Thrombin Generation in a Hemophilic Newborn

摘要: In hemophilia, excessive bleeding during delivery and in the neonatal period is a rare event.A French study of 754 hemophilic neonates corroborate their postulation – only 8 % newborns showed clinically overt bleeding, 71.5 patients diagnosis was made after neonatal-period. These findings are surprising since trauma normal vaginal may result such as echymosis or hematomas even healthy should be enough to trigger major suffering from hemophilia [1, 2]. The reasons for low incidence with probably peculiarities hemostatic system. Healthy term have values many clotting factors but also inhibitors, especially antithrombin (AT), tissue factor pathway inhibitor (TFPI), protein C. Despite procoagulant they an excellent hemostasis [3, 4]. previous work we focused on this discrepancy between prolonged conventional tests shown that these physiological levels natural inhibitors AT TFPI plasma compensate concentrations by allowing sufficient thrombin generation despite prothrombin [3–5]. Conventional vitro assays activated partial time (aPTT) (PT) give very simplified picture relative importance various components recent years, studies activation via extrinsic addition amounts lipidated (TF) activator more compatible milieu [6] suitable sensitive detection effects different proand anticoagulants, including VIII 50 150 %, (TG) [7]. aim measure TG small TF FVIII-deficient investigate whether physiologic explain hemophilia. lack plasma, pooled FVIII depleted. Then effect raising adult investigated. one newborn measured.