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The dysfibrinogenemias are a group of autosomal dominant disorders of qualitatively abnormal fibrinogens.^[1] There are more than 350 different fibrinogen abnormalities, each named after the place where it was discovered.^[2] Each dysfibrinogenemia is associated with slightly different effects on the thrombin time and on normal clotting. Some dysfibrinogenemias cause abnormal bleeding or even thrombosis, while others have no effect on either bleeding or thrombosis.^[3]

Examples

Amsterdam is a major defect, characterized by aggregation of fibrin monomers, prolonged thrombin time, and an inhibitory effect on normal clotting - but it is asymptomatic.
Detroit is a major defect, there is fibrinopeptide release, the thrombin time is prolonged, there is an inhibitory effect on normal clotting and there is abnormal bleeding.
Wiesbaden is a major defect, there is aggregation of fibrin monomers, the thrombin time is prolonged, there is an inhibitory effect on normal clotting and there is both bleeding and thrombosis.

References

^ Dysfibrinogenemia at eMedicine
^ McDonagh, J (2001). "Dysfibrinogenemia and other disorders of fibrinogen structure or function". In Colman R, Hirsh J, Marder V, Clowes A, George J. Hemostasis and Thrombosis (4th ed.). Philadelphia: Lippincott Williams & Wilkins. pp. 855–92. ISBN 978-0-7817-1455-6.
^ Hayes, T (2002). "Dysfibrinogenemia and thrombosis". Archives of Pathology & Laboratory Medicine 126 (11): 1387–90. doi:10.1043/0003-9985(2002)126<1387:DAT>2.0.CO;2 (inactive 2015-05-13). PMID 12421146.

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UpToDate Contents

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1. フィブリノゲンの異常 disorders of fibrinogen
2. 静脈血栓症の原因の概要 overview of the causes of venous thrombosis
3. 肝疾患患者における凝固異常 coagulation abnormalities in patients with liver disease
4. 血栓溶解異常による血栓性および出血性疾患 thrombotic and hemorrhagic disorders due to abnormal fibrinolysis
5. 出血性素因を有する成人患者へのアプローチ approach to the adult patient with a bleeding diathesis

English Journal

Fibrinogen Replacement Therapy for Congenital Fibrinogen Deficiency.

Bornikova L, Peyvandi F, Allen G, Bernstein J, Manco-Johnson MJ.SourceHemophilia and Thrombosis Center, Department of Medicine, University of Colorado, Aurora, CO, USA Maggiore Hospital Foundation at the University of Milan, Milan, Italy Rhode Island Hospital, Brown University Medical Center, Providence, RI, USA Children's Center for Cancer and Blood Diseases of Las Vegas, Las Vegas, NV, USA Hemophilia and Thrombosis Center, Department of Pediatrics, University of Colorado, Aurora, CO, USA.
Journal of thrombosis and haemostasis : JTH.J Thromb Haemost.2011 Jun 29. doi: 10.1111/j.1538-7836.2011.04424.x. [Epub ahead of print]
Background: This review of published studies was conducted to derive data on patients with congenital fibrinogen deficiency (CFD) including dosing of fibrinogen replacement therapy, outcome and adverse events either temporally related or distant to fibrinogen replacement in order to assist clinician
PMID 21711446

Japanese Journal

Analysis of plasmin generation and clot lysis of plasma fibrinogen purified from a heterozygous dysfibrinogenemia, B beta Gly15Cys (Hamamatsu II)

Kamijyo Yuka,Hirota-Kawadobora Masako,Terasawa Fumiko,Honda Takayuki,Ikeya Manae,Okumura Nobuo
BLOOD COAGULATION & FIBRINOLYSIS 20(8), 726-732, 2009-12
… 20(8):726-732, December 2009.We found a heterozygous dysfibrinogenemia caused by the substitution of B beta Gly15Cys and designated it fibrinogen Hamamatsu II (H-II). … Therefore, we did not clarify an association between the B beta Gly15Cys dysfibrinogenemia and arterial thrombosis. …
NAID 120002696304