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INTRODUCTIONPaget’s disease is a chronic condition of bonecharacterized by disorder of the normal boneremodeling process.Characterized by excessive breakdown of bonetissue followed by abnormal bone formation.Also known as Osteitis Deformans is a bonedisease unknown cause.Affecting men twice as freaquently as women.Named after the England Surgeons Sir JamesPaget.
DISEASE CLASSIFICATION INFLAMMATORY CONGENTIAL DISEASE•Multifocal chronic •Certain of genes mayskeletal disease due to Been associated withChronic paramyxoviral Paget’s disease, includingVirus infection. The Sequestrosome 1•As a result, the bone that Gene On Chromosome 5.is formed is abnormal.
ETIOLOGYThe cause of Paget’s disease is unknown.Disease may be caused by a virus.Paramyxovirus infection of osteoblasts mayactivate of the c-fos proto-oncogene,resulting in localized abnormal osteoclasticactivity typical of paget’s disease.Also, a person’s genes may predispose themto developing paget’s disease.
Paget’s disease affects older skeletal bone of adults. There is also an extremely rare form of paget’s disease in children, referred to as Juvenile Paget’s disease.
Pathogenesis Hyper vascular/ Initial phase of Osteolytic phase Disorder involves Bone resorption by Abnormal matrix osteoclastsPersists but cellular Activity is nearly Absent. Intermediate Paget’s disease phase Exhaustive Osteoclytic activity (burn out) + stage Osteoblastic activity
MANIFESTATIONS - SYMPTOMSPaget’s disease is more commonly asymptomatic,but may exhibit a variety of non-specificsymptoms due to increased bone turnover.The bones that are move commonly affectedinclude the pelvis, spine, skull, and the long bone.Individuals may experience any or a combinationof the following symptoms; Bone pain fractures
Skeletal Deformities – bowed legs, Fronto-occipital skull enlargement.Warm sensation – due to increased bloodsupply to abnormal bones.Nerve damage – hearing loss, visualabnormalities.Cardiac abnormalities – shortness of breath.
MANIFESTATIONS - SIGNSigns may be bitemporal skullenlargement with frontal “bossing”,diluted scalp veins, nerve deafness in oneor both ears.Also angioid streaks in the fundus of theeyes and a short kyphotic trunk.
SIGNIFICANT LAB TESTSBlood chemistry (blood patient who havepaget’s disease) results indicate very highalkaline phosphates levels with normalserum calcium and phosphorus.There is no known cure for this disease.Most cases are mild and asymptomatic notreatment is necessary in symptomaticcases medications.
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).
RADIOLOGICAL APPEARANCERadionuclide bone scans readily detectPaget’s disease even in its very early stage.Radiographically the affected bonetypically demonstrate cortical thickening,with a coarse, thickened trabecular pattern.Mixed areas of radiolucent Osteolysis andradiopaque Osteosclerosis may be seen.
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.
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.