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.[1]
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.[1]
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.[2]
Congenital dysfibrinogenemia, an inherited disorder in which normal levels of fibrinogen composed at least in part of a dysfunctional fibrinogen may cause abnormal bleeding and thrombosis.[3]
Hereditary fibrinogen Aα-Chain amyloidosis, a form of dysfibrinogenemia in which certain fibrinogen mutations cause blood fibrinogen to accumulate in the kidney and cause one type of familial renal amyloidosis; the disorder is not associated with abnormal bleeding or thrombosis.[4]
Acquired dysfibrinogenemia, a disorder in which normal levels of fibrinogen are composed at least in part of a dysfunctional fibrinogen due to an acquired disorder (e.g. liver disease) that leads to the synthesis of an incorrectly glycosylated (i.e. wrong amount of sugar residues) added to an otherwise normal fibrinogen. The incorrectly glycosalated fibrinogen is dysfunctional and may cause pathological episodes of bleeding and/or blood clotting.[5]
Congenital hypodysfibrinogenemia, an inherited disorder in which low levels of fibrinogen composed at least in part of a dysfunctional fibrinogen may cause pathological episodes of bleeding or blood clotting.[6]
Cryofibrinogenemia, an acquired disorder in which fibrinogen precipitates at cold temperatures and may lead to the intravascular precipitation of fibrinogen, fibrin, and other circulating proteins thereby causing the infarction of various tissues and bodily extremities.[7]
^ abCasini A, de Moerloose P, Neerman-Arbez M (2016). "Clinical Features and Management of Congenital Fibrinogen Deficiencies". Seminars in Thrombosis and Hemostasis. 42 (4): 366–74. doi:10.1055/s-0036-1571339. PMID 27019462. S2CID 12038872.
^Casini A, Sokollik C, Lukowski SW, Lurz E, Rieubland C, de Moerloose P, Neerman-Arbez M (2015). "Hypofibrinogenemia and liver disease: a new case of Aguadilla fibrinogen and review of the literature". Haemophilia. 21 (6): 820–7. doi:10.1111/hae.12719. PMID 25990487. S2CID 44911581.
^Casini A, Neerman-Arbez M, Ariëns RA, de Moerloose P (2015). "Dysfibrinogenemia: from molecular anomalies to clinical manifestations and management". Journal of Thrombosis and Haemostasis. 13 (6): 909–19. doi:10.1111/jth.12916. PMID 25816717. S2CID 10955092.
^Gillmore JD, Lachmann HJ, Rowczenio D, Gilbertson JA, Zeng CH, Liu ZH, Li LS, Wechalekar A, Hawkins PN (2009). "Diagnosis, pathogenesis, treatment, and prognosis of hereditary fibrinogen A alpha-chain amyloidosis". Journal of the American Society of Nephrology. 20 (2): 444–51. doi:10.1681/ASN.2008060614. PMC 2637055. PMID 19073821.
^Besser MW, MacDonald SG (2016). "Acquired hypofibrinogenemia: current perspectives". Journal of Blood Medicine. 7: 217–225. doi:10.2147/JBM.S90693. PMC 5045218. PMID 27713652.
^Casini A, Brungs T, Lavenu-Bombled C, Vilar R, Neerman-Arbez M, de Moerloose P (2017). "Genetics, diagnosis and clinical features of congenital hypodysfibrinogenemia: a systematic literature review and report of a novel mutation". Journal of Thrombosis and Haemostasis. 15 (5): 876–888. doi:10.1111/jth.13655. PMID 28211264.
^Caimi G, Canino B, Lo Presti R, Urso C, Hopps E (2017). "Clinical conditions responsible for hyperviscosity and skin ulcers complications" (PDF). Clinical Hemorheology and Microcirculation. 67 (1): 25–34. doi:10.3233/CH-160218. hdl:10447/238851. PMID 28550239.
and 24 Related for: List of fibrinogen disorders information
Fibrinogendisorders are a set of hereditary or acquired abnormalities in the quantity and/or quality of circulating fibrinogens. The disorders may lead...
after thrombin completes conversion offibrinogen into fibrin, factor XIIIa (plasma transglutaminase; activated form of fibrin-stabilizing factor) promotes...
component offibrinogen, a blood-borne glycoprotein composed of three pairs of nonidentical polypeptide chains. Following vascular injury, fibrinogen is cleaved...
gene lead to several disorders, including afibrinogenemia, dysfibrinogenemia, hypodysfibrinogenemia and thrombotic tendency. Fibrinogen beta chain has been...
(HDL) variation, found by Eric Brunner (epidemiologist) of UCL; Sir Richard Way; fibrinogen variation, found by Prof Sam Machin, haematologist at University...
is influenced by the aggregation of red blood cells: blood plasma proteins, mainly fibrinogen, promote the formation of red cell clusters called rouleaux...
age-specific differences in the structure of blood clotting proteins are:[citation needed] Fibrinogen The fetal form offibrinogen has increased sialic acid and phosphorus...
pollution may increase the risk of developmental disorders (e.g., autism), neurodegenerative disorders, mental disorders, and suicide. It is associated...
2009). "Comparison of cytokine levels in depressed, manic and euthymic patients with bipolar disorder". Journal of Affective Disorders. 116 (3): 214–7....
therapy of choice in many conditions. Cryoprecipitate or fibrinogen concentrates should be used when fibrinogen is needed. For treatment of hemophilia...
activated in a sequence of events known as 'coagulation cascade' which leads to the formation of fibrin from inactive fibrinogen plasma protein. Thus, a...
liver disease, unlike chronic liver disease in which concentration offibrinogen remains unchanged. A previously undiagnosed liver disease may become...
Fibrinogen alpha chain is a protein that in humans is encoded by the FGA gene. The protein encoded by this gene is the alpha component offibrinogen, a...
and a wide range of other disorders. The term proteopathy was first proposed in 2000 by Lary Walker and Harry LeVine. The concept of proteopathy can trace...
fibers in the form offibrinogen.[citation needed] Blood performs many important functions within the body, including: Supply of oxygen to tissues (bound...
(2009, November 9). Blistering disorders in infancy. Retrieved from http://www.docstoc.com/docs/15402241/Blistering-Disorders-in-Infancy Kumar, V, A Abbas...
cleaves and therefore inactivates a significant part of circulating plasma fibrinogen. Fibrinogen is often found in increased concentrations in arteriae...
her research into fibrinogen. Thomas worked in the NHS as a haematologist for nearly 40 years where she specialised in the care of children with congenital...
isotope is necessary in tests that require several days of imaging, for example, fibrinogen scan imaging to detect blood clots. Both 123I and 125I emit...
treatment in the U.S. for acute bleeding episodes in patients with congenital fibrinogen deficiency, and the Swiss government awarded CSL Behring the Tell Award...
apolipoproteins A-I and B, fibrinogen, white blood cell count, homocysteine, N-terminal pro B-type natriuretic peptide (NT-proBNP), and markers of kidney function...
IXa in the activation of factor X, which, in turn, with its cofactor factor Va, activates more thrombin. Thrombin cleaves fibrinogen into fibrin which polymerizes...
Global Information