Bypassing agents are the most commonly used medicines for the treatment of hemophilia patients with inhibitors. The aim of this study is to identify the cost components of management of bleeding vents in hemophilia patients with inhibitors in Iran. This study is a cross-sectional study using a bottom-up approach to determine the cost components of treatment of hemophilia patients with inh...

background: haemophilia a (ha) is an x-linked bleeding disorder caused by the absence or reduced activity of coagulation factor viii (fviii). coagulation factors are a group of related proteins that are essential for the formation of blood clots. the aim of this study was to genotype the coagulation factor viii gene mutations using inverse shifting pcr (is-pcr) in an iranian family with severe ...

conclusions vaccination of non-immune individuals against hav infection in high risk groups especially hemophilia and thalassemia patients is recommended. results did not show any differences about seroprevalence of hev among iranian general population. results anti-hav igg antibodies were observed more frequently in thalassemia patients (60/64; 93.8%) than in hemophilia patients (104/155; 67.1...

Background: Hemophilia A and B are the most frequent congenital coagulation disorders. This study was conducted to determine the prevalence of hepatitis B, C and human immunodeficiency viruses among hemophilic patients in Hamadan, Iran. Patients and Methods: In this study, patients with hemophilia A and B treated in Hamedan Hemophilia Center, Hamedan, Iran, were screened for hepatitis B, C and...

Objectives To gain insights into the usage of factor VIII (FVIII) products by patients diagnosed with moderate/severe hemophilia A, and to assess the impact and perceived importance of product storage. Methods In this study, 200 patients diagnosed with moderate or severe hemophilia A across seven countries participated. Data were collected via a 30-minute, face-to-face interview in six countr...

During a 4-year multicenter cooperative study of acquired factor VIII inhibitors in persons with hemophilia A. new inhibitors were detected in 31 of 1 .306 patients who entered the study without an inhibitor or the history of an inhibitor. The incidence of new inhibitors was eight per 1 .000 patient-years of observation. The factor Vlll:C level before inhibitor development was 0.03 U/mi in 29 m...

The plasma clearance rates of factors IX and VIII were determined in patients with hemophilia A and B who had received factor replacement by prolonged, continuous infusion of factor concentrates. The clearance rates were calculated by dividing the factor infusion rates by the steady-state plasma factor activities corrected for base-line factor activities. The mean factor IX clearance rate in ei...

The present study reports on the treatment of bleeding episodes and the natural history of factor VIII inhibitors in 4 patients with acquired haemophilia A postpartum. Low titre type II factor VIII inhibitors in 3 patients and high titre type I inhibitor in 1 patient became apparent immediately to 7 months after delivery. High dose human factor VIII concentrate substitution was effective in con...

The fifth edition of the haemophilia B database lists in easily accessible form all known factor IX mutations due to small changes (base substitutions and short additions and/or deletions of < 30bp) identified in haemophilia B patients. The 1,142 patient entries are ordered by the nucleotide number of their mutation. Where known, details are given on: factor IX activity, factor IX antigen in ci...

This review summarizes the evidence that collagen IV binding is physiologically important, and that the extravascular compartment of FIX is composed of type IV collagen. As we have previously demonstrated, 7 days post-infusion, FIXWT (BeneFIX) is able to control bleeding as well as the same dosage of Alprolix in hemophilia B mice, tested using the saphenous vein bleeding model (Alprolix is a ch...