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Osteogenesis Imperfecta

 

作者:

 

期刊: The British Journal of Radiology  (WILEY Available online 1943)
卷期: Volume 16, issue 185  

页码: 129-136

 

年代: 1943

 

DOI:10.1259/0007-1285-16-185-129

 

出版商: The British Institute of Radiology

 

数据来源: WILEY

 

摘要:

The characteristic features of this dystrophy are, in the infantile form, the failure for most of the ossification to pass beyond the stage of calcification, and the presence of multiple fractures; in the infant, multiple fractures, flattened vertebral bodies, expanded extremities of the tubular bones with a relative hypoplasia of the shaft, tendency to ballooning of a thin skull. Towards puberty, marked irregularity in growth in the metaphyseal borders of the epiphysis and expanded ends of the diaphyses, suggesting proliferation of cartilage, osteoid or other pre-osseous tissue instead of bone. In the adolescent and adult, localised thinning and bulging of the skull, marked deformities of the ribs and pelvis. Slender shafts of the tubular bones of the lower extremity, composed chiefly of compact cortical tissue with extremities which are expanded to almost normal size, but made up of an open cancellous structure which makes them relatively radio-transparent. Marked bending of the pelvis and bones of the lower extremity indicate an unusual degree of plasticity.

 

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