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Congenital Afibrinogenemia
Congenital afibrinogenemia is a rare, genetically inherited blood fibrinogen disorder in which the blood does not clot normally due to the lack of fibrinogen, a blood protein necessary for coagulation. This disorder is autosomal recessive, meaning that two unaffected parents can have a child with the disorder. The lack of fibrinogen expresses itself with excessive and, at times, uncontrollable bleeding. Signs and symptoms As this is a disorder that is present in an individual from birth, there are no warning signs to look for. The first symptom usually seen is hemorrhage from the umbilical cord that is difficult to stop. Other symptoms include: * Nasal and oral mucosa bleeds * Gastrointestinal bleeding * Excessive/spontaneous bleeding or bruising from minor injury * Prolonged menstruation in women * Spontaneous abortion during pregnancy * CNS hemorrhaging Causes A missense or nonsense mutation to the genes that code for the fibrinogen protein are affected. Usually the mutat ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
List Of Fibrinogen Disorders
Fibrinogen disorders are a set of hereditary or acquired abnormalities in the quantity and/or quality of circulating fibrinogens. The disorders may lead to pathological bleeding and/or blood clotting or the deposition of fibrinogen in the liver, kidneys, or other organs and tissues. These disorders include: * Congenital afibrinogenemia, an inherited blood disorder in which blood does not clot normally due to the lack of fibrinogen; the disorder causes abnormal bleeding and thrombosis. * Congenital hypofibrinogenemia, an inherited disorder in which blood may not clot normally due to reduced levels of fibrinogen; the disorder may cause abnormal bleeding and thrombosis. * Fibrinogen storage disease, a form of congenital hypofibrinogenemia in which specific hereditary mutations in fibrinogen cause it to accumulate in, and damage, liver cells. The disorder may lead to abnormal bleeding and thrombosis but also to cirrhosis. * Congenital dysfibrinogenemia, an inherited disorder in which ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Thrombin
Prothrombin (coagulation factor II) is encoded in the human by the F2-gene. It is proteolytically cleaved during the clotting process by the prothrombinase enzyme complex to form thrombin. Thrombin (Factor IIa) (, fibrose, thrombase, thrombofort, topical, thrombin-C, tropostasin, activated blood-coagulation factor II, E thrombin, beta-thrombin, gamma-thrombin) is a serine protease, that converts fibrinogen into strands of insoluble fibrin, as well as catalyzing many other coagulation-related reactions. History After the description of fibrinogen and fibrin, Alexander Schmidt hypothesised the existence of an enzyme that converts fibrinogen into fibrin in 1872. Prothrombin was discovered by Pekelharing in 1894. Physiology Synthesis Thrombin is produced by the enzymatic cleavage of two sites on prothrombin by activated Factor X (Xa). The activity of factor Xa is greatly enhanced by binding to activated Factor V (Va), termed the prothrombinase complex. Prothrombin is prod ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Factor I Deficiency
Fibrinogen deficiency, also known as factor I deficiency, is a rare inherited bleeding disorder related to fibrinogen function in the coagulation cascade. It is typically subclassified into four distinct fibrinogen disorders: afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia, and hypodysfibrinogenemia. * Congenital afibrinogenemia is defined as a lack of fibrinogen in the blood, clinically <20 mg/deciliter of plasma. The frequency of this disorder is estimated at between 0.5 and 2 per million. Within the United States, afibrinogenemia accounts for 24% of all inherited abnormalities of fibrinogen, while hypofibrinogenemia and dysfibrinogenemia account for 38% each. * Congenital hypofibrinogenemia is defined as a partial deficiency of fibrinogen, clinically 20–80/deciliter of plasma. Estimated frequency va ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Consanguinity
