Skip to main content
SpringerLink
Log in

Thrombozyten und Gerinnung

  • Chapter
  • First Online:
Pädiatrie

  • 795 Accesses

Zusammenfassung

An den Aufgaben der Hämostase, die Fließfähigkeit des Bluts optimal zu erhalten und gleichzeitig Blutverluste nach Verletzungen zu verhindern, sind im Wesentlichen 3 Systeme beteiligt:

  1. 1.

    das Gefäßsystem selbst, ausgekleidet mit Endothel, das den Durchtritt von Wasser, Ionen, Sauerstoff, CO2, Nährstoffen, Stoffwechselprodukten, Proteinen und zellulären Komponenten ermöglichen, auf der anderen Seite jedoch den Austritt von Blut verhindern soll,

  2. 2.

    die Blutplättchen oder Thrombozyten und

  3. 3.

    die plasmatische Gerinnung.

Gefäßwand und Thrombozyten kooperieren in der primären Hämostase. Den Vorgang der Blutgerinnung bezeichnet man als sekundäre Hämostase, an der neben den eigentlichen Gerinnungsfaktoren, deren Inhibitoren und den Faktoren der Fibrinolyse auch die Thrombozyten einen wesentlichen Anteil haben.

Das zentrale Enzym der Hämostase ist Thrombin – wie die meisten der enzymatisch aktiven Gerinnungsfaktoren eine Serinprotease. Es ist der letztlich wirksame Faktor, der aus Fibrinogen proteolytisch Fibrinmonomere als Grundsubstanz für das Fibringerüst erzeugt. Die Generation von Thrombin ist das Produkt der Gerinnungskaskade, an der neben anderen aus Zymogenen aktivierten Serinproteasen, wie FXIIa, FXIa, FIXa, FVIIa, FXa, auch Kofaktoren wie FVIIIa sowie FVa beteiligt sind (Abb. 180.1).

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 249.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Literatur

  • Andrew M, Vegh P, Johnston M, Bowker J, Ofosu F, Mitchell L (1992) Maturation of the hemostatic system during childhood. Blood 80:1998–2005

    CAS  PubMed  Google Scholar 

  • Aschka I, Aumann V, Bergmann F et al (1996) Prevalence of factor V Leiden in children with thrombo-embolism. Eur J Pediatr 155:1009–1014

    Article  CAS  PubMed  Google Scholar 

  • Aster RH (1995) Heparin-induced thrombocytopenia and thrombosis. N Engl J Med 332:1374–1376

    Article  CAS  PubMed  Google Scholar 

  • Bazzano LA, Reynolds K, Holder KN, He J (2006) Effect of folic acid supplementation on risk of cardiovascular diseases: a meta-analysis of randomized controlledtrials. JAMA 296:2720–2726

    Article  CAS  PubMed  Google Scholar 

  • Bertina RM (1998) The prothrombin 20210 G to A variation and thrombosis. Curr Opin Hematol 5:339–342

    Article  CAS  PubMed  Google Scholar 

  • Bertina RM, Koeleman BP, Koster T et al (1994) Mutation in blood coagulation factor V associated with resistance to activated Protein C [see comments. Nature 369:64–67

    Article  CAS  PubMed  Google Scholar 

  • Bick RL (1998) Disseminated intravascular coagulation: pathophysiological mechanisms and manifestations. Semin Thromb Hemost 24:3–18

    Article  CAS  PubMed  Google Scholar 

  • Brenner B, Sanchez-Vega B, Wu SM, Lanir N, Stafford DW, Solera J (1998) A missense mutation in gamma-glutamyl carboxylase gene causes combined deficiency of all vitamin K-dependent blood coagulation factors. Blood 92:4554–4559

    CAS  PubMed  Google Scholar 

  • Broze GJ Jr (1995) Tissue factor pathway inhibitor. Thromb Haemost 74:90–93

    CAS  PubMed  Google Scholar 

  • Cooper DN, Millar DS, Wacey A, Banner DW, Tuddenham EG (1997) Inherited factor VII deficiency: molecular genetics and pathophysiology. Thromb Haemost 78:151–160

