Bone mass, bone markers and prevalence of fractures in adults with osteogenesis imperfecta

被引：42
作者：
Wekre L.L. [1 ]
Eriksen E.F. [2 ]
Falch J.A. [3 ]
机构：
[1] TRS National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital, University of Oslo
[2] Department of Endocrinology, Faculty of Medicine, University of Oslo, Oslo
[3] Department of Chronic Diseases, Norwegian Institute of Public Health, Oslo
来源：
Archives of Osteoporosis | 2011年 / 6卷 / 1-2期
关键词：
Adult; Bone marker; Bone mineral density; Osteogenesis imperfecta; Osteoporosis;
D O I：
10.1007/s11657-011-0054-z
中图分类号：
学科分类号：
摘要：
Summary Still little is known about the manifestations of osteogenesis imperfecta (OI) in adults. We therefore initiated this study of bone mass, bone turnover and prevalence of fractures in a large cohort of adult patients. We found a surprising low prevalence (10%) of osteoporosis. These patients, however, expressed the most severe disease. Purpose To characterize bone mineral density, bone turnover, calcium metabolism and prevalence of fractures in a large cohort of adults with osteogenesis imperfecta. Methods One hundred fifty-four patients with adult OI participated and 90 (age range 25-83) provided dual X-ray absorptiometry (DXA) measurements. According to Sillence classification criteria, 68 persons were classified as OI type I, 9 as type III, 11 type IV and 2 were unclassified. Fracture numbers were based on self-reporting. Biochemical markers of bone turnover were measured and bone mineral density (BMD) of the spine, femoral neck and total body were determined by DXA. Results Only 10% of adults with OI exhibited osteoporotic T scores (T≤-2.5) but compared to patients with normal T scores this subgroup had a threefold higher fracture risk (22 vs. 69). s-PTH, s-Ca and 25[OH] vitamin D were all normal. Bone markers did not display major deviations from normal, but patients with OI type III displayed higher resorption marker levels than type I and IV. Multivariate regression analysis showed that only gender and total body BMD were significant determinants of fracture susceptibility, and the differences for total body BMC, BMD and Z scores were significant between the OI subtypes. Conclusions In adult OI, DXA measurements only identified few patients as osteoporotic. These patients, however, exhibited a much higher fracture propensity. Due to deformities, low body height and pre-existing fractures, DXA assessment is complicated in this disease, and further studies are needed to work out how to minimize the impact of these confounders. © International Osteoporosis Foundation and National Osteoporosis Foundation 2011.
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页码：31 / 38
页数：7
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