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PubMed1981

[Intra-osseus hemorrhagic lesions in congenital afibrinogenemia. A new case (author's transl)].

Congenital afibrinemia is a rare disease, transmitted by an autosomal recessive mode, and due to deficient fibrinogen. Osseous involvement is exceptional, since only 2 cases have been reported. We describe a third case. The roentgenographic features are well limited radiolucent lesions, arising in the medullary space, and slightly expanding. These lesions are related to fibrous cysts, resulting from intraosseous hemorrhages. Spontaneous resolution may occur: the absence of fibrin deposits and blood clot probably favours resorption.

Afibrinogenemia↗
PubMed1995

[Consumption coagulopathy during pregnancy: a case of afibrinogenemia during the second trimester].

We report a case of a membrane disruption at 19 weeks 3 days of gestation complicated by a consumption coagulopathy with clotting tests suggesting primary fibrinogenolysis rather than a disseminated intravascular coagulation usually seen in such cases. A review of the literature revealed 5 cases of coagulopathy during the second trimester of pregnancy among which only two were suspected to be associated with primary fibrinogenolysis. Management of this coagulopathy is discussed.

Abruptio Placentae↗
PubMed1975

The effect of heparin on platelet aggregation by common inductors and by ristocetin in congenital bleeding disorders due to factor VIII or fibrinogen defects.

Platelet aggregation by various inductors was studied in citrated and heparinized plasma of the following groups of subjects: Normal, hemophilia A, combined factor V and factor VIII deficiency, v. Willeprand's disease and congenital afibrinognemia. The results may be summarized as follows: A-platelet aggregation in citrated plasm 1) platelet aggregation by common inductors ADP, adrenalin and collagen was normal in all groups of subjects but for the patients with congential afibrinogenemia in whom adrenalin induced aggregation was absent or markedly refuced whereas ADP and collagen gave slightly reduced or near normal aggregation curves. 2) platelet aggregation by ristocetin was normal in all groups of subjects but for v. Willebrand's disease in which it was absent. B-platelet aggregation in heparized plasma. 1) platelet aggregation by common inductors resulted to be normal in all groups of subjects except in congenital afibrinogenemia. In this latter case the pattern was still mildly defective but here was an increased aggregation as compared to citrated plasma. These findings have been interpretemmon inductors. 2) platelet aggregation by ristocetin resulted to be absent in all groups of subjects investigated. The possible mechanism of action of the inhibitory effect exercised py heparin with regard to restocetin is discussed.

Adenosine Diphosphate↗
PubMed1988

Importance of fibrinogen and platelet membrane glycoprotein IIb/IIIa in shear-induced platelet aggregation.

The mechanism of shear-induced platelet aggregation was investigated using a polycarbonate cone and plate viscometer. After exposed to shear stress of 54-90 dyne/cm2 for 2 min. at 37 degrees C, platelets aggregated without a significant amount of serotonin release and lactic dehydrogenase leakage from platelets. Under this conditions, platelets from 2 patients with thrombasthenia and a patient with congenital afibrinogenemia failed to aggregate. When fibrinogen was added to platelet rich plasma from a patient with afibrinogenemia, shear-induced platelet aggregation occurred at the same extent of aggregation as observed in normal platelets. Shear-induced platelet aggregation was inhibited by monoclonal antibody to GPIIb/IIIa (1 microgram/ml) and synthetic peptide, Arg-Gly-Asp-Ser (RGDS) (1 mM). Apyrase and hirudin showed no effect on this aggregation. Indomethacin (100 microM) and thromboxane A2 synthetase inhibitor, OKY-046 (100 microM) markedly inhibited aggregation, while thromboxane A2 competitive inhibitor, ONO-3708 (100 microM) exhibited only partial inhibition. These results indicate that fibrinogen and GPIIb/IIIa are important for shear-induced platelet aggregation and that the induction of fibrinogen receptor on GPIIb/IIIa may partially depend upon thromboxane A2 synthesis in platelets.

Afibrinogenemia↗
PubMed2002

Fibrinogen stabilizes placental-maternal attachment during embryonic development in the mouse.

In humans, maternal fibrinogen (Fg) is required to support pregnancies by maintaining hemostatic balance and stabilizing uteroplacental attachment at the fibrinoid layer found at the fetal-maternal junction. To examine relationships between low Fg levels and early fetal loss, a genetic model of afibrinogenemia was developed. Pregnant mice homozygous for a deletion of the Fg-gamma chain, which results in a total Fg deficiency state (FG(-/-)), aborted the fetuses at the equivalent gestational stage seen in humans. Results obtained from timed matings of FG(-/-) mice showed that vaginal bleeding was initiated as early as embryonic day (E)6 to 7, a critical stage for maternal-fetal vascular development. The condition of afibrinogenemia retarded embryo-placental development, and consistently led to abortion and maternal death at E9.75. Lack of Fg did not alter the extent or distribution pattern of other putative factors of embryo-placental attachment, including laminin, fibronectin, and Factor XIII, indicating that the presence of fibrin(ogen) is required to confer sufficient stability at the placental-decidual interface. The results of these studies demonstrate that maternal Fg plays a critical role in maintenance of pregnancy in mice, both by supporting proper development of fetal-maternal vascular communication and stabilization of embryo implantation.

Abortion, Spontaneous↗