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Clinical Trial
. 1999 Oct;88(10):1083-8.
doi: 10.1080/08035259950168135.

Bone mineral content and collagen defects in osteogenesis imperfecta

A M Lund  1 , C Mølgaard, J Müller, F Skovby
Affiliations
Clinical Trial

Bone mineral content and collagen defects in osteogenesis imperfecta

A M Lund et al. Acta Paediatr. 1999 Oct.

Abstract

Whole-body and spine dual-energy X-ray absorptiometry was done in 63 patients with osteogenesis imperfecta aged 5 to 63 y, and the results were compared with OI types and collagen defects. Bone mineral content (BMC)-for-age, bone area (BA)-for-age, bone mineral density (BMD)-for-age, and BMC-for-BA were reduced, especially in patients with OI III/IV and/or in those with a qualitative collagen defect. BA-for-height was normal. Some patients with OI I and/or a quantitative collagen defect had BMD at or above -2 z-scores. We conclude (i) that both BMC and BMD differ significantly between OI types and collagen defects, (ii) that reduced BMC-for-age in OI patients is due mainly to reduced height ("short bones") and reduced BMC-for-BA ("light bones"), whereas BA-for-height ("bone width") is normal, (iii) that most OI patients have lower than average BMC, but in some mildly affected patients brittleness may exist with only small reductions in BMC.

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