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Diagnostic value of clot formation parameters determined by rotational thromboelastometry in 63 patients with congenital dysfibrinogenemia.
Simurda, Tomas; Marchi, Rita; Casini, Alessandro; Neerman-Arbez, Marguerite; Drotarova, Miroslava; Skornova, Ingrid; Zolkova, Jana; Kolkova, Zuzana; Loderer, Dusan; Brunclikova, Monika; Belakova, Kristina Maria; Stasko, Jan.
Afiliación
  • Simurda T; National Centre of Hemostasis and Thrombosis, Department of Hematology and Transfusiology, Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, Martin, Slovakia.
  • Marchi R; Division of Angiology and Hemostasis, University Hospitals of Geneva and Faculty of Medicine.
  • Casini A; Division of Angiology and Hemostasis, University Hospitals of Geneva and Faculty of Medicine.
  • Neerman-Arbez M; Department of Genetic Medicine and Development, Faculty of Medicine, University of Geneva, Geneva, Switzerland.
  • Drotarova M; National Centre of Hemostasis and Thrombosis, Department of Hematology and Transfusiology, Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, Martin, Slovakia.
  • Skornova I; National Centre of Hemostasis and Thrombosis, Department of Hematology and Transfusiology, Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, Martin, Slovakia.
  • Zolkova J; National Centre of Hemostasis and Thrombosis, Department of Hematology and Transfusiology, Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, Martin, Slovakia.
  • Kolkova Z; Biomedical Center Martin, Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin, Martin, Slovakia.
  • Loderer D; Biomedical Center Martin, Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin, Martin, Slovakia.
  • Brunclikova M; National Centre of Hemostasis and Thrombosis, Department of Hematology and Transfusiology, Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, Martin, Slovakia.
  • Belakova KM; National Centre of Hemostasis and Thrombosis, Department of Hematology and Transfusiology, Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, Martin, Slovakia.
  • Stasko J; National Centre of Hemostasis and Thrombosis, Department of Hematology and Transfusiology, Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin and University Hospital in Martin, Martin, Slovakia.
Blood Coagul Fibrinolysis ; 35(2): 56-61, 2024 Mar 01.
Article en En | MEDLINE | ID: mdl-38251440
ABSTRACT
Rotational thromboelastometry (ROTEM) is a global hemostasis assay. The diagnosis added value of ROTEM in congenital dysfibrinogenemia remains to be established. The aim of this study was to analyze clot formation by ROTEM in a cohort of dysfibrinogenemic patients and to establish correlations with genotype, clinical features, and coagulation parameters. The study included genetically confirmed congenital dysfibrinogenemia cases (n = 63) and healthy controls ( n  = 50). EXTEM, INTEM, FIBTEM tests were used to measure ROTEM parameters, that is, clotting time (CT), clot formation time (CFT), maximal clot firmness (MCF) and amplitude 10 min after CT (A10). The ISTH bleeding assessment tool was used to determine bleeding episodes. CT (INTEM) was statistically significantly shorter in congenital dysfibrinogenemia patients compared to controls while CFT (EXTEM) was prolonged. Patients's MCF in EXTEM, INTEM, and FIBTEM were similar to controls while A10 (FIBTEM) was statistically significantly lower. Fibrinogen activity was positively correlated with fibrinogen antigen, A10 and MCF in all three assays. Bleeding phenotypes were observed in 23 (36.5%) patients. Only CFT in EXTEM and CT in INTEM were statistically different in patients with bleeding phenotype versus controls. Carriers of the FGA mutation p.Arg35His had a CT (EXTEM) slightly prolonged and a reduced A10 (FIBTEM) compared to controls. Some ROTEM parameters were able to distinguish congenital dysfibrinogenemia patients from controls, and patients with a bleeding phenotype. Prolonged CFT in EXTEM were associated with congenital dysfibrinogenemia and bleeding phenotype. Bleeding episodes in most patients were generally mild and prevalence of thrombosis was very low.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Piperidonas / Tromboelastografía / Afibrinogenemia / Bencenoacetamidas / Hemorragia Tipo de estudio: Diagnostic_studies / Observational_studies / Risk_factors_studies Límite: Humans Idioma: En Revista: Blood Coagul Fibrinolysis Asunto de la revista: ANGIOLOGIA / HEMATOLOGIA Año: 2024 Tipo del documento: Article País de afiliación: Eslovaquia Pais de publicación: Reino Unido
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Piperidonas / Tromboelastografía / Afibrinogenemia / Bencenoacetamidas / Hemorragia Tipo de estudio: Diagnostic_studies / Observational_studies / Risk_factors_studies Límite: Humans Idioma: En Revista: Blood Coagul Fibrinolysis Asunto de la revista: ANGIOLOGIA / HEMATOLOGIA Año: 2024 Tipo del documento: Article País de afiliación: Eslovaquia Pais de publicación: Reino Unido