Clinical management of osteogenesis imperfecta.

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Abstract

[Indexed for MEDLINE] 8. Calcif Tissue Int. 2003 Nov;73(5):441-5. doi: 10.1007/s00223-002-2101-7. Epub 2003 Sep 10. The interaction between Sillence type and BMD in osteogenesis imperfecta. Kok DJ(1), Uiterwaal CS, Van Dongen AJ, Kramer PP, Pruijs HE, Engelbert RH, Verbout AJ, Schweitzer DH, Sakkers RJ. Author information: (1)Department of Orthopedic Surgery, KE 4.140.5, Wilhelmina Children's Hospital, University Medical Center, 3508 AB Utrecht, The Netherlands. Clinical studies with bisphosphonates in children with osteogenesis imperfecta (OI) show an increase in BMD and a decrease in fracture rate. Bone strength in children with OI is not only influenced by changes in BMD but also by changes in collagen I structure of the organic bone matrix. Therefore, we studied the interaction between these two factors in a cross-sectional, single center study including 54 children. We assumed that vertebral deformities in OI represent an unbalance between load and bone strength. Body weight was considered to be a well quantifiable load on vertebral bodies. BMD served as a marker, representing the amount of bone tissue available for vertebral load bearing, and the Sillence classification, either type I or III/IV, as a marker representing the quality of the organic bone matrix. Independent associations were observed between the prevalence of vertebral deformities and (1) Sillence type (OR: 5.7, 95%Cl:1.2-26.8), (2) BMD (OR: 0.003, 95%Cl: 0-0.25) and (3) body weight (OR: 1.15, 95%Cl: 1.05-1.25). Regarding the anthropometrical differences among the different types of OI, the BMD/body weight ratio was introduced to evaluate the BMD in relation to body size. Prevalent vertebral deformities were associated with low BMD/body weight ratios (OR: 0.04, 95%Cl: 0.008-0.2) in OI type I, but no association was found in type III/IV. It was concluded that BMD and Sillence type have independent relationships with vertebral deformities. The BMD/body weight ratio correlates with vertebral deformities in children with OI type I. Its meaning in types III/IV needs further research with larger samples because of the relatively high prevalence of vertebral deformities in this group. DOI: 10.1007/s00223-002-2101-7