Abstract
Reduced bone mineral density (BMD) was sporadically reported in patients with Marfan syndrome. This may or may not place the Marfan patient at increased risk for bone fracture. In comparing the BMDs of our patients with those reported in the literature, it seemed that agreement between values, and hence the degree of osteoporosis or osteopenia reported, was dependent on the instrumentation used. The objective of this study was to statistically assess this impression. Bone mineral density measurements from our previously published study of 30 adults with Marfan syndrome performed on a Lunar DPXL machine were compared with studies published between 1993–2000 measured using either Lunar or Hologic bone densitometry instruments. The differences of our measurements compared with those made on other Lunar machines were not statistically significant, but did differ significantly with published results from Hologic machines (P < 0.001). Before progress can be made in the assessment of BMD and fracture risk in Marfan patients and in the evidence-based orthopedic management of these patients, standardization of instrumental bone density determinations will be required along with considerations of height, obesity, age, and sex.
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References
De Paepe A, Devereux RB, Dietz HC et al (1996) Revised diagnostic criteria for the Marfan syndrome. Am J Med Genet 62:417–426
Pyeritz RE, McKusick VA (1979) The Marfan syndrome: diagnosis and management. N Engl J Med 300:772–777
Miller DC (2003) Valve-sparing aortic root replacement in patients with the Marfan syndrome. J Thorac Cardiovasc Surg 125:773–778
Peters KF, Kong F, Hanslo M et al (2002) Living with Marfan syndrome III. Quality of life and reproductive planning. Clin Genet 62:110–120
Gray JR, Bridges AB, Mole PA et al (1993) Osteoporosis and the Marfan syndrome. Postgrad Med J 69:373–375
Kohlmeier L, Gasner C, Marcus R (1993) Bone mineral status of women with Marfan syndrome. Am J Med 95:568–572
Tobias JH, Dalzell N, Child AH (1995) Assessment of bone mineral density in women with Marfan syndrome. Br J Rheumatol 34:516–519
Kohlmeier L, Gasner C, Bachrach LK et al (1995) The bone mineral status of patients with Marfan syndrome. J Bone Miner Res 10:1550–1555
LeParc JM, Plantin P, Jondeau G et al (1999) Bone mineral density in sixty adult patients with Marfan syndrome. Osteoporos Int 10:475–479
Carter N, Duncan E, Wordsworth P (2000) Bone mineral density in adults with Marfan syndrome. Rheumatology (Oxford) 39:307–309
Giampietro PF, Peterson M, Schneider R et al (2003) Assessment of bone mineral density in adults and children with Marfan syndrome. Osteoporos Int 14:559–563
Woo SB, Hellstein JW, Kalmar JR (2006) Narrative (corrected) review: bisphosphonates and osteonecrosis of the jaws. Ann Intern Med 144:753–761
Cawte SA, Pearson D, Green DJ et al (1999) Cross-calibration, precision and patient dose measurements in preparation for clinical trials using dual energy x-ray absorptiometry of the lumbar spine. Br J Radiol 72:354–362
Lu Y, Mathur AK, Blunt BA et al (1996) Dual x-ray absorptiometry quality control: comparison of visual examination and process-control charts. J Bone Miner Res 11:626–637
Faulkner KG, Roberts LA, McClung MR (1996) Discrepancies in normative data between Lunar and Hologic DXA systems. Osteoporos Int 6:432–436
Lu Y, Fuerst T, Hui S et al (2001) Standardization of bone mineral density at femoral neck, trochanter and Ward’s triangle. Osteoporos Int 12:438–444
Ingle BM, Sherwood KE, Eastell R (2001) Comparison of two methods for measuring ultrasound properties of the heel in postmenopausal women. Osteoporos Int 12:500–505
Pearson D, Cawte SA, Green DJ (2002) A comparison of phantoms for cross-calibration of lumbar spine DXA. Osteoporos Int 13:948–954
Gjesdal CG, Aanderud SJ, Haga HJ et al (2004) Femoral and whole-body bone mineral density in middle-aged and older Norwegian men and women: suitability of the reference values. Osteoporos Int 15:525–534
Tothill P, Laskey MA, Orphanidou CI et al (1999) Anomalies in dual energy x-ray absorptiometry measurements of total-body bone mineral during weight change using Lunar, Hologic and Norland instruments. Br J Radiol 72:661–669
Gilsanz V (1998) Bone density in children: a review of the available techniques and indications. Eur J Radiol 26:177–182
Kharrazi FD, Rodgers WB, Coran DL et al (1997) Protrusio acetabuli and bilateral basicervical femoral neck fractures in a patient with Marfan syndrome. Am J Orthop 26:689–691
Peters KF, Horne R, Kong F et al (2001) Living with Marfan syndrome. II. Medication adherence and physical activity modification. Clin Genet 60:283–292
Kerlikowske K, Grady D, Barclay J et al (1998) Variability and accuracy in mammographic interpretation using the American College of Radiology Breast Imaging Reporting and Data System. J Natl Cancer Inst 90:1801–1809
Pisano ED, Gatsonis CA, Yaffe MJ (2005) American College of Radiology Imaging Network digital mammographic imaging trial: objectives and methodology. Radiology 236:404–412
Acknowledgments
We acknowledge the assistance of Elizabeth Wood with construction of the database. We also thank Marshfield Clinic Research Foundation for its support through the assistance of Linda Weis, Alice Stargardt, Ingrid Glurich and Jennifer Hayes in the preparation of this manuscript.
This study was supported in part by Children’s Clinical Research Center of Weill Medical College of Cornell University (GCRCMO1RR06020), Adult Clinical Research Center of Weill Medical College (M01RR00047), and the John R. Cobb Scoliosis Research Fund.
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Giampietro, P.F., Peterson, M.G.E., Schneider, R. et al. Bone Mineral Density Determinations by Dual-Energy x-ray Absorptiometry in the Management of Patients with Marfan Syndrome—Some Factors Which Affect the Measurement. HSS Jrnl 3, 89–92 (2007). https://doi.org/10.1007/s11420-006-9030-3
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DOI: https://doi.org/10.1007/s11420-006-9030-3