Calcified Tissue International

, Volume 64, Issue 3, pp 185–190

The Mineralization Density of Iliac Crest Bone from Children with Osteogenesis Imperfecta

Authors

  • A.  Boyde
    • Department of Anatomy and Developmental Biology, University College, London, UK
  • R.  Travers
    • Genetics Unit, Shriners Hospitals for Children, Montreal, Quebec, Canada
  • F. H.  Glorieux
    • Genetics Unit, Shriners Hospitals for Children, Montreal, Quebec, Canada
  • S. J.  Jones
    • Department of Anatomy and Developmental Biology, University College, London, UK

DOI: 10.1007/s002239900600

Cite this article as:
Boyde, A., Travers, R., Glorieux, F. et al. Calcif Tissue Int (1999) 64: 185. doi:10.1007/s002239900600

Abstract.

We studied iliac crest biopsy cores taken from young individuals with osteogenesis imperfecta of several types, and from age-matched normals; the same samples had been used in prior studies using conventional light microscopic histomorphometric procedures. The PMMA blocks were micro-milled to a fine finish, carbon coated, and imaged using backscattered electrons (BSE) in an automated digital scanning electron microscope (SEM). For comparison of BSE signal levels between samples, microscope operation parameters were standardized by reference to halogenated dimethacrylate standards, and recording data from stereological arrays of 512*512 nonoverlapping pixels at 3.5 μm separation. All OI types showed higher average mineralization densities than age- and site-matched normals. This is interpreted as the result of the failure in matrix assembly, such that it has a higher water volume fraction available for mineral deposition. Added to the net deficit in bone quantity, the predicted higher stiffness of the more mineralized bone will account for much of the observed `brittleness' that characterizes this class of genetic disease. The mean mineralization density, which was higher in types III, IV, and V than in type I, appears to be correlated with disease severity.

Key words: Bone density — Osteoporosis — Osteopenia — Osteogenesis imperfecta — Quantitative backscattered electron image analysis.

Copyright information

© 1999 Springer-Verlag New York Inc.