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Paget disease of bone

Paget disease of bone is a condition characterised by focal regions of accelerated bone turnover due to increased number and size of osteoclasts resulting in disorganised bone structure. It affects mainly the axial skeleton.

Risk factors for Pagets:
  • Increasing age - it is rare in under 40s
  • Anglo-saxon origin
  • Positive family history
  • Males slightly more affected than females

Features of Paget disease of bone include:
  • Bone pain
  • Bone deformities – bowing of a limb, bossing of the skull
  • Fractures
  • Arthritis
  • Hearing loss
  • Tinnitus
  • Headache
  • Cardiac failure
However, it is often asymptomatic

Osteosarcoma develops in less than 1% of patients

Bloods show raised ALP and a normal calcium and phosphate
A bone scan should be performed.

Treatment
  • No cure
  • Bisphosphonates (which block osteoclast formation and induce osteoclast apoptosis) can induce remission
  • Calcintonin (which blocks osteoclast formation and action) can also induce remission

References:
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Abdelghafour

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Secret collector of interesting anonymised ECGs. Fan of the Bath Photomarathon. Lover of cream teas. [Sarah Hudson] (Your Picture)