title: Assessment of Selected ROTEM Parameters, Kinetics of Fibrinogen Polymerization and Plasmin Amidolytic Activity in Patients with Congenital Fibrinogen Defects.
abstract: BACKGROUND
  Congenital fibrinogen disorders (CFD) are rare fibrinogen deficiencies which may be quantitative or functional. The clinical course of hypofibrinogenemia (hypoFI) or dysfibrinogenemia (dysFI) is unpredictable and cannot be determined by the application of standard hemostasis tests.
  
  
  OBJECTIVE
  The main aim of this study was to assess ROTEM parameters in CFD patients.
  
  
  METHODS
  Nine patients with CFD were studied. The fibrinogen concentration was measured functionally and antigenically. EXTEM, INTEM, FIBTEM and APTEM tests were used to measure selected ROTEM parameters, including maximum clot firmness (MCF). Fibrin plasma polymerization, clot lysis and plasmin amidolytic activity were determined by spectrophotometric methods.
  
  
  RESULTS
  Incorporating the antigenic, ELISA method, to the diagnostic workup allowed the initial diagnosis to be switched from hypoFI to dysFI in 3/7 patients. MCF readings (the most important parameter describing fibrin polymerization capacity) were significantly lower in patients than in controls according to all ROTEM tests. Cases with hypoFI demonstrated markedly lower readings of MCF according to all ROTEM tests than cases with dysFI. All patients demonstrated disturbances of fibrin polymerization process assessed by turbidimetry. In contrast, no marked differences were identified between studied groups in reference to plasmin amidolytic activity.
  
  
  CONCLUSIONS
  Our data suggests that ROTEM and fibrin plasma polymerization according to the turbidimetric method have a high sensitivity towards detection of different CFD. Although ROTEM MCF assessment may help discriminate patients with hypoor dysfibrinogenemia, this finding has to be confirmed on larger groups of patients.
authors.affiliation: Department of Hematology, Medical University of Łódź, Poland.|Department of Hemostasis, Medical University of Łódź, Poland.
authors.forename: Jacek
authors.initials: J
authors.lastname: Treliński
authors.affiliation: Department of Hemostasis, Medical University of Łódź, Poland.
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Pachniewska
authors.affiliation: Department of General Biochemistry, Faculty of Biology and Environmental Protection, University of Łódź, Poland.
authors.forename: Justyna
authors.initials: J
authors.lastname: Matczak
authors.affiliation: Department of Hemostasis, Medical University of Łódź, Poland.
authors.forename: Marta
authors.initials: M
authors.lastname: Robak
authors.affiliation: Department of Hemostasis, Medical University of Łódź, Poland.
authors.forename: Krzysztof
authors.initials: K
authors.lastname: Chojnowski
mesh_terms: D000328:Adult; D000347:Afibrinogenemia / Q000097:blood*; D000369:Aged, 80 and over; D000577:Amides / Q000378:metabolism*; D001777:Blood Coagulation; D005260:Female; D005340:Fibrinogen / Q000378:metabolism*; D005341:Fibrinolysin / Q000378:metabolism*; D006461:Hemolysis; D006487:Hemostasis; D006801:Humans; D007700:Kinetics; D008297:Male; D008875:Middle Aged; D058105:Polymerization; D012016:Reference Values; D012399:Rotation; D013916:Thrombelastography / Q000379:methods*; D055815:Young Adult
chemical_list: D000577:Amides; D005340:Fibrinogen; D005341:Fibrinolysin
keywords: ROTEM; congenital fibrinogen disorders; fibrin plasma polymerization by turbidimetric method