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Paget's Disease - Coggle Diagram
Paget's Disease
Clinical Presentation
Joint pain- when an involved bone is close to a joint - leading to cartilage damage and osteoarthritis
Deformities, in particular bowed tibia and skull changes
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Neurological complications
- Nerve compression - deafness (CN8) & paraparesis - partial paralysis of lower limbs
- Hydrocephalus due to blockage of the aqueduct of Sylvius
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Common sites include; pelvis, lumbar spine, femur, thoracic spine, skull and tibia - although any bone can be involved but small bones are rare
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Diagnosis
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X-ray - localised bony enlargement and distortion, sclerotic changes (increased density), osteolytic areas (loss of bone and reduced density)
Increased serum alkaline phosphatase with normal calcium and phosphate - reflects increased bone turnover
Isotope bone scans - useful to distinguish the extent of skeletal involvement but are unable to distinguish between Paget's and sclerotic metastatic carcinoma
Risk Factors & Aetiology
May result from latent viral infection (canine distemper virus, measles or respiratory syncytial virus) is osteoclasts in genetically susceptible individuals