Search results for "CONGENITAL AFIBRINOGENEMIA"
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Plasma viscosity pattern and erythrocyte aggregation in two patients with congenital afibrinogenemia
2020
: In this case report, we examine the behavior of plasma viscosity, explored at high and low shear rates, and erythrocyte aggregation in two patients with congenital afibrinogenemia, a clinical disorder firstly described in 1920 and that has an estimated incidence of 1 : 1-200 0000. The two hemorheological parameters examined by us showed a marked decrease in both patients, in one of whom erythrocyte aggregation was even undetectable. Keeping in mind that spontaneous thrombosis (venous and arterial) has been often described in congenital afibrinogenemia, it can be hypothesized that the decrease in plasma viscosity and erythrocyte aggregation might cause a reduction of the endothelial synthe…
Haemorheological profile in congenital afibrinogenemia and in congenital dysfibrinogenemia: A clinical case report
2019
Although the inherited quantitative and qualitative disorders of fibrinogen are rare, in the course of time patients may develop complications including episodes of arterial and venous thrombosis. It can be useful to complete the laboratory assessment of these clinical conditions with the evaluation of the haemorheological profile. The data obtained from this study showed that congenital afibrinogenemia was characterized by a primary plasma hypoviscosity, whereas congenital dysfibrinogenemia by a primary plasma hyperviscosity. Both these haemorheological alterations may concur, with different mechanisms, to the pathogenesis of thrombotic vascular complications.
"Incidence of bleeding symptoms in 100 patients with inherited afibrinogenemia or hypofibrinogenemia."
2006
Congenital deficiency of plasma fibrinogen is a hereditary bleeding disorder with an autosomal recessive pattern of inheritance [1] and an estimated incidence of 1–2 per million in the general population [1]. Due to the rarity of the disorder, the available data on the incidence of bleeding episodes, prevalent clinical manifestations and treatment modalities are scarce [2-7]. Afibrinogenemia is sometimes associated with symptoms that are unusual in patients with defects of coagulation factors, such as thrombotic complications and miscarriages [8-12]. However, knowledge on the incidence and significance of these unusual symptoms is influenced by publication bias. Fresh frozen plasma, cryopre…