Abnormal mineral composition of osteogenesis imperfecta bone as determined by electron probe X-ray microanalysis on conventional and cryosections

Osteogenesis imperfecta (OI) is a genetic disorder of the connective tissue characterized by frequent bone fractures. The cause of bone fragility is still unknown even though substantial work on collagen has been done. We measured the calcium to phosphorus ratio (Ca/P) of bone mineral from 35 OI bon...

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Bibliographic Details
Published in:Calcified tissue international 1999-07, Vol.65 (1), p.11-15
Main Authors: Sarathchandra, P, Kayser, M V, Ali, S Y
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
Bone Density
Calcium - analysis
Child
Child, Preschool
Cryoelectron Microscopy - methods
Electron Probe Microanalysis - methods
Femur - diagnostic imaging
Femur - embryology
Femur - metabolism
Fetal Diseases - diagnostic imaging
Fetal Diseases - metabolism
Fetal Diseases - pathology
Fetus - diagnostic imaging
Fetus - metabolism
Fetus - pathology
Gestational Age
Humans
Microscopy, Electron
Middle Aged
Osteogenesis Imperfecta - metabolism
Osteogenesis Imperfecta - pathology
Phosphorus - analysis
Radiography
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Summary:Osteogenesis imperfecta (OI) is a genetic disorder of the connective tissue characterized by frequent bone fractures. The cause of bone fragility is still unknown even though substantial work on collagen has been done. We measured the calcium to phosphorus ratio (Ca/P) of bone mineral from 35 OI bone samples and 25 age- and site-matched control specimens, using electron probe X-ray microanalysis in the transmission electron microscope. Ultra-thin cryosections and conventionally prepared resin sections were used. Cryo-ultramicrotomy avoids any possible artifactual demineralization that may occur in conventional aqueous media. The Ca/P ratio obtained by these two methods was compared and there was no statistical difference between them. The results were differentiated according to the clinical types of OI for the first time. The Ca/P ratio of OI bone mineral was lower than normal in both resin 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-matched controls (Ca/P = 1.69). This abnormal mineral composition in OI type II could be a contributory factor to bone fragility in OI bone.
ISSN:0171-967X
1432-0827
DOI:10.1007/PL00005829