Osteoporosis in adult males is an underrecognized problem. Patients with haemophilia have several predisposing factors for developing decreased bone mineral density (BMD) including prolonged periods of immobility, reduced weight bearing and co-morbidities associated with bone loss. To establish prevalence and risk factors associated with decreased BMD in patients with haemophilia. Adults with moderate or severe haemophilia A or B underwent dual-energy X-ray absorptiometry (DXA). BMD was correlated to laboratory values, joint mobility measurements and physical activity questionnaires. Thirty patients completed evaluations. The median age was 41.5 years (range 18-61). Median lowest T-score by DXA was )1.7 (range: )5.8 to +0.6), with the femoral neck being the site of the lowest T-scores. Based on World Health Organization criteria, 70% of patients had decreased BMD. Twenty-seven per cent of the participants (n = 8) had osteoporosis and 43% (n = 13) had osteopenia. Variables associated with increased bone loss included lower serum 25-hydroxyvitamin D levels (P = 0.03), lower body mass index (P = 0.047), lower activity scores (P = 0.02), decreased joint range of motion (P = 0.046), HIV (P = 0.03), HCV (P = 0.02), history of inhibitor (P = 0.01) and age (P = 0.03). Adults with haemophilia are at increased risk for developing osteoporosis. A history of HCV and HIV infections, decreased joint range-of-motion, decreased activity levels, history of an inhibitor and low body weight predict bone loss and suggest a population to target for screening. A high prevalence of vitamin D insufficiency was observed. Future studies should investigate interventions, including vitamin D supplementation, to prevent bone loss and fractures for this at-risk population.

Multiple factors place adults with haemophilia at risk for depression. Health outcomes can be compromised in depressed patients secondary to increased risk taking behaviour and poor compliance with treatment recommendations. To assess the prevalence and risk factors associated with depression in adult patients with haemophilia treated at a haemophilia treatment centre. Adults with haemophilia were screened for depression during their annual clinic visit using the Patient Health Questionnaire 9 (PHQ-9), a validated tool for depression screening in adults. Depression was defined as a PHQ-9 score ≥ 5. Risk factors associated with depression were collected by chart review and correlated with depression scores. A total of 41 adult patients consented to the study and 37% met criteria for depression. Fifty-three per cent of patients with depression reported moderate to severe symptoms of depression (PHQ-9 score >10). Seventy-six per cent of patients with depression reported suffering functional impairment due to their depressive symptoms. Lack of social support and unemployment were significantly associated with higher PHQ-9 scores (P = 0.04 and P = 0.01 respectively). Adult patients with haemophilia have a high prevalence of depression. The addition of depression screening to the comprehensive care of adults with haemophilia may result in improved overall health outcomes and treatment adherence.

Prophylaxis for hemophilia A with conventional factor VIII (FVIII) products requires frequent intravenous dosing, which may reduce adherence. Recombinant factor VIII Fc fusion protein (rFVIIIFc) has a prolonged half-life compared with conventional rFVIII, and has demonstrated safety and efficacy for the prevention and treatment of bleeding episodes in phase 3 studies of patients with severe hemophilia A. Most subjects experienced reduced prophylactic dosing frequency with rFVIIIFc compared with prestudy FVIII; the median total weekly prophylactic consumption was comparable. No subjects developed inhibitors. These results suggest that prophylaxis with rFVIIIFc in patients with hemophilia A may allow less frequent prophylactic dosing while maintaining efficacy, with comparable prophylactic consumption.

Background: Osteoporosis among adult males is a major and under-recognized problem in the United States. Patients with hemophilia have several predisposing risks for developing decreased bone mineral density (BMD) and osteoporosis, and may represent an important group to target for screening and treatment for fracture prevention. Patients and Methods: Patients over the age of 18 with moderate or severe hemophilia A or B (as defined by factor activity < 5%) and no history of prophylactic factor use prior to age 10 were eligible. Bone mineral densities were obtained using DEXA scans (DXA) along with measurements of joint mobility and physical activity and laboratory parameters. Results: Twenty-eight patients have been consented with accrual ongoing. 21 have undergone DXA scans. Median age of 39 (range 18–61), 86% HCV positive, 26% HIV positive. Median T-score for all sites (lumbar, femoral neck, hip, and other) was −1.7 (−5.8–0.6), with the most effected area being the femoral neck, T-score −1.7 (−5.8–0.8). Based on WHO criteria, 76% of patients had decreased BMD, 33% (n=7) with osteoporosis, and 43% (n=9) with osteopenia. Trends associated with decreased BMD included decreased serum 25-hydroxy-vitamin D levels, increased alkaline phosphatase, and decreased weight. All patients with osteoporosis were HCV positive, and all HIV positive patients had decreased BMD. Median activity scores were lower among osteoporotic patients vs normal BMD. Joint range-of-motion in the lower extremities was limited to 59.5% of predicted values in patients with osteoporosis, 84% in osteopenia, and 93% in patients with normal BMD. Summary: Patients with hemophilia are at markedly increased risk for developing osteoporosis and osteopenia. Potential predictors of risk for decreased BMD are concurrent HCV and HIV infection, low vitamin D levels, elevated alkaline phosphatase, lower weight, decreased range of motion and lower activity scores. More aggressive screening for decreased BMD among moderate and severe hemophilia patients with initiation of therapy is appropriate. Median Values Worst T-Score Activity Score (1–5) Joint ROM (% Pred) Weight (kg) 25-Hydroxy-D (ng/mL) Alk Phos (IU/L) Normal BMD 0.1 5 94.0 91.6 28.0 59 Osteopenia −1.6 4 85.0 80.7 23.0 86 Osteoporosis −3.0 3 68.5 73.0 21.8 99

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