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Sponastrime dysplasia

A radiologic-pathologic correlation

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Abstract

The 2nd family with Sponastrime Dysplasia is described. The clinical, radiologic and chondro-osseous morphology of boy and girl siblings are presented. The facial appearance is an “oriental look” with midface hypoplasia and a saddle nose. The radiological findings include the spinal changes of lordosis, osteoporosis and pearshaped vertebrae, as well as striated metaphyses (osteopathia striata). The morphological findings suggest a disturbance in the formation of cartilage, with a defect in collagen and proteoglycans synthesis in this rare autosomal recessive skeletal dysplasia.

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This study was supported in part by USPHS NIH program project grant (HD-22657)

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Lachman, R.S., Stoss, H. & Spranger, J. Sponastrime dysplasia. Pediatr Radiol 19, 417–424 (1989). https://doi.org/10.1007/BF02387640

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  • DOI: https://doi.org/10.1007/BF02387640

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