Bone mineral density in Marfan syndrome. A large case-control study.
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.


CONCLUSIONS
Men and women with Marfan syndrome have significant osteopenia independent from BMI.
Moura
Bertrand
B
Rheumatology Department, Ambroise-Paré Teaching Hospital, 9, avenue Charles-de-Gaulle, 92100 Boulogne-Billancourt, France.
Tubach
Florence
F
Sulpice
Moana
M
Boileau
Catherine
C
Jondeau
Guillaume
G
Muti
Christine
C
Chevallier
Bertrand
B
Ounnoughene
Yasmine
Y
Le Parc
Jean-Marie
JM
D015502:Absorptiometry, Photon; D000328:Adult; D015992:Body Mass Index; D015519:Bone Density; D001851:Bone Diseases, Metabolic / Q000000981:diagnostic imaging* / Q000453:epidemiology*; D016022:Case-Control Studies; D005260:Female; D005272:Femur Neck / Q000000981:diagnostic imaging; D050723:Fractures, Bone / Q000453:epidemiology; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000000981:diagnostic imaging* / Q000453:epidemiology*; D008875:Middle Aged; D012307:Risk Factors; D014955:Wrist Joint / Q000000981:diagnostic imaging