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  • Title: Sponastrime dysplasia. A radiologic-pathologic correlation.
    Author: Lachman RS, Stoss H, Spranger J.
    Journal: Pediatr Radiol; 1989; 19(6-7):417-24. PubMed ID: 2771481.
    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 pear-shaped 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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