Assessment of bone mineral density in women with Marfan syndrome.

Although radiological studies suggest that Marfan syndrome is associated with osteopenia, investigations utilizing measurements of bone mass have yielded conflicting results. To address this question further, we measured bone mineral density (BMD) in 14 women with Marfan syndrome at the right hip and lumbar spine by dual energy X-ray absorptiometry (DXA). Results were compared with the age- and weight-matched reference population supplied by the DXA manufacturer, and with our own control population of normal women, the latter being matched for height, in addition to age and weight. We found that BMD was very similar in our Marfan women as compared with the two reference populations, at the lumbar spine and femoral neck, while trochanteric BMD was reduced. However, the relationship between an isolated reduction in trochanteric BMD and future fracture risk is unclear, and, on the basis of our results, we conclude that Marfan syndrome is not associated with a clinically significant increase in the risk of osteoporotic fracture.