Assessment of Bone Mineral Density in Women With Marfan Syndrome

Abstract

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.

Bone Mineral Density Determinations by Dual-Energy x-ray Absorptiometry in the Management of Patients with Marfan Syndrome—Some Factors Which Affect the Measurement

“…The results of comparisons of the study of Giampietro et al [11] and the other published studies that reported actual BMD values [7][8][9][10] in male and female subjects with Marfan syndrome are summarized in Tables 1 and 2. Reduced BMD was previously reported in the femoral neck of adult men in two studies [9,10].…”

“…Analytical studies of BMD reported in the literature on adult patients with Marfan syndrome [7][8][9][10][11] were retrospectively reviewed. The following definitions for categories established by the World Health Organization for clinical characterization of BMD will be operative for this report.…”

“…One-way ANOVA with Student-Newman-Keuls pairwise comparisons was used to compare BMD values obtained from the study of Giampietro et al [11] to other published studies [7][8][9][10].…”

“…Conflicting reports have described generalized or localized decreased BMD in Marfan syndrome [5][6][7][8][9][10]. Information is lacking regarding the management and prevention of osseous complications in patients with Marfan syndrome.…”

“…We speculated whether these differences could be related to the instrumentation used. To address this possibility, we compared the results of the study of Giampietro et al [11] to other published studies, which employed either the Lunar DPX or Hologic QDR 1000W dual-energy x-ray absorptiometry (DXA) bone densitometers to assess BMD in patients with Marfan syndrome [7][8][9][10].…”