The microscopic structure of bone in normal children and patients with osteogenesis imperfecta: a survey using backscattered electron imaging.
The microstructure of iliac crest biopsies from normal children or from those afflicted with osteogenesis imperfecta (OI) has not previously been studied to determine the tissue histology in the context of the degree of mineralization. The material in this study comprised 112 iliac crest biopsies from children aged 1.9-22.9 years. Fifty-eight were reference biopsies taken from children with no bone disease and the remainder were biopsies from children diagnosed as having OI (23 were Type I, 8 Type III, 18 Type IV, and 5 Type V). The specimens, which had been embedded in polymethylmethacrylate (PMMA), were micromilled and carbon coated to permit backscattered electron imaging. Reference biopsies from very young children often contained densely mineralized cartilage, and evidence of rapid cortical drift. Circumferential lamellae became a prominent feature after the toddler stage, and active remodeling and slower cortical drift continued through childhood. The biopsies from older teenagers and young adults were indistinguishable. Occasional mineralized osteocyte lacunae were detected in even the youngest children. Bone from children with OI Type I often appeared normal in microstructure and amount, but in some there was a dearth of bone and an abundance of osteocytes. Compared with age-matched controls, cortical and trabecular bone from children with OI Types III and IV were markedly sparse and very cellular, and primary osteonal systems continued to be formed later than expected. A distinguishing feature of the bone from OI Type V patients was the failure of patches of bone to mineralize, especially adjoining a reversal line. Packets of bone tissue exhibiting either considerably higher than normal or deficient mineralization would contribute to the characteristic trait of mechanical weakness.
Jones
S J
SJ
Department of Anatomy and Developmental Biology, University College London, London WCIE 6BT, United Kingdom.
Glorieux
F H
FH
Travers
R
R
Boyde
A
A
D000293:Adolescent; D000328:Adult; D001706:Biopsy; D001842:Bone and Bones / Q000473:pathology* / Q000648:ultrastructure*; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D008855:Microscopy, Electron, Scanning; D010013:Osteogenesis Imperfecta / Q000473:pathology*