Objective: To measure bone mineral density (BMD) in a group of patients meeting Gand criteria for Marfan syndrome, comparatively with a group of healthy controls.
Methods: Dual-energy X-ray absorptiometry (DEXA) was used to measure BMD at the hip and wrist in 130 patients seen at the Multidisciplinary Marfan Clinic, Paris, France. Results were compared to values in the database of the absorptiometry machine (Hologic QDR100) and to values in 72 healthy height-matched controls including 35 whose body mass index (BMI) values were similar to those in the patients.
Results: A history of fractures was noted in 32 (24.6%) patients. Z-score values were significantly decreased in the patients compared to the Hologic database values at the femoral neck (-1.190+/-0.098, P<0.0001) and wrist (-1.403+/-1.06; P < 0.001). Patients had significantly lower BMD values at the femoral neck compared to the height-matched controls (0.841+/-0.15 versus 1.010+/-0.017; P<0.0001). BMD values were also significantly lower in the patients compared to the controls of similar height and BMI. BMD values did not correlate with history of fractures or acetabular protrusion. In the patients, BMD values lower than -2.5 correlated with presence of dural ectasia.
Conclusion: Men and women with Marfan syndrome have significant osteopenia independent from BMI.