Osteoporosis and the Marfan syndrome

Gray, Jonathon; Bridges, A. B.; Mole, P. A.; Th, Pringle; Boxer, M.; Paterson, C. R.

doi:10.1136/pgmj.69.811.373

Cited by 30 publications

(12 citation statements)

References 5 publications

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“…Two other studies reported no difference in BMD in MFS patients. Gray et al [10] in 1993 found no difference in BMD at the radius in 14 patients using single photon absorptiometry compared with a control population matched for age and sex but not height. In 1995, Tobias et al [11], using dual-energy X-ray absorptiometry (DXA) compared BMD of the hip and lumbar spine in 14 postmenopausal MFS women versus controls matched for age, sex, weight and height.…”

Section: Discussionmentioning

confidence: 95%

Bone Mineral Density in Sixty Adult Patients with Marfan Syndrome

Parc

Plantin

Jondeau

et al. 1999

Osteoporosis International

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Sixty adult patients (40 women, 20 men) with Marfan syndrome (MFS) according to the Berlin criteria had a full clinical examination and bone mineral density (BMD) measurement by dual-energy X-ray absorptiometry of the hip and nondominant forearm. BMD was expressed as a Z-score and compared with the reference population of the Hologic database. In MFS men, BMD (g/cm(2)) was compared with the BMD of 45 normal tall Caucasian adults. Osteocalcin was measured by radioimmunoassay. In patients with MFS, BMD was compared between patients with and without previous fractures and according to the phenotypic severity of MFS. The mean age of the patients was 32.9 +/- 9.3 years (women 32.5 +/- 9.7, men 33.4 +/- 8.6), mean height was 180.3 +/- 10.3 cm (women 176.3 +/- 9.2, men 188.1 +/- 7.5) and mean body mass index 20.9 +/- 3.6 kg/m(2) (women 20.8 +/- 3.4, men 20.95 +/- 3.97). Hyperlaxity score (Beighton criteria) was 6.9 +/- 1. 1. Six patients (10%) had a previous fracture. Thirty per cent of patients had had at least one previous operation for scoliosis, aortic dilatation or eye problems. BMD values in the 60 patients were as follows: Z-score of the hip, -1.26 +/- 0.93, p<10(-9) (neck, -0.93 +/- 1.09, p<10(-9); trochanter, -1.31 +/- 0.85, p<10(-9); intertrochanter, -1.39 +/- 0.99, p<10(-9); Ward's triangle, -0.93 +/- 1.88, p<10(-9)); Z-score of the radius: -1.6 +/- 1.06, p<10(-9) (1/3 proximal, -1.29 +/- 1.03; mid-radius, -1.94 +/- 1.04; ultradistal, -0.68 +/- 1.1, p<10(-9)). The decrease in BMD was similar in men and women at both the hip and the radius. BMD in MFS patients was significantly decreased at cortical compared with trabecular sites (radius 1/3 proximal vs ultradistal, p<0.0001; total femur vs Ward's triangle, p<0.0005). No difference in BMD was found between MFS patients with or without previous fractures and those with severe or less severe phenotypic expression of MFS. An influence of height and weight in MFS on BMD is suspected. Osteocalcin was not increased in our group of MFS patients. Thus both men and women with MFS have a significant deficit of BMD at the hip and radius. The decrease in BMD is present equally in both sexes and is more pronounced at predominantly cortical sites. In our group of patients we found no increase in fractures and no relation between decreased BMD and phenotypic expression of the syndrome.

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Section: Discussionmentioning

confidence: 95%

Bone Mineral Density in Sixty Adult Patients with Marfan Syndrome

Parc

Plantin

Jondeau

et al. 1999

Osteoporosis International

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“…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.…”

Section: Introductionmentioning

confidence: 99%

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

Giampietro

Peterson

Schneider

et al. 2007

HSS Journal®: The Musculoskeletal Journal of Hospital for Speci

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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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“…The existence of decreased BMD in patients with Marfan syndrome has been a subject of interest during the past two decades [6][7][8][9][10][11][12][13]. Reports have been conflicting and both generalized and localized decreased BMD have been described in the presence of Marfan syndrome.…”

Section: Marfan Syndromementioning

confidence: 99%

Alterations in Bone Mineral Density in Marfan Syndrome and Homocystinuria

Giampietro

Peterson

Raggio

2008

Clinic Rev Bone Miner Metab

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Homocystinuria and Marfan syndrome represent distinct genetic conditions that share phenotypically similar skeletal features. An overview of the current understanding of genetic and physiologic contributing to the etiology of these conditions is summarized. The focus of this review is to explore the present understanding of the pathophysiology of Marfan syndrome and homocystinuria relative to the occurrence of osteoporosis in both conditions. Osteoporosis has been reported in association with homocystinuria. However, evidence supporting an association of osteoporosis with Marfan syndrome is equivocal and sources of ambiguity are critically reviewed. Advisability and approaches to bone mineral density monitoring in patients with Marfan syndrome or homocystinuria to inform clinical management are discussed. Finally, future research foci are proposed which will improve understanding of association of osteoporosis with Marfan syndrome or homocystinuria.

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Osteoporosis and the Marfan syndrome

Cited by 30 publications

References 5 publications

Bone Mineral Density in Sixty Adult Patients with Marfan Syndrome

Bone Mineral Density in Sixty Adult Patients with Marfan Syndrome

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

Alterations in Bone Mineral Density in Marfan Syndrome and Homocystinuria

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