Sponastrime dysplasia. A radiologic-pathologic correlation.

R S Lachman; H Stoss; J Spranger

doi:10.1007/bf02387640

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Sponastrime dysplasia. A radiologic-pathologic correlation.

R S Lachman·H Stoss·J Spranger

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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 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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Bone Diseases, Developmental Bone and Bones Child, Preschool Female Humans Male Microscopy, Electron Radiography Syndrome Case Reports Comparative Study Journal Article Research Support, U.S. Gov't, P.H.S.

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R S Lachman, H Stoss, J Spranger. 1989. Sponastrime dysplasia. A radiologic-pathologic correlation.. https://doi.org/10.1007/bf02387640

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