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Journal of Bone and Joint Surgery, 1973;55:506-515.
© 1973 by The Journal of Bone and Joint Surgery, Inc

Osteogenesis Imperfecta

A HISTOPATHOLOGICAL CASE REPORT

JAMES W. MILGRAM M.D.1, MICHAEL R. FLICK M.D.1, and CHARLES A. ENGH M.D.1

1 From the Department of Pathology, The Johns Hopkins Hospital, Baltimore

A sixty-nine-year old woman exhibited during her lifetime many of the characteristics of osteogenesis imperfecta, including multiple fractures with dwarfism and deformity, hearing failure, and blue sclerae. Otosclerosis was related to fixation of the stapedial foot plates in the inner ears. The osseous structure consisted of mature lamellar bone of deficient quantity. The authors suggest a defect in matrix production by the osteoblasts as the basic defect.


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