M.S. is now a 23 year old man who lives in the Caribbean islands. He was born with severe Factor VIII deficiency and at an early age developed Factor VIII inhibitors. He and his family fly from the islands to the U.S. mainland to receive routine hemophilia care since there are no physicians taking care of hemophilia patients and no hemophilia treatment centers on the islands. He has been expose...

By utilizing a simple modification of previous immunological assays, we have demonstrated that most, if not all, hemophilic plasmas contain antigen reactive with human antibodies directed against Factor VIII procoagulant activity (VIIIc). Antibodies developing in a nonhemophiliac patient and in a hemophiliac patient gave similar results. The VIIIc antigen so identified was removed from hemophil...

Background: Psoriatic arthropathy is a seronegative arthropathy seen in some patients with psoriasis, a self-limited and genetically determined skin disease. Objective: To determine the prevalence and risk factors of arthropathy in patients with psoriasis. Patients and Methods: In a prospective, cross-sectional study, psoriatic patients referred to Razi Skin Hospital in Tehran, Iran in 20...

OBJECTIVE Children with severe hemophilia are at risk for reduced bone mineral density (BMD) because of reduced weight-bearing exercise and hepatitis C infection. Reduced bone density in childhood is a risk factor for osteoporosis in later life. STUDY DESIGN We performed a cross-sectional survey of bone density among 19 children with severe hemophilia, at the Royal Children's Hospital. Result...

OBJECTIVE The purpose of this study was to compare mortality risks of patients with Charcot arthropathy with those of patients with diabetic foot ulcer and those of patients with diabetes alone (no ulcer or Charcot arthropathy). RESEARCH DESIGN AND METHODS A retrospective cohort of 1,050 patients with incident Charcot arthropathy in 2003 in a large health care system was compared with patient...

Worldwide, haemophilia is the most common hereditary bleeding disorder. The incidence of haemophilia B, however, is considerably less than haemophilia A and consequently appears to have received less attention in the research literature. This article aims to summarise the available evidence documenting the patient and economic burden associated with haemophilia B and current methods of disease ...

BACKGROUND AND OBJECTIVES Recombinant factor IX Fc fusion protein (rFIXFc) is a clotting factor developed using monomeric Fc fusion technology to prolong the circulating half-life of factor IX. The objective of this analysis was to elucidate the pharmacokinetic characteristics of rFIXFc in patients with haemophilia B and identify covariates that affect rFIXFc disposition. METHODS Population p...

Haemophilia is characterized by intra-articular bleeding, often requiring immobilization, which may result in muscle atrophy and impaired proprioception. The aim of the study was to investigate differences in proprioceptive performance and isometric muscular strength of the lower limbs in haemophilic subjects compared with control subjects. Twelve subjects with severe haemophilia (11 haemophili...