All are the features of Paget's disease except-
*Paget's disease is slightly more common in males and is seen after 40years of age. Paget&;s Disease (Osteitis Deformans) # Excess of bone destruction & =organized bone formation and repair. The 2 nd most common bone disorder in the U.S. # The etiology is unknown # Usually affects the axial skeleton, veebrae and skull, although the pelvis, tibia, femur are the other common sites of disease. # Most persons are asymptomatic & diagnosis is incidental. # Vascularity is increased in affected poions of the skeleton. Lesions may occur in one or more bones, does not spread from bone to bone. # Deformities & bony enlargement often occur. Bowing of the limbs & spinal curvature in persons with advanced disease. # Bone pain- is the most common symptom. Is is usually worse with ambulation or activity but may also occur at rest. Involved bones may feel spongy & warm because of increased vascularity . # Skull pain is usually accompanied with headache, warmth, tenderness & enlargement of the head. # Radiograph reveals radiolucent areas in the bone, typical of increased bone resorption. Deformities & fractures may also be present. Goals of the treatment- to relieve pain & prevent fracture & deformities. Pharmacologic agents are used to suppress osteoclastic activity. Bisphosphonates & calcitonin are effective agents to decrease bone pain & bone warmth & also relieve neural decompression, joint pain & lytic lesions. # Use of analgesics & NSAIDs. Assistive devices, including cane, walker . Ref: Turek 6th/e p.274-275
Defect in osteoclasts
Common in female
Can cause deafness
Can cause osteosarcoma
Metabolic and endocrine disorders