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Pagets disease

Paget’s disease of bone is a condition characterized by abnormal and anarchic resorption and deposition of bone, resulting in distortion and weakening of the affected bones

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Pagets disease

  1. 1. Paget’s Disease of Bone (Osteitis Deformans)
  2. 2. What is Paget’s disease? Paget’s disease of bone is a condition characterized by abnormal and anarchic resorption and deposition of bone, resulting in distortion and weakening of the affected bones
  3. 3. Bone consists of Osteoblasts: Bone forming cells Osteoclasts: Bone breaking cells Osteocytes: Present in the matrix Matrix: Collagen, Calcium, Osteocalcin
  4. 4. Bone remodeling
  5. 5. Bones commonly affected
  6. 6. Clinical & Radiographic Features Pain in or over a bone Joint pain and limited mobility The shape of the bone may change -the bone in the legs may bend or bow out -the bone in the skull may get bigger Maxillary disease is more common than mandibular Lionlike facial deformity (leontiasis ossea) Patchy sclerotic areas of Skull RG (cotton wool) Bone Scan of Mandible may show Codylar to condylar uptake(Black beard or Lincoln’s sign)
  7. 7. Clinical & Radiographic Features Bone breaks more easily In late stages hipjoint may damage Hearing problems may occur because the bone expands and heart problems may also occur COTTON WOOL APPEARANCE
  8. 8. Histopathological Features Uncontrolled alternating resorption and formation of bone Basophilic reversal lines- indicate the junction between alternating resorptive and formative phases result in a jigsaw puzzle, or mosaic appearance of the bone REVERSAL LINES
  9. 9. NORMAL SKULL DISEASED SKULL SKULL SIZE IN PAGETS DISEASE
  10. 10. What causes Paget’s disease Unknown; evidence favors an infection of genetically predisposed individuals with a measles virus of the paramyxovirus family Studies have demonstrated that abnormal virus- infected osteoclasts generate IL-6, a resorptive cytokine from the bone marrow of patients with Paget’s disease
  11. 11. How common is Paget’s disease The exact number of Paget’s disease is not known Men are more likely to be affected than woman  It usually affects people over the age of 40 years It’s estimated to affect 3% of people over the age of 40
  12. 12. Epidemiology Prevalence High: United Kingdom, United States, Australia, New Zealand, Western Europe Low: Scandinavia, China, Japan, India Family History: positive in as many as 40% of patients
  13. 13. Treatment At this time there is no cure for Paget’s disease. Therefore treatment is designed to control the symptoms and change the rate of bone growth. When symptomatic, bone pain is noted most frequently and often may be controlled by acetaminophen or nonsteroidal antiinfl ammatory drugs (NSAIDs).
  14. 14. Treatment Biphosphonates heplfull in rebuilding bone Inhibits bone resorption and mineralisation by promoting apoptosis of osteoclasts for example Didronel, Fosamex and Actonel
  15. 15. Treatment Calcitonin (Parathrmone Antogonist) Slows bone loss and bone growth Gives relieve of bone pain Reduces skin temperature over affected bone sites For example acetaminophen (Tylenol, Panadol)
  16. 16. Treatment Surgery 1. Fractures: to allow fractures to heal in better position 2. Bone deformity: cutting and realigning of the affected bones 3. Severe degenerative arthritis: joint replacement of the hips, knees

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