Fibrinogen deficiency

Palumbo, J. S., Potter, J. M., Kaplan, L. S., Talmage, K., Jackson, D. G., and Degen, J. L. (2002). Spontaneous hematogenous and lymphatic metastasis, but not primary tumor growth or angiogenesis, is diminished in fibrinogen-deficient mice. Cancer Res. 62, 6966-6972. 

S., and Degen, J. L. (1995). Resolution of spontaneous bleeding events but failure of pregnancy in fibrinogen-deficient mice. Genes Develop. 9, 2020-2033. 

Inceman S, Caen X, Bernard X Aggregation, adhesion, and viscous metamoiphosis of platelets in congenital fibrinogen deficiency. J Lab Clin Med 1966 68 21-32. 

Zervos, N., Vlachos, J., Karpathios, T., Mantas, J. Giant hemangioma of the spleen with thrombocytopenia and fibrinogen deficiency. Acta pae-diat. scand. 172, 206-209 (1967)... 

Systematic reviews Several European coxmtries use fibrinogen concentrates, yet the United States and the United Kingdom reserve on-label use of these products for congenital fibrinogen deficiency [119 ]. 

For plasminogen-deficient fibrinogen from blood plasma, the anticoagulated blood was centrifuged and the plasma was frozen and washed with saline solution. Treated with charcoal and freeze-thawed. Dialysed versus Tris/NaCl buffer. [Maxwell and Nikel Biochem Prep 12 16 1968.]... 

Several substances that contribute to the blood coagulation process are formed in the liver. These include fibrinogen, prothrombin, and several of the blood clotting factors (II, VII, IX, and X). Deficiency in any of these substances leads to impaired blood coagulation. 

IL-6 participates in both atherogenesis and inflammatory processes. In one interesting mouse model that was double deficient at the apoE and IL-6 loci, animals displayed similar hypercholesterolemia compared to apoE-null mice, but disclosed larger and more calcified lesions at 1 year of age (Klinge 2001). Thus, IL-6 appears to be involved at the fibrous plaque stage of the atherosclerotic process. Moreover, IL-6 is a key factor in the generation of the hepatic acute-phase response and so increases the levels of CRP, fibrinogen, platelet... 

Figure 8.2 A developing blood clot is shown in this picture. A blood clot is made of platelets, membrane fragments of a bone marrow cell, and a network of insoluble proteins, particularly fibrin generated from a precursor protein, fibrinogen, through the work of a cascade of protein clotting factors. Several bleeding disorders result from inherited deficiencies in clotting proteins.

Clinical pharmacology Activated factor IX in combination with activated factor VIII activates factor X. This results ultimately in the conversion of prothrombin to thrombin. Thrombin then converts fibrinogen to fibrin, and a clot can be formed. Factor IX is the specific clotting factor deficient in patients with hemophilia B and in patients with acquired factor IX deficiencies. The administration of Coagulation Factor IX (Recombinant) increases plasma levels of factor IX and can temporarily correct the coagulation defect in these patients. 

Cry precipitate is a plasma protein fraction obtainable from whole blood. It is used to treat deficiencies or qualitative abnormalities of fibrinogen, such as that which occurs with disseminated intravascular coagulation and liver disease. A single unit of cryoprecipitate contains 300 mg of fibrinogen. 

Plant, P. W., and Grieninger, G. (1986). Noncoordinate synthesis of the fibrinogen subunits in hepatocytes cultured under hormone-deficient conditions. J. Biol. Chem. 261, 2331-2336. 

Factor deficiencies include disorders of fibrinogen such as afibrinogenemia and dysfibrinogenemias, prothrombin deficiency, factor V VII, X, XI, XII, and XIII deficiency, prekallikrein and high-molecular-weight kininogen deficiency, combined factor deficiencies, a2 anti-plasmin deficiency, a] antitrypsin Pittsburgh, and protein Z deficiency. 

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