New and expensive therapeutic products can place great pressure on health care purchasers. Often, evidence to support the inclusion of such products in the purchasing process is lacking or confusing, yet demand can be organized and forceful. In this paper we use the example of the introduction of recombinant factor VIII (rFVIII) in the management of haemophilia A, to highlight some problems pur...

Six recombinant factor VIII (rFVIII) products have been marketed worldwide. In 2013, the Research of Determinants of Inhibitor Development (RODIN) study group reported an unexpectedly high risk of inhibitor development with a second-generation full-length rFVIII (Product D) in previously untreated patients (PUPs) with severe hemophilia A (HA). In 1994, French public health authorities establish...

A genomic DNA obtained from a female patient with complete b-subunit deficiency was examined by Southern blotting analysis and in vitro amplification. Nucleotide sequence analysis showed that adenosine-4161 at the acceptor splice junction of intron A/exon II was deleted in half of the amplified DNAs, resulting in a loss of the obligatory AG splicing sequence. The absence of adenosine-4161 was c...

Patients with severe coagulation factor VIII deficiency require frequent infusions of human factor VIII (hFVIII) concentrates to treat life-threatening hemorrhages. Because these patients are immunologically hFVIII-naive, a significant treatment complication is the development of inhibitors or circulating alloantibodies against hFVIII, which bind the replaced glycoprotein, increase its plasma c...

Classic hemophilia A (factor VIII:C deficiency) was diagnosed in a miniature Schnauzer dog and a breeding program established. Inbreeding and crossbreeding produced 16 hemophilic animals. All were initially treated with canine cryoprecipitate, as required, for sporadic hemorrhagic events. Five animals developed potent antibodies to canine factor VIII:C. All were the offspring of obligate carrie...

Venous thromboembolism is uncommon after knee arthroscopy, and there are no guidelines for thromboprophylaxis in elective routine knee arthroscopy. Preoperative evaluation of common thrombophilias should provide guidance for postarthroscopy thromboprophylaxis in otherwise healthy patients who are at high risk for venous thromboembolism. This study assessed 10 patients with venous thromboembolis...

Introduction. Hemophilia is an X-linked hereditary blood clotting disorder caused by insufficiency of factor VIII or IX that affects mainly men. In extremely rare cases, the disease can be observed in women, which most often associated with asymmetric inactivation X chromosome. The severity hemophilia women does not differ from Aim – to present a clinical observation surgical treatment stage 4 ...

Von Willebrand disease (vWD) is a frequent inherited disorder of hemostasis that affects both sexes. Two abnormalities are characteristic of the disease, which is caused by a deficiency or a defect in the multimeric glycoprotein called von Willebrand factor: low platelet adhesion to injured blood vessels and defective intrinsic coagulation owing to low plasma levels of factor VIII. There are 2 ...

Correspondence: Başak Ünver Koluman, Dışkapı Yıldırım Beyazıt Eğitim ve Araştırma Hastanesi, Hematoloji Kliniği, Ankara, Türkiye Email: [email protected] Received: 02.12.2013, Accepted: 04.03.2014 Copyright © JCEI / Journal of Clinical and Experimental Investigations 2014, All rights reserved JCEI / 2014; 5 (2): 323-325 Journal of Clinical and Experimental Investigations doi: 10.5799/ahinjs....

Von Willebrand disease (vWD) is a frequent inherited disorder of hemostasis that affects both sexes. Two abnormalities are characteristic of the disease, which is caused by a deficiency or a defect in the multimeric glycoprotein called von Willebrand factor: low platelet adhesion to injured blood vessels and defective intrinsic coagulation owing to low plasma levels of factor VIII. There are 2 ...