Abstract
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
Bioavailable vitamin D levels are reduced and correlate with bone mineral density and markers of mineral metabolism in adults with nephrotic syndrome
