P. Sarathchandra et al., Abnormal mineral composition of osteogenesis imperfects bone as determinedby electron probe X-ray microanalysis on conventional and cryosections, CALCIF TIS, 65(1), 1999, pp. 11-15
Osteogenesis imperfecta (OI) is a genetic disorder of the connective tissue
characterized by frequent bone fractures. The cause of bone fragility is s
till unknown even though substantial work on collagen has been done. We mea
sured the calcium to phosphorus ratio (Ca/P) of bone mineral from 35 OI bon
e samples and 25 age- and site-matched control specimens, using electron pr
obe X-ray microanalysis in the transmission electron microscope. Ultrathin
cryosections and conventionally prepared resin sections were used. Cryo-ult
ramicrotomy avoids any possible artifactual demineralization that may occur
in conventional aqueous media. The Ca/P ratio obtained by these two method
s was compared and there was no statistical difference between them. The re
sults were differentiated according to the clinical types of OI for the fir
st time. The Ca/P ratio of OI bone mineral was lower than normal in both re
sin and cryosections, and mirrored the severity of the disease. OI type II
had the lowest ratio (Ca/P = 1.49) compared with normal age- and site-match
ed controls (Ca/P 1.69). This abnormal mineral composition in OI type II co
uld be a contributory factor to bone fragility in OI bone.