Hemophilia B Leyden is characterized by low levels of factor IX antigen and activity before the age of 15, whereas after puberty factor IX levels rise at a rate of about 5% per year. A single base substitution (-A----T) at position -20 was identified in the putative promoter of the gene cloned from a patient with hemophilia B Leyden. This nucleotide change was confirmed in a second patient from...

Background: Factor VIII administration to hemophilia A patients results in an immune response (inhibitor formation) which significantly complicates the therapy. The present study was performed to determine the prevalence of inhibitor development in hemophilia A patients receiving recombinant factor VIII therapy. Materials and Methods: This was an observational descriptive study. Clotting fac...

This case describes the management of a 12 year-old boy with mild hemophilia A, who was referred to Department of Pediatric Dentistry for orthodontic extractions. This case was treated under Desmopressin and tranexamic acid cover with caution related to local anesthesia administration technique which highlighted the importance of close and detailed liaison with the hematology team to formulate ...

1. Goudemand J, Rothschild C, Demiguel V, et al. Influence of the type of factor VIII concentrate on the incidence of factor VIII inhibitors in previously untreated patients with severe hemophilia A. Blood. 2006;107:46-51. 2. Chalmers EA, Brown SA, Keeling D, et al. Early factor VIII exposure and subsequent inhibitor development in children with severe haemophilia A. Haemophilia. 2007;13:149-15...

Inhibitory antibodies to factors VIII or IX have the potential to affect a broad range of outcomes among people with hemophilia; however, their possible effect on growth and maturation has not been explored. We evaluated skeletal maturation (bone age), pubertal progression, serum testosterone levels, height velocity, and stature in the multicenter Hemophilia Growth and Development Study. A tota...

We investigated the presence of a recombinant event between the F8A gene located in intron 22 of the factor VIII gene and the two additional copies of F8A lying 500 Kb upstream of FVIII in severe hemophilic patients. The genomic DNA of 146 unrelated Italian patients with severe hemophilia A (HA) was hybridized with an F8A gene probe to detect the abnormal band patterns. A recombinant event was ...

Introduction: Hemophilia is uncommon in females and there is little knowledge about the clinical

1Thrombosis and Hemostasis Unit, Ospedale Niguarda, Milan, Italy; 2Arthur Bloom Haemophilia Centre, University Hospital of Wales, School of Medicine, Cardiff University, Cardiff, United Kingdom; 3SRH Kurpfalzkrankenhaus Heidelberg GmbH and Hemophilia Center, Heidelberg, Germany; 4Department of Internal Medicine, Rouen University Hospital, Rouen Cedex, France; 5Unidad de Hemostasia y Trombosis, ...

Our aim was to test the hypothesis that breastfeeding may reduce development of inhibitors in male infants with haemophilia by inducing an oral immune tolerance to factor VIII. To achieve that goal, we performed a structured epidemiological survey comprising all males born with severe haemophilia A (in all 100 patients, 19 with inhibitors) or haemophilia B (in all 16 patients, six with inhibito...