    CAS  PubMed  Google Scholar 

  • Cooper DN, Millar DS, Wacey A, Pemberton S, Tuddenham EG (1997) Inherited factor X deficiency: molecular genetics and pathophysiology. Thromb Haemost 78:161–172

    CAS  PubMed  Google Scholar 

  • Dahlback B, Carlsson M, Svensson PJ (1993) Familial thrombophilia due to a previously unrecognized mechanism characterized by poor anticoagulant response to activated Protein C: prediction of a cofactor to activated Protein C [see comments. Proc Natl Acad Sci U S A 90:1004–1008

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • David M, Andrew M (1993) Venous thromboembolic complications in children. J Pediatr 123:337–346

    Article  CAS  PubMed  Google Scholar 

  • David M, Manco-Johnson M, Andrew M (1995) Diagnosis and treatment of venous thromboembolism in children and adolescents. On behalf of the Subcommittee on Perinatal Haemostasis of the Scientific and Standardization Committee of the ISTH. Thromb Haemost 74:791–792

    CAS  PubMed  Google Scholar 

  • Eichinger S, Pabinger I, Stümpflen A et al (1997) The risk of recurrent venousthromboembolism in patients with and without factor V Leiden. Thromb Haemost 77:624–628

    CAS  PubMed  Google Scholar 

  • Engesser L, Broekmans AW, Briet E, Brommer EJP, Bertina RM (1987) Hereditary protein S deficiency: clinical manifestations. Ann Intern Med 106:677–682

    Article  CAS  PubMed  Google Scholar 

  • Fisher S, Rikover M, Naor S (1966) Factor 13 deficiency with severe hemorrhagic diathesis. Blood 28:34–39

    CAS  PubMed  Google Scholar 

  • Furie B, Furie BC (1990) Molecular basis of vitamin K-dependent gamma-carboxylation. Blood 75:1753–1762

    CAS  PubMed  Google Scholar 

  • George JN, Woolf SH, Raskob GE et al (1996) Idiopathic thrombocytopenic purpura: a practice guideline developed by explicit methods for the American Society of Hematology [see comments. Blood 88:3–40

    CAS  PubMed  Google Scholar 

  • Griffin JH, Evatt B, Zimmerman TS, Kleiss AJ, Wideman C (1981) Deficiency of protein C in congenital thrombotic disease. J Clin Invest 68:1370–1373

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Ingram GIC (1997) The history of haemophilia. Haemophilia 3(1):5–15

    Article  Google Scholar 

  • Haemost, T (2001) Homozygous antithrombin deficiency type II (99 Leu to Phe mutation) and childhood thromboembolism. 86:1007-1011.

    Google Scholar 

  • Higham JM, O’Brien PM, Shaw RW (1990) Br J Obstet Gynaecol 97:734–739

    Article  CAS  PubMed  Google Scholar 

  • Kuhle S, Lane DA, Jochmanns K, Male C, Quehenberger P, Lechner K, Pabinger I (2001) Homozygous antithrombin deficiency type II (99 Leu to Phe mutation) and childhood thromboembolism. Thromb Haemost. 86:1007–101

    Google Scholar 

  • Lillicrap D (1998) The molecular basis of haemophilia B. Haemophilia 4:350–357

    Article  CAS  PubMed  Google Scholar 

  • Mammen EF (1983) Factor V deficiency. Semin Thromb Hemost 9:1–72

    Article  CAS  PubMed  Google Scholar 

  • Mannucci PM (1998) Hemostatic drugs. N Engl J Med 339:245–253

    Article  CAS  PubMed  Google Scholar 

  • Mannucci PM (1998) treatment protocols around the world: towards a consensus. Haemophilia 4:421

    Article  CAS  PubMed  Google Scholar 

  • Mannucci PM (1998) Treatment of von Willebrand disease. Haemophilia 4:661–664

    Article  CAS  PubMed  Google Scholar 

  • McDonagh J, Carrell N, Lee MH (1994) Dysfibrinogenemia and other disorders of fibrinogen structure and function. In: Colman RW, Hirsh J, Marder VJ, Salzman JB (Hrsg) Hemostasis and thrombosis: basic principles and clinical practice, 3. Aufl. Lippincott, Philadelphia, S 314–334

