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Biochemical Analysis of a Hyperplastic Callus in Osteogenesis Imperfecta Type IV

  • R. E. Brenner
  • U. Vetter
  • O. Wörsdorfer
  • A. Nerlich
  • W. M. Teller
  • P. K. Müller
Conference paper

Abstract

Osteogenesis imperfecta (OI) is an inherited disease of connective tissue, which is characterized by a high fracture rate and skeletal deformations. In addition other tissues containing high amounts of type I collagen like the skin, sklerae and teeth can be affected. According to clinical features it has been divided into 4 different types (1). Various metabolic and structural abnormalities of type I collagen have been reported underlining the clinical heterogeneity. Fibroblasts of some patients produce overhydroxylated and overglycosylated type I collagen and in sporadic cases mutations of the α1 (I)- or α2 (I)-genes have been described (2).

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Reference

  1. (1).
    Sillence, D.O., Senn, A., Danks, D.M. (1979) Genetic heterogeneity in osteogenesis imperfecta, J. Med. Gen. 16:101–116CrossRefGoogle Scholar
  2. (2).
    Prockop, D., Kuivaniemi, H. (1986) Inborn errors of collagen, Rheumatology vol. 10:246–271, Karger, BaselGoogle Scholar
  3. (3).
    Smith, R., Francis, M.J.O., Houghton, G.R. (1983): The brittle bone Syndrome–Osteogenesis imperfecta, Butterworths: 30–33Google Scholar
  4. (4).
    Fairbank, H.A.T., Baker, S.L. (1948): Hyperplastic callus formation with or without evidence of a fracture in osteogenesis imperfecta with an account of the histology, British J. Surgery. Vol. 36, No 141: 1–16Google Scholar
  5. (5).
    Krepler, R., Zhuber, K. (1974): Hyperplastische Kallusbildung bei Osteogenesis imperfecta, Z. Orthop. 112:306–313Google Scholar

Copyright information

© Springer-Verlag · Berlin Heidelberg 1988

Authors and Affiliations

  • R. E. Brenner
    • 1
  • U. Vetter
    • 2
  • O. Wörsdorfer
    • 3
  • A. Nerlich
    • 1
  • W. M. Teller
    • 2
  • P. K. Müller
    • 1
  1. 1.Abteilung für BindegewebsforschungMax-Planck-Institut für BiochemieMartinsriedGermany
  2. 2.Abteilung für KinderheilkundeUniversität UlmUlmGermany
  3. 3.Abteilung für UnfallchirurgieUniversität UlmUlmGermany

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