Document Detail


Associated prothrombotic conditions are probably responsible for the occurrence of thrombosis in almost all patients with congenital FVII deficiency. Critical review of the literature.
MedLine Citation:
PMID:  20044773     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The occasional occurrence of thrombosis in patients with congenital bleeding disorders has received considerable attention during the past decade. Myocardial infarction, ischemic strokes and venous thromboembolism have been reported in hemophilia A or B patients, in von Willebrand disease and, also, in rare coagulation disorders, especially in factor VII (FVII) deficiency. To explain the relatively high frequency of thrombotic phenomena, mainly venous, seen in the last condition, it was speculated that a special form or variant of FVII deficiency could exist. The presence of associated prothrombotic risk factors has been occasionally reported to be present in these patients but the matter has never been duly evaluated and emphasized. The purpose of the present paper was to evaluate if the clinical setting in which thrombosis appeared in these patients could explain the occurrence of the thrombosis. All reported cases of thrombosis seen in patients with FVII deficiency have been analyzed and the presence of associated risk factors recorded. Out of a population of 33 documented cases, the presence of prothrombotic risk factors was reported in 30 instances. In two of the remaining cases, no mention is made about associated risk factors. In the last case they were explicitly excluded. The critical evaluation of the literature suggests that the occurrence of thrombosis in FVII deficiency may be due to common prothrombotic risk factors. As a consequence it may be only stated that FVII deficiency does not protect from thrombosis.
Authors:
A Girolami; F Tezza; R Scandellari; S Vettore; B Girolami
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Journal of thrombosis and thrombolysis     Volume:  30     ISSN:  1573-742X     ISO Abbreviation:  J. Thromb. Thrombolysis     Publication Date:  2010 Aug 
Date Detail:
Created Date:  2010-07-26     Completed Date:  2010-11-08     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9502018     Medline TA:  J Thromb Thrombolysis     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  172-8     Citation Subset:  IM    
Affiliation:
Department of Medical and Surgical Sciences, University of Padua Medical, Padua, Italy. antonio.girolami@unipd.it
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Aged
Aged, 80 and over
Child
Evidence-Based Medicine
Factor VII Deficiency / blood,  complications*,  genetics
Female
Genetic Predisposition to Disease
Humans
Male
Middle Aged
Risk Assessment
Risk Factors
Thrombosis / blood,  etiology*,  genetics
Young Adult

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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