Studies of factor-VIll-related activities were performed in 62 obligate carriers of hemophilia A. comparing 23 daughters of hemophilic men (paternal carriers) with 39 mothers of hemophilic Sons whose fathers were normal by history (maternal carriers). Nineteen of the maternal carriers were mothers of at least 2 hemophiliacs and 20 were mothers of one. but from families whose other close male me...

Individuals with severe factor VIII deficiency experience recurrent hemorrhages and develop progressive joint damage. Large retrospective, nonrandomized studies of patient cohorts followed over decades show that factor prophylaxis initiated at an early age before the onset of recurrent bleeding reduces the incidence of hemophilic arthropathy. Two recent prospective, multicenter, randomized tria...

The eighth edition of the haemophilia B database (http://www.umds.ac. uk/molgen/haemBdatabase.htm ) lists in an easily accessible form all known factor IX mutations due to small changes (base substitutions and short additions and/or deletions of <30 bp) identified in haemophilia B patients. The 1713 patient entries are ordered by the nucleotide number of their mutation. Where known, details are...

BACKGROUND Children with hemophilia can experience problems with their mental health status and social functioning. AIM To assess the mental health status of hemophilic children. MATERIAL AND METHODS Thirty four hemophilic children aged 5 to 13 years were studied. A translation of the special survey to assess mental health in children, denominated "Domingo" Mental Health Survey was applied....

We used immunoblotting of purified factor VIII coagulant protein (FVIII) to localize FVIII inhibitor epitopes in 76 inhibitor plasmas to either the 92-kd FVIII polypeptide (and its 54-kd and/or 44-kd thrombin fragments), the 80-kd polypeptide (and its 72-kd thrombin fragment), or both of these polypeptides. We also used immunoblotting to examine the immunoglobulin class and subclass content of ...

INTRODUCTION Muscle hematomas are the second most common complication of hemophilia and insufficient treatment may result in serious and even life-threatening complications. Hemophilic dogs and rats do experience spontaneous muscle bleeding, but currently, no experimental animal model is available specifically investigating spontaneous muscle bleeds in a hemophilic setting. AIM The objective ...

The procoagulant protein F.VIII:C is noncovalently bound to von Willebrand factor (vWF) to give the factor VIII macromolecular complex. New highly purified preparations of isolated human F.VIII:C, devoid of vWF and about 500,000-fold purified, were administered to hemophilia A and von Willebrand disease (vWD) dogs to determine their hemostatic effectiveness and survival in the circulation. Two ...

Background and objectives: In hemophiliac patients, recurrent intra-articular bleeding leads to progressive arthropathy. Magnetic resonance imaging (MRI) is the gold-standard technique for joint assessment. The development of ultrasound protocols standardized scales such as “Hemophilia Early Arthropathy Detection with Ultrasound” (HEAD-US) “Point-of-care Ultrasonography” (POC-US) allow patient ...

A five year survey of the treatment of patients in the United Kingdom suffering from haemophilia and related disorders was carried out on behalf of the directors of haemophilia centres. The survey showed an increase in the number of patients receiving treatment from the centres, a substantial increase in the total amount of therapeutic materials used, and an increase in the average amount of fa...