Advertisement for orthosearch.org.uk
You currently have no access to view or download this content. Please log in with your institutional or personal account if you should have access to through either of these
The Bone & Joint Journal Logo

Receive monthly Table of Contents alerts from The Bone & Joint Journal

Comprehensive article alerts can be set up and managed through your account settings

View my account settings

A NEW LOOK AT OSTEOGENESIS IMPERFECTA



Download PDF

Abstract

In a clinical, radiological and biochemical study of forty-two patients from Oxford with osteogenesis imperfecta, it was found that patients could be divided simply into mild, moderate and severe groups according to deformity of long bones. In the severe group (seventeen patients) a family history of affected members was uncommon and fractures began earlier and were more frequent than in the mild group (twenty-two patients); sixteen patients in the severe group had scoliosis and eleven had white sclerae; no patients in the mild group had white sclerae or scoliosis. Radiological examination of the femur showed only minor modelling defects in patients in the mild group, whereas in the severe group five distinct appearances of bone (thin, thick, cystic and buttressed bones, and those with hyperplastic callus) were seen. The polymeric (structural) collagen from skin was unstable to depolymerisation in patients in the severe group, but normal in amount, whereas the reverse was found in the mild group. This division according to long bone deformity may provide a basis for future research more useful than previous classifications.

For access options please click here