Q:

The earliest radiographic sign in Hurler syndrome is

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The earliest radiographic sign in Hurler syndrome is 


  1. thick ribs
  2. thickened calvarium
  3. enlarged J-shaped sella
  4. abnormal spacing of teeth
  5. premature closure of lambdoid suture

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(A). Hurler syndrome; Radiographs show a characteristic skeletal dysplasia known as dysostosis multiplex. The earliest radiographic signs are thick ribs and ovoid vertebral bodies. Skeletal abnormalities include enlarged, coarsely trabeculated diaphyses of the long bones with irregular metaphyses and epiphyses. With progression of the disease macrocephaly develops with thickened calvarium, premature closure of lambdoid and sagittal sutures, shallow orbits, enlarged J-shaped sella, and abnormal spacing of teeth with dentigerous cyst

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