paget disease

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PAGET’S DISEASE : 

PAGET’S DISEASE SYEDA TOOBA ZAIDI Department of Oral Pathology and Diagnosis

What is Paget’s Disease : 

What is Paget’s Disease Paget’s disease of bone is a chronic disorder of the skeleton in which areas of bone undergo abnormal turnover , resulting in areas of enlarged and softened bone. or It is a disorder that involves abnormal destruction and regrowth, which results in deformity. The disease named after SIR JAMES PAGET , the British surgeon who first described in 1877. Previously called osteitis deformans now preferred term is PAGET DISEASE.

CAUSES : 

CAUSES The cause of Paget's disease is unknown. The disorder tends to run in families, and recent information suggest a possible contribution of group of genetic defects. Also, some evidence suggests that a virus is involved. Even if a virus is involved, there is no evidence that the disorder is contagious. viral Paget disease may be caused by a slow virus infection (paramyxoviruses) present for many yrs before symptoms appear. Genetic There is also a hereditary factor.

Pathogenesis (cont) : 

Pathogenesis (cont) The pathogenesis of Paget's disease is described in 3 stages which are: i. Osteoclastic or osteolytic stage. ii. Mixed osteoclastic-osteoblastic stage.  iii. Exhaustive (burnt out) stage.

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Normally, cells that break down old bone (osteoclasts) and cells that form new bone (osteoblasts) work in balance to maintain bone structure and integrity. Phase 1: Osteoclastic activity increases results in bone destruction. Phase 2: Both osteoclasts and osteoblasts become overactive in some areas of bone, and the rate at which bone is broken down and rebuilt increases tremendously in involved areas. The overactive areas enlarge but, despite being large, are structurally abnormal and weak. Phase 3: Both osteoclastic and osteoblastic activity ceases the bone is sclerotic , weak and brittle

HISTOPATHOLOGY : 

HISTOPATHOLOGY In this picture the old bone and new bone are deposited and junction of both new and old bone show prominent cement lines , appearing as typicall mosaic pattern pathognomonic of disease , the areas between the bones are fibrous from which bone has been resorbed.

Symptoms : 

Symptoms Many patients do not know they have Paget's disease because they have a mild case with no symptoms. Sometimes, symptoms may be confused with those of arthritis or other disorders. In other cases, the diagnosis is made only after complications have developed. Symptoms can include: Bone pain is the most common symptom. Bone pain can occur in any bone affected by Paget's disease. It often localizes to areas adjacent to the joints. Headaches and hearing loss may occur when Paget's disease affects the skull. Pressure on nerves may occur when Paget's disease affects the skull or spine.

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Increased head size, bowing of limb, or curvature of spine may occur in advanced cases. Hip pain may occur when Paget's disease affects the pelvis or thighbone. Damage to joint cartilage may lead to osteoarthritis. Chalkstick fractures. Hypercementosis in teeth may occur.

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AP radiograph of the hip in a patient with paget disease demonstrates dense sclerosis involving the femoral head and neck. This is a high risk area for insufficiency fracture.

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Lateral radiograph of the calvarium in a patient with paget disease reveals multiple patches of sclerotic bone in the calvarium ( cotton wool appearance) Large osteolytic lesion in the skull of a woman with Paget's disease

Diagnosis : 

Diagnosis Paget's disease may be diagnosed using one or more of the following tests: Paget bone has a characteristic appearance on X-rays. A skeletal survey is therefore indicated. Bone scans are useful in determining the extent and activity of the condition. Bone biopsy. LABORATORY FEATURES Elevated urinary hydroxyproline Elevated levels of alkaline phosphatase Calcium and phosphorus usually normal

TREATMENT / MANAGEMENT : 

TREATMENT / MANAGEMENT Therapy Calcitonin Oral biphosphonates Supportive braces Response to therapy Alkaline phosphatase and urinary hydroxyproline determinations. Orthopedic surgery may be need to correct a deformity in severe cases.

Oral & Facial findings : 

Oral & Facial findings

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