    Google Scholar 

  • Peake I (1998) The molecular basis of haemophilia A. Haemophilia 4:346–349

    Article  CAS  PubMed  Google Scholar 

  • Ragni MV, Sinha D, Seaman F, Lewis JH, Spero JA, Walsh PN (1985) Comparison of bleeding tendency, factor XI coagulant activity, and factor XI antigen in 25 factor XI-deficient kindreds. Blood 65:719–724

    CAS  PubMed  Google Scholar 

  • Remond-O’Donnell E, Rosen FS, Kenney DM (1996) Defects in Wiskott-Aldrich syndrome blood cells. Blood 87:2621–2631

    Google Scholar 

  • Rost S, Fregin A, Ivaskevicius V et al (2004) Mutations in VKORC1 cause warfarin resistance and multiple coagulation factor deficiency type 2. Nature 427:537–541

    Article  CAS  PubMed  Google Scholar 

  • Ruggeri ZM (1991) Structure and function of von Willebrand factor: relationship to von Willebrand’s disease. Mayo Clin Proc 66:847–861

    Article  CAS  PubMed  Google Scholar 

  • Schneppenheim R, Thomas KB, Sutor AH (1995) Von Willebrand disease in childhood. Semin Thromb Hemost 21:261–275

    Article  CAS  PubMed  Google Scholar 

  • Schneppenheim R, Budde U (2011) von Willebrand factor: the complex molecular genetics of a multidomain and multifunctional protein. JTH 9(1):209–215

    CAS  PubMed  Google Scholar 

  • Schneppenheim R, Budde U, Oyen F et al (2003) Von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP. Blood 101:1845–1850

    Article  CAS  PubMed  Google Scholar 

  • Sutor AH (1995) Thrombocytosis in childhood. Semin Thromb Hemost 21:330–339

    Article  CAS  PubMed  Google Scholar 

  • Triplett DA (1998) Many faces of lupus anticoagulants. Lupus 7(2):18–22

    Article  Google Scholar 

  • Tuddenham EG, Pemberton S, Cooper DN (1995) Inherited factor VII defi­ciency: genetics and molecular pathology. Thromb Haemost 74:313–321

    CAS  PubMed  Google Scholar 

  • Wood AJJ (1998) Thrombopoietin. N Engl J Med 339:746–754

    Article  Google Scholar 

  • Zimmerman TS, Ratnoff OD, Powell AE (1971) Immunologic differentiation of classic hemophilia (factor 8 deficiency) and von Willebrand’s dissase, with observations on combined deficiencies of antihemophilic factor and proaccelerin (factor V) and on an acquired circulating anticoagulant against antihemophilic factor. J Clin Invest 50:244–254

    Article  CAS  PubMed Central  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. MVZ wagnerstibbe, amedes Gruppe, Georgstr. 50, 30159, Hannover, Deutschland

    Frauke Bergmann Dr. med.

  2. Klinik und Poliklinik für Pädiatrische Hämatologie und Onkologie, Universitätsklinikum Hamburg-Eppendorf, Martinistr. 52, 20246, Hamburg, Deutschland

    Reinhard Schneppenheim Prof. Dr. rer. nat.

Authors
  1. Reinhard Schneppenheim Prof. Dr. rer. nat.
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Frauke Bergmann Dr. med.
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Zentrum für Kinder und Jugendmedizin, Universitätsklinikum Heidelberg, Heidelberg, Germany

    Georg F. Hoffmann

  2. Forschungsinstitut für Kinderernährung, Dortmund, Germany

    Michael J. Lentze

  3. Zentrum für Kinder- und Jugendmedizin, Universitätsmedizin Mainz, Mainz, Germany

    Jürgen Spranger

  4. Zentrum für Kinder- und Jugendmedizin, Universitätsmedizin Mainz, Mainz, Germany

    Fred Zepp

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Schneppenheim, R., Bergmann, F. (2014). Thrombozyten und Gerinnung. In: Hoffmann, G., Lentze, M., Spranger, J., Zepp, F. (eds) Pädiatrie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-41866-2_180

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-41866-2_180

  • Published:

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-41865-5

  • Online ISBN: 978-3-642-41866-2

  • eBook Packages: Medicine (German Language)

Publish with us

Policies and ethics

Search

Navigation