CENTRIFUGAL OSTEOPETROSIS - APPENDICULAR SCLEROSIS WITH RELATIVE SPARING OF THE VERTEBRAE

A previously unreported pattern of bone involvement in autosomal domin ant osteopetrosis is described in three affected members of a Vietname se kindred. The hands, feet, distal bones, and skull were markedly ost eosclerotic on radiologic examination. The vertebrae displayed very mi nimal thickening of the endplates in two of the cases, and no visible involvement in the third. The vertebral bone density (assessed by dual -energy X-ray absorptiometry) was markedly elevated, suggesting that t he spine was not completely spared. This pattern contrasts with the pr edominantly axial distribution of the recognized variants of osteopetr osis.