Seltene Ursache einer hämorrhagischen Diathese: Diagnostik und perioperatives Management bei hereditärer Dysfibrinogenämie

Research output: Contribution to journalResearch articleContributedpeer-review

Contributors

  • J. Radke - , University Hospital Carl Gustav Carus Dresden, Department of internal Medicine I (Author)
  • M. Teich - , Onkologische Gemeinschaftspraxis Dres. Grundeis und Teich (Author)
  • M. Meyer - , University of Applied Sciences Jena (Author)
  • S. Kirschner - , University Hospital Carl Gustav Carus Dresden, University Center for Orthopedics, Trauma and Plastic Surgery (OUPC) (Author)
  • J. Neidel - , University Hospital Carl Gustav Carus Dresden, Department of Anesthesiology and Intensive Care Medicine (Author)
  • G. Ehninger - , University Hospital Carl Gustav Carus Dresden, Department of internal Medicine I (Author)
  • G. Siegert - , University Hospital Carl Gustav Carus Dresden, Institute for Clinical Chemistry and Laboratory Medicine (Author)
  • U. Platzbecker - , University Hospital Carl Gustav Carus Dresden, Department of internal Medicine I (Author)

Abstract

Before elective surgery, it is mandatory that a precise history be taken to detect increased hemorrhagic diathesis and that thrombocytes, Quick/INR, and aPTT be determined. If pathological levels are found, further laboratory tests are necessary after frequent causes (e.g., liver cirrhosis) have been excluded. Single-factor analysis for the von Willebrand's factor antigen and if necessary further tests to check for von Willebrand's syndrome (multimeric analysis) as well as platelet function tests should be performed. Dysfibrinogenemia is a rare coagulation disorder, which causes elevated INR. It shows a wide spectrum of clinical manifestations including thrombophilia, excessive bleeding, and even asymptomatic cases. We present a 72-year-old patient with asymptomatic dysfibrinogenemia who needed hip replacement due to arthrosis. Lowered fibrinogen levels were substituted prior to operation and the clinical course afterwards was uneventful under additional prophylactic anticoagulation in order to prevent thrombosis. The case report illustrates the interdisciplinary teamwork which is very important in the management of patients with coagulation disorders.

Translated title of the contribution
A rare coagulation disorder. Diagnostics and management in cases of hereditary dysfibrinogenemia

Details

Original languageGerman
Pages (from-to)230-234
Number of pages5
JournalInternist
Volume50
Issue number2
Publication statusPublished - Feb 2009
Peer-reviewedYes

External IDs

PubMed 19043687

Keywords

ASJC Scopus subject areas

Keywords

  • Coagulation disorders, Hereditary dysfibrinogenemia, Hypofibrinogenemia, Perioperative management