Osteogenesis imperfecta

Definition

  • AKA “Brittle Bone Disease”
  • Heterogeneous group of inheritable diseases
  • Characterized by an abnormality of Type 1 collagen
  • Manifested by bone fragility, deformity, & propensity to fracture
  • Characterised by
    • Bone fragility
    • Deformity
    • Propensity to fracture
  • Ivar the Boneless (9th century)
    • Scandinavian invader of England
    • Carried into battle on his shield
    • William the Conqueror exhumed & burnt his skeleton