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Paget disease

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Paget disease

  1. 1. PATHOLOGY.. Paget’s Disease Maryam Sharifian MSc student In Isfahan University Of medical Sciences
  2. 2. INTRODUCTION      Paget’s disease is a chronic condition of bone characterized by disorder of the normal bone remodeling process. Characterized by excessive breakdown of bone tissue followed by abnormal bone formation. Also known as Osteitis Deformans is a bone disease unknown cause. Affecting men twice as freaquently as women. Named after the England Surgeons Sir James Paget.
  3. 3. DISEASE CLASSIFICATION INFLAMMATORY DISEASE •Multifocal chronic skeletal disease due to Chronic paramyxoviral Virus infection. •As a result, the bone that is formed is abnormal. CONGENTIAL •Certain of genes may Been associated with Paget’s disease, including The Sequestrosome 1 Gene On Chromosome 5.
  4. 4. ETIOLOGY The cause of Paget’s disease is unknown Disease may be caused by a virus: Paramyxo virus Resulting in localized abnormal osteoclastic activity typical of paget’s disease. Also, a person’s genes may predispose them to developing paget’s disease.
  5. 5. Paget’s disease affects skeletal older bone of adults There is also an extremely rare form of paget’s disease in children, referred to as Juvenile Paget’s disease In two of every three cases of Paget's disease, more than one bone is affected
  6. 6. Pathogenesis Hyper vascular/ Osteolytic phase Abnormal matrix Persists but cellular Activity is nearly Absent. Exhaustive (burn out) stage Initial phase of Disorder involves Bone resorption by osteoclasts Paget’s disease Intermediate phase Osteolytic activity + Osteoblastic activity
  7. 7. MANIFESTATIONS - SYMPTOMS Paget’s disease is more commonly asymptomatic, but may exhibit a variety of non-specific symptoms due to increased bone turnover. The bones that are move commonly affected include the pelvis, spine, skull, and the long bone. Individuals may experience any or a combination of the following symptoms; Bone pain fractures
  8. 8. symptoms … Pain Stiffness Difficulty moving your affected joints Nerve damage Loss of balance bone fractures
  9. 9. Other symptoms … Hearing loss Headaches Osteoarthritis visual abnormalities Cardiac abnormalities Skeletal Deformities
  10. 10. SIGNIFICANT LAB TESTS Blood chemistry (blood patient who have paget’s disease) results indicate very high alkaline phosphates levels with normal serum calcium and phosphorus. There is no known cure for this disease. Most cases are mild and asymptomatic no treatment is necessary in symptomatic cases medications.
  11. 11. IMAGING CONSIDERATION X-ray of the skull, spine, pelvis and long bone. CT scan MRI (Magnetic Resonance Imaging) Radionuclide bone scan or RNI (Radionuclide Imaging).
  12. 12. RADIOLOGICAL APPEARANCE Radionuclide bone scans readily detect Paget’s disease even in its very early stage. Radio graphically of the affected bone typically demonstrate cortical thickening, with a coarse, thickened trabecular pattern.
  13. 13. Paget’s disease…. Radiograph of the fibula and tibia of the patient in figure demonstrating the effect of advanced proliferative Paget’s disease on the fibula and tibia.
  14. 14. Paget’s disease on pelvis… Paget’s disease involving the left hemi pelvis and right, there is severe osteoarthritis of the left hip but a relatively normal joint space in the right hip.
  15. 15. Paget’s disease….
  16. 16. Treatment … Not everyone with Paget's disease will require treatment. If treatment is needed, medication is most often used.
  17. 17. Maryam sharifian
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