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SummaryEssentials    Recombinant von Willebrand factor (rVWF) is effective in von Willebrand disease (VWD).  A phase 3 study of rVWF, with/without recombinant factor VIII (rFVIII) before surgery in VWD.  Overall rVWF's efficacy was rated excellent/good; rVWF was administered alone in most patients.  rVWF was well‐tolerated and hemostasis was achieved in patients with severe VWD undergoing surgery.Summary   Background  Recombinant von Willebrand factor ( rVWF ) has demonstrated efficacy for on‐demand treatment of bleeding in severe von Willebrand disease ( VWD ), warranting evaluation in the surgical setting.    Objectives  This study ( NCT 02283268) evaluated the hemostatic efficacy/safety profile of  rVWF , with/without recombinant factor  VIII  ( rFVIII ), in patients with severe  VWD  undergoing surgery.    Patients/Methods  Patients received  rVWF  40–60  IU  kg −1 ,  VWF  ristocetin cofactor activity was measured 12–24 h before surgery. If endogenous  FVIII  activity ( FVIII :C) target levels were achieved 3 h before surgery,  rVWF  was administered alone 1 h before surgery;  rVWF  was co‐administered with  rFVIII  if target endogenous  FVIII  levels were not achieved.  rVWF  was infused postoperatively to maintain target trough levels. Overall and intraoperative hemostatic efficacy, the pharmacodynamics of  rVWF  administration and the incidence of adverse events ( AE s) were assessed.    Results  All patients treated with  rVWF  for major ( n  = 10), minor ( n  = 4) and oral ( n  = 1) surgery had overall and intraoperative hemostatic efficacy ratings of excellent (73.3% and 86.7%) or good (26.7% and 13.3%). Most  rVWF  infusions (89.4%) were administered alone, resulting in hemostatically effective levels of endogenous  FVIII  within 6 h, which were sustained for 72–96 h; 70% ( n  = 7/10) of major surgeries were performed without  rFVIII  co‐administration. Six patients reported 12 treatment‐emergent  AE s. Two patients each had one serious  AE : diverticulitis (not treatment related) and deep vein thrombosis (sponsor‐assessed as possibly treatment related). No severe allergic reactions or inhibitory antibodies were reported.    Conclusions  These data support the efficacy and safety profile of  rVWF  in patients with severe  VWD  undergoing elective surgery.

Phase 3 study of recombinant von Willebrand factor in patients with severe von Willebrand disease who are undergoing elective surgery