Factor XIII deficiency enhances thrombin generation due to impaired fibrin polymerization — An effect corrected by Factor XIII replacement

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• 作者：Hanna H. Pitkä ; nen^a ; ^b ; Annukka Jouppila^a ; Marja Lemponen^c ; Minna Ilmakunnas^b ; Jouni Ahonen^b ; ^d ; Riitta Lassila^c ; ^{riitta.lassila@hus.fi}
• 关键词：Congenital Factor XIII deficiency ; Thrombin generation ; Fibrin polymerization ; GPRP ; FXIII concentrate
• 刊名：Thrombosis Research
• 出版年：2017
• 出版时间：January 2017
• 年：2017
• 卷：149
• 期：Complete
• 页码：56-61
• 全文大小：383 K
• 卷排序：149

文摘

FXIII deficiency increases both TG in plasma and circulating prothrombin fragments. GPRP enhanced TG in normal and FXIII recovery plasma, indicating fibrin involvement. GPRP-modified fibrin structure mimicked FXIII deficiency. Modified fibrin structure attenuated the ATI action of polymerizing fibrin. In ROTEM FXIII replacement partially corrected the defect in fibrin clot formation.