Bone mineral density in adults with Marfan syndrome.
OBJECTIVE
Reduced bone mineral density (BMD) has been reported in pre-menopausal women and children with Marfan syndrome (MFS). The bone mineral status of adult men with MFS is unknown. The objective of this study was to determine the BMD of adult men and women with MFS.


METHODS
BMD (g/cm(2)) was measured by dual-energy X-ray absorptiometry (DXA) of the lumbar spine (L1-L4), femoral neck and total hip in 25 adults (12 male) who fulfilled the 1996 MFS diagnostic criteria. The results were compared with age- and sex-matched controls and expressed as S.D. from the population mean (z score).


RESULTS
Overall, BMD was significantly reduced in the lumbar spine (z = -0.42 +/- 0.97, P < 0.05), total hip (z = -0.57 +/- 0.88, P < 0.005) and femoral neck (z = -0.51 +/- 0.88, P < 0. 005). In women alone, BMD was reduced at the femoral neck (z = -0.53 +/- 0.95, P < 0.05) and at the hip (z = -0.64 +/- 0.77, P < 0.005). In men, BMD was reduced at the femoral neck (z = -0.48 +/- 0.84, P < 0.05) with a non-significant trend to lower BMD at the hip (z = -0. 49 +/- 1.01, P = 0.054) and lumbar spine (z = -0.59 +/- 1.02, P = 0. 09).


CONCLUSIONS
Axial BMD is lower than normal in Marfan adults. This reduction may contribute to fractures seen in the Marfan population.
Carter
N
N
Wellcome Trust Centre for Human Genetics, Roosevelt Drive, Headington, Oxford OX3 7BN, UK.
Duncan
E
E
Wordsworth
P
P
D015502:Absorptiometry, Photon; D000328:Adult; D000368:Aged; D015519:Bone Density; D005260:Female; D005272:Femur Neck / Q000000981:diagnostic imaging; D006615:Hip / Q000000981:diagnostic imaging; D006801:Humans; D008159:Lumbar Vertebrae / Q000000981:diagnostic imaging; D008297:Male; D008382:Marfan Syndrome / Q000378:metabolism*; D008875:Middle Aged; D012727:Sex Characteristics