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. 1977 Dec 9;90(2):223-31.
doi: 10.1007/BF00414994.

[Pathophysiology and metabolism in osteogenesis imperfecta (author's transl)]

[Article in German]

[Pathophysiology and metabolism in osteogenesis imperfecta (author's transl)]

[Article in German]
K J Münzenberg. Arch Orthop Unfallchir. .

Abstract

The tissue changes in osteogenesis imperfecta apparently lie in the collagen and in the glycosaminoglycans. The collagen shows structural development disturbance and incomplete calcification. The tissue glycosaminoglycans are increased. The basis probably lies in a disturbed ATP metabolism (Solomons and Millar, 1973). An increased pyrophosphate concentration explains the decreased calcification and the structural changes. The increased osteolysis could be explained by increased quantities of cAMP.

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