) HAEMOPHILIA. 16(suppl. 5). p.146-151
abstract
While the majority of this session will deal with selected inherited vascular abnormalities that may manifest as a haemorrhagic disorder, the initial discussion by Dr Key will focus on the interplay between the vessel wall and components of the coagulation system, with a focus on haemophilia A and B. Although it is generally accepted that physiological haemostasis is triggered by contact of blood with tissue factor (TF), there remains some controversy regarding the cellular origin of TF in vivo. In addition, the initiation and propagation of thrombin generation are highly dependent on the balance of pro- and anticoagulant functions of endothelium, a profile that varies significantly throughout the vasculature. Drs De Paepe and Malfait address heritable collagen disorders such as the Ehlers-Danlos syndromes (EDS), a heterogeneous group of diseases involving the skin, ligaments and joints, blood vessels and internal organs. Most EDS subtypes are caused by mutations in genes encoding fibrillar collagens, or in genes coding for enzymes involved in posttranslational modifications of collagens. Accurate biochemical and molecular testing is now available for most EDS subtypes and can direct genetic counselling and medical management for these disorders. Dr Shovlin reviews recent developments in hereditary haemorrhagic telengiectasia (HHT), a frequently undiagnosed disorder characterized by arteriovenous malformations in multiple organs. These abnormal blood vessels are the result of mutations in one of a number of genes whose protein products influence TGF-beta signalling in vascular endothelial cells. Several HHT management guidelines have been published and are discussed.
Please use this url to cite or link to this publication:
author
organization
year
type
journalArticle (original)
publication status
published
subject
keyword
endothelium, hereditary haemorragic telengiectasia, Ehlars-Danlos, collagen, HEMOPHILIA-A, SLEEPING-BEAUTY, IN-VIVO, MICE, EXPRESSION, DEFICIENCY, ENDOTHELIAL-CELLS, TISSUE FACTOR, HEREDITARY HEMORRHAGIC TELANGIECTASIA, EHLERS-DANLOS-SYNDROME
journal title
HAEMOPHILIA
Haemophilia
volume
16
issue
suppl. 5
pages
146 - 151
Web of Science type
Article
Web of Science id
000279076100026
JCR category
HEMATOLOGY
JCR impact factor
2.364 (2010)
JCR rank
39/65 (2010)
JCR quartile
3 (2010)
ISSN
1351-8216
DOI
10.1111/j.1365-2516.2010.02313.x
language
English
UGent publication?
yes
classification
A1
copyright statement
I have transferred the copyright for this publication to the publisher
id
1064338
handle
http://hdl.handle.net/1854/LU-1064338
date created
2010-10-26 12:26:52
date last changed
2016-12-19 15:46:27
@article{1064338,
  abstract     = {While the majority of this session will deal with selected inherited vascular abnormalities that may manifest as a haemorrhagic disorder, the initial discussion by Dr Key will focus on the interplay between the vessel wall and components of the coagulation system, with a focus on haemophilia A and B. Although it is generally accepted that physiological haemostasis is triggered by contact of blood with tissue factor (TF), there remains some controversy regarding the cellular origin of TF in vivo. In addition, the initiation and propagation of thrombin generation are highly dependent on the balance of pro- and anticoagulant functions of endothelium, a profile that varies significantly throughout the vasculature. Drs De Paepe and Malfait address heritable collagen disorders such as the Ehlers-Danlos syndromes (EDS), a heterogeneous group of diseases involving the skin, ligaments and joints, blood vessels and internal organs. Most EDS subtypes are caused by mutations in genes encoding fibrillar collagens, or in genes coding for enzymes involved in posttranslational modifications of collagens. Accurate biochemical and molecular testing is now available for most EDS subtypes and can direct genetic counselling and medical management for these disorders. Dr Shovlin reviews recent developments in hereditary haemorrhagic telengiectasia (HHT), a frequently undiagnosed disorder characterized by arteriovenous malformations in multiple organs. These abnormal blood vessels are the result of mutations in one of a number of genes whose protein products influence TGF-beta signalling in vascular endothelial cells. Several HHT management guidelines have been published and are discussed.},
  author       = {Key, NS and De Paepe, Anne and Malfait, Fransiska and Shovlin, CL},
  issn         = {1351-8216},
  journal      = {HAEMOPHILIA},
  keyword      = {endothelium,hereditary haemorragic telengiectasia,Ehlars-Danlos,collagen,HEMOPHILIA-A,SLEEPING-BEAUTY,IN-VIVO,MICE,EXPRESSION,DEFICIENCY,ENDOTHELIAL-CELLS,TISSUE FACTOR,HEREDITARY HEMORRHAGIC TELANGIECTASIA,EHLERS-DANLOS-SYNDROME},
  language     = {eng},
  number       = {suppl. 5},
  pages        = {146--151},
  title        = {Vascular haemostasis},
  url          = {http://dx.doi.org/10.1111/j.1365-2516.2010.02313.x},
  volume       = {16},
  year         = {2010},
}

Chicago
Key, NS, Anne De Paepe, Fransiska Malfait, and CL Shovlin. 2010. “Vascular Haemostasis.” Haemophilia 16 (suppl. 5): 146–151.
APA
Key, N., De Paepe, A., Malfait, F., & Shovlin, C. (2010). Vascular haemostasis. HAEMOPHILIA, 16(suppl. 5), 146–151.
Vancouver
1.
Key N, De Paepe A, Malfait F, Shovlin C. Vascular haemostasis. HAEMOPHILIA. 2010;16(suppl. 5):146–51.
MLA
Key, NS, Anne De Paepe, Fransiska Malfait, et al. “Vascular Haemostasis.” HAEMOPHILIA 16.suppl. 5 (2010): 146–151. Print.