Document Detail


The plasma concentration of activated protein C appears normal in patients with haemophilia.
MedLine Citation:
PMID:  19175419     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Increased concentration of activated protein C (APC) has been observed in patients with thromboembolic disorders, but whether the level of APC in patients with bleeding disorders is decreased remains unknown. Seventy patients with haemophilia A or B with mild, moderate or severe form were studied. Detailed information on bleeding, arthropathy and factor consumption was collected during a 10-year period. The clinical severity of the disease was expressed as the Hemophilia Severity Score (HSS). Plasma concentration of APC was measured as APC in complex with protein C inhibitor. The median concentration of APC-PCI complex in patients with haemophilia was 0.14 microg L(-1) and it did not differ between the types and forms. In 16 patients with severe haemophilia A and the inversion mutation in intron 22, there was no correlation between clinical severity and the concentration of APC-PCI complex. Patients with haemophilia appear to generate normal concentrations of APC during basal conditions. APC does not seem to be an important modulator of the phenotypic expression of haemophilia.
Authors:
S Schulman; A Eelde; M Blombäck; J Stenflo
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Publication Detail:
Type:  Journal Article     Date:  2009-01-20
Journal Detail:
Title:  Haemophilia : the official journal of the World Federation of Hemophilia     Volume:  15     ISSN:  1365-2516     ISO Abbreviation:  Haemophilia     Publication Date:  2009 Mar 
Date Detail:
Created Date:  2009-04-01     Completed Date:  2009-12-14     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9442916     Medline TA:  Haemophilia     Country:  England    
Other Details:
Languages:  eng     Pagination:  566-70     Citation Subset:  IM    
Affiliation:
Department of Hematology, Karolinska University Hospital, Stockholm, Sweden. schulms@mcmaster.ca
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MeSH Terms
Descriptor/Qualifier:
Adult
Analysis of Variance
Biological Markers / blood
Enzyme Activation
Female
Hemophilia A / blood*,  genetics
Hemophilia B / blood*,  genetics
Humans
Male
Middle Aged
Phenotype
Predictive Value of Tests
Protein C / genetics,  metabolism*
Thrombin / genetics,  metabolism*
Chemical
Reg. No./Substance:
0/Biological Markers; 0/Protein C; EC 3.4.21.5/Thrombin

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


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