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Thumbnail imageThumbnail imageMucopolysaccharidosis

Hurler’s

Oar shaped ribs, bullet shaped metacarpals/phalanges of hand, inferior/anterior beaking VB

Distinguish from Morquio’s – central/anterior beaking, not MR, atlantoaxial instability

  • AKA – dysostosis multiplex
  • Heredetary deficiency of lysomal enzymes required for the degardation of mucopolysaccharides or glycosaminoglycans. Results in accumulation in RES, connective tissue, skin, and nervous system
  • Mentally retarded (not Morquio’s) dwarfs with short trunks.
  • 7 types, all AR except Hunter’s (II, x-linked)
  • General Radiographic Features – beaked VB, dwarfism, bullet shaped metacarpals with tapering of digits, short trunk dwarfism from playspondyly, atlantoaxial subluxation/instability, HSM
  • Know Hurler’s and Morquio’s

Hurler’s – gargoyle face, j-shaped sella, inferior beaking VB, oar shaped ribs(central narrowing with peripheral widening)
Morquio’s – short wide ribs, central VB beaking

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