Academic Journals Database
Disseminating quality controlled scientific knowledge

Congenital Factor V Deficiency: Moroccans Cases

ADD TO MY LIST
 
Author(s): Oukkache Bouchra Louai | Marielle Igala

Journal: Macedonian Journal of Medical Sciences
ISSN 1857-5749

Volume: 6;
Issue: 3;
Start page: 278;
Date: 2013;
VIEW PDF   PDF DOWNLOAD PDF   Download PDF Original page

Keywords: Factor V | Coagulation | Deficit | Congenital | Coagulopathy.

ABSTRACT
Background and Objective: The congenital factor V deficiency, known as Owren's disease or parahemophilia is a rare autosomic recessive haemorrhagic disease of coagulation, identified for the first time in Norway in 1943. In Morocco, there is no national registry including cases of congenital coagulopathy. This study highlights the rarity of inherited factor V deficiency deficiency and the importance of coagulation testing in the diagnosis of condition.Patients and Methods: We report twelve cases of factor V deficiency at our institution. Results: Of the 12 cases, seven were followed at the haematology department; the remaining five were discovered during a family survey. The average age of our patients is 16 years old [1 - 40 years], with a female dominance (Sex ratio F/M: 2). Parental consanguinity was found in three patients, bleeding family history were found in 5 patients. The concept of bleeding was found in 6 of 7 cases of patients followed, in 1 patient’s case the discovery was fortuitous.Conclusion: Inherited factor V deficiency is a rare inherited disorder. The diagnosis is mainly based on coagulation tests; however, the bleeding tendency may be severe and occurs early in life with immeasurable factor II levels.
RPA Switzerland

RPA Switzerland

Robotic process automation

    

Tango Jona
Tangokurs Rapperswil-Jona