Consanguinity (from Latin '':wikt: consanguinitas, consanguinitas'' 'blood relationship') is the characteristic of having a kinship with a relative who is descended from a common ancestor. Many jurisdictions have laws prohibiting people who are closely related by blood from Consanguine marriage, marrying or having sexual relations with each other. The degree of relationship, degree of consanguinity that gives rise to this prohibition varies from place to place. On the other hand, around 20% of the global population lives in areas where some consanguinous marriages are preferred. The degree of relationships are also used to determine heirs of an estate according to statutes that govern intestacy, intestate succession, which also vary from jurisdiction to jurisdiction. In some communities and time periods, cousin marriage is allowed or even encouraged; in others, it is taboo, and considered to be incest. The degree of relative consanguinity can be illustrated with a ''consanguinity ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Blood Plasma
Blood plasma is a light Amber (color), amber-colored liquid component of blood in which blood cells are absent, but which contains Blood protein, proteins and other constituents of whole blood in Suspension (chemistry), suspension. It makes up about 55% of the body's total blood volume. It is the Intravascular compartment, intravascular part of extracellular fluid (all body fluid outside cells). It is mostly water (up to 95% by volume), and contains important dissolved proteins (6–8%; e.g., serum albumins, globulins, and fibrinogen), glucose, clotting factors, electrolytes (, , , , , etc.), hormones, carbon dioxide (plasma being the main medium for excretory product transportation), and oxygen. It plays a vital role in an intravascular osmotic effect that keeps electrolyte concentration balanced and protects the body from infection and other blood-related disorders. Blood plasma can be separated from whole blood through blood fractionation, by adding an anticoagulant to a tube ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Cryoprecipitate
Cryoprecipitate, also called cryo for short, or Cryoprecipitate Antihemophilic factor (AHF), is a frozen blood product prepared from blood plasma. To create cryoprecipitate, plasma is slowly thawed to 1–6 °C. A cold-insoluble precipitate is formed, which is collected by centrifugation, resuspended in a small amount of residual plasma (generally 10–15 mL) and then re-frozen for storage. Cryoprecipitate contains fibrinogen, Factor VIII, Factor XIII and vWF. In many clinical contexts, use of cryoprecipitate has been replaced with use of clotting factor concentrates (where available), but the whole form is still routinely stocked by many hospital blood banks. Cryo can be stored at −18 °C or colder for 12 months from the original collection date or up to 36 months in Europe if stored below -25 °C. After thawing, single units of cryo (or units pooled using a sterile method) can be stored at 20–24 °C for up to 6 hours. If units of cryo are pooled in an ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Platelet Aggregation Function
Platelets or thrombocytes () are a part of blood whose function (along with the coagulation factors) is to react to bleeding from blood vessel injury by clumping to form a blood clot. Platelets have no cell nucleus; they are fragments of cytoplasm from megakaryocytes which reside in bone marrow or lung tissue, and then enter the circulation. Platelets are found only in mammals, whereas in other vertebrates (e.g. birds, amphibians), thrombocytes circulate as intact mononuclear cells. One major function of platelets is to contribute to hemostasis: the process of stopping bleeding at the site where the lining of vessels (endothelium) has been interrupted. Platelets gather at the site and, unless the interruption is physically too large, they plug the hole. First, platelets attach to substances outside the interrupted endothelium: ''adhesion''. Second, they change shape, turn on receptors and secrete chemical messengers: ''activation''. Third, they connect to each other through r ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Thrombin Time
The thrombin time (TT), also known as the thrombin clotting time (TCT), is a blood test that measures the time it takes for a clot to form in the plasma of a blood sample containing anticoagulant, after an excess of thrombin has been added. It is used to diagnose blood coagulation disorders and to assess the effectiveness of fibrinolytic therapy. This test is repeated with pooled plasma from normal patients. The difference in time between the test and the 'normal' indicates an abnormality in the conversion of fibrinogen (a soluble protein) to fibrin, an insoluble protein. The thrombin time compares the rate of clot formation to that of a sample of normal pooled plasma. Thrombin is added to the samples of plasma. If the time it takes for the plasma to clot is prolonged, a quantitative (fibrinogen deficiency) or qualitative (dysfunctional fibrinogen) defect is present. In blood samples suspected to contain heparin, a substance derived from snake venom called batroxobin (formerly ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Reptilase Time
Reptilase time (RT) is a blood test used to detect deficiency or abnormalities in fibrinogen, especially in cases of heparin contamination. Reptilase, an enzyme found in the venom of Bothrops snakes, has activity similar to thrombin. Unlike thrombin, reptilase is resistant to inhibition by antithrombin III. Thus, the reptilase time is not prolonged in blood samples containing heparin, hirudin, or direct thrombin inhibitors, whereas the thrombin time will be prolonged in these samples. Reptilase also differs from thrombin by releasing fibrinopeptide A, but not fibrinopeptide B, in its cleavage of fibrinogen Fibrinogen (coagulation factor I) is a glycoprotein protein complex, complex, produced in the liver, that circulates in the blood of all vertebrates. During tissue and vascular injury, it is converted Enzyme, enzymatically by thrombin to fibrin .... References Blood tests {{pathology-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Fibrinogen
Fibrinogen (coagulation factor I) is a glycoprotein protein complex, complex, produced in the liver, that circulates in the blood of all vertebrates. During tissue and vascular injury, it is converted Enzyme, enzymatically by thrombin to fibrin and then to a fibrin-based Thrombus, blood clot. Fibrin clots function primarily to occlude blood vessels to stop bleeding. Fibrin also binds and reduces the activity of thrombin. This activity, sometimes referred to as antithrombin I, limits clotting. Fibrin also mediates blood platelet and endothelial cell spreading, tissue fibroblast proliferation, Capillary action, capillary tube formation, and angiogenesis and thereby promotes revascularization and wound healing. Reduced and/or dysfunctional fibrinogens occur in various congenital and acquired human List of fibrinogen disorders, fibrinogen-related disorders. These disorders represent a group of rare conditions in which individuals may present with severe episodes of pathological bleed ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Partial Thromboplastin Time
The partial thromboplastin time (PTT), also known as the activated partial thromboplastin time (aPTT or APTT), is a blood test that characterizes coagulation of the blood. A historical name for this measure is the Kaolin-cephalin clotting time (KCCT), reflecting kaolin and cephalin as materials historically used in the test. Apart from detecting abnormalities in blood clotting, partial thromboplastin time is also used to monitor the treatment effect of heparin, a widely prescribed drug that reduces blood's tendency to clot. The PTT measures the overall speed at which blood clots form by means of two consecutive series of biochemical reactions known as the ''intrinsic'' pathway and common pathway of coagulation. The PTT indirectly measures action of the following coagulation factors: I (fibrinogen), II (prothrombin), V (proaccelerin), VIII (anti-hemophilic factor), X (Stuart–Prower factor), XI (plasma thromboplastin antecedent), and XII (Hageman factor). The PTT is ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Prothrombin Time
The prothrombin time (PT) – along with its derived measures of prothrombin ratio (PR) and international normalized ratio (INR) – is an assay for evaluating the Coagulation#Extrinsic pathway, extrinsic pathway and Coagulation#Common pathway, common pathway of coagulation. This blood test is also called ''protime INR'' and ''PT/INR''. They are used to determine the Thrombophilia, clotting tendency of blood, in conditions such as the measure of warfarin dosage, liver damage (cirrhosis), and vitamin K status. PT measures the following Coagulation#Coagulation factors, coagulation factors: fibrinogen, I (fibrinogen), thrombin, II (prothrombin), Factor V, V (proaccelerin), Factor VII, VII (proconvertin), and Factor X, X (Stuart–Prower factor). PT is often used in conjunction with the partial thromboplastin time, activated partial thromboplastin time (aPTT) which measures the Coagulation#intrinsic pathway, ''intrinsic'' pathway and common pathway of coagulation. Laboratory measur ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
