Fibrinogen replacement therapy for congenital fibrinogen deficiency
نویسندگان
چکیده
منابع مشابه
Fibrinogen replacement therapy for congenital fibrinogen deficiency.
This review of published studies was conducted to derive data on patients with congenital fibrinogen deficiency (CFD), including dosing of fibrinogen replacement therapy, outcome, and adverse events, either temporally related or distant to fibrinogen replacement, in order to assist clinicians in developing treatment plans for patients with CFD. A systematic review was performed of case reports ...
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A 17 yr old female with a congenital bleeding disorder was found to suffer from dysfibrinogenemia. Whole blood and plasma coagulation times were delayed and thrombelastograms were grossly abnormal. Clottability of plasma fibrinogen by addition of thrombin was not demonstrated during the 30 min test period. Fibrinogen was revealed by turbidometric and immunologic techniques. Other coagulation fa...
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Afibrinogenemia is a rare bleeding disorder with an estimated prevalence of 1:1,000,000. It is an autosomal recessive disease resulting from mutations in any of the 3 genes that encode the 3 polypeptide chains of fibrinogen and are located on the long arm of chromosome 4. Spontaneous bleeding, bleeding after minor trauma and excessive bleeding during interventional procedures are the principal ...
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Fibrinogen from normal platelets and plasma was compared with material derived from a subject with the congenital fibrinogen abnormality termed fibrinogen Paris I. The molecular abnormality in this subject is characterized by the presence (in plasma molecules) of relatively large mutant ‘y-chains (‘y-Paris I) that replace 50% or more of the normal “y-chain population and that, unlike normal ‘y-...
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There is a growing interest in the role that fibrinogen plays in major haemorrhage. It has been known since 1995 that fibrinogen is one of the first coagulation proteins to fall to critically low levels during major blood loss [1], but the clinical relevance of this is only now being evaluated. Injury is the leading cause of death worldwide for patients aged 1-45 and accounts for 7,800 deaths i...
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ژورنال
عنوان ژورنال: Journal of Thrombosis and Haemostasis
سال: 2011
ISSN: 1538-7933
DOI: 10.1111/j.1538-7836.2011.04424.x