Centrifugal osteopetrosis: appendicular sclerosis with relative sparing of the vertebrae
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A previously unreported pattern of bone involvement in autosomal dominant osteopetrosis is described in three affected members of a Vietnamese kindred. The hands, feet, distal bones, and skull were markedly osteosclerotic on radiologic examination. The vertebrae displayed very minimal thickening of the endplates in two of the cases, and no visible involvement in the third. The vertebral bone density (assessed by dual-energyX-ray absorptiometry) was markedly elevated, suggesting that the spine was not completely spared. This pattern contrasts with the predominantly axial distribution of the recognized variants of osteopetrosis.
Key wordsOsteopetrosis Bone mineral density Bone dysplasia Absorptiometry, dual energyX-ray Osteosclerosis
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- 1.Aurbach GD, Marx SJ, Spiegel AM. Metabolic bone disease. In: Wilson JD, Foster DW, eds, Williams textbook of endocrinology. 8th edn. Philadelphia: Saunders, 1992; 1477–1517.Google Scholar
- 4.Whyte MP. Hereditary metabolic and dysplastic skeletal disorders. In: Coe FL, Favus MJ, eds. Disorders of bone and mineral metabolism. New York: Raven; 1992: 977–1026.Google Scholar
- 5.Whyte MP. Sclerosing bone dysplasias. In: Favus MJ, ed. Primer on the metabolic bone diseases and disorders of mineral metabolism. 2nd edn. New York: Raven; 1993: 327–344.Google Scholar