American Journal of Neuroradiology, Vol 7, Issue 3 505-511, Copyright © 1986 by American Society of Neuroradiology
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Cranial CT findings in sclerosteosis
SC Hill, SA Stein, A Dwyer, J Altman, R Dorwart and J Doppman
Sclerosteosis or Van Buchem's disease is a rare genetic craniotubular hyperostosis that becomes evident in early childhood and is associated with progressive involvement of the skull. The pathologic changes in the cranium noted on CT are described in three cases. Although the disease is incurable, CT is useful to display the morbid anatomy of the cranium before palliative surgery.
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  P. t. Dijke, C. Krause, D. J. J. de Gorter, C. W.G.M. Lowik, and R. L. van Bezooijen Osteocyte-Derived Sclerostin Inhibits Bone Formation: Its Role in Bone Morphogenetic Protein and Wnt Signaling J. Bone Joint Surg. Am., February 1, 2008; 90(Supplement_1): 31 - 35. [Abstract] [Full Text] [PDF]
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 R. L. van Bezooijen, S. E. Papapoulos, N. A. Hamdy, P. ten Dijke, and C. W. Lowik Control of Bone Formation by Osteocytes? Lessons from the Rare Skeletal Disorders Sclerosteosis and van Buchem Disease IBMS BoneKEy, December 1, 2005; 2(12): 33 - 38. [Full Text] [PDF]
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 J. C. Gardner, R. L. van Bezooijen, B. Mervis, N. A. T. Hamdy, C. W. G. M. Lowik, H. Hamersma, P. Beighton, and S. E. Papapoulos Bone Mineral Density in Sclerosteosis; Affected Individuals and Gene Carriers J. Clin. Endocrinol. Metab., December 1, 2005; 90(12): 6392 - 6395. [Abstract] [Full Text] [PDF]
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 R. L. van Bezooijen, B. A.J. Roelen, A. Visser, L. van der Wee-Pals, E. de Wilt, M. Karperien, H. Hamersma, S. E. Papapoulos, P. ten Dijke, and C. W.G.M. Lowik Sclerostin Is an Osteocyte-expressed Negative Regulator of Bone Formation, But Not a Classical BMP Antagonist J. Exp. Med., March 15, 2004; 199(6): 805 - 814. [Abstract] [Full Text] [PDF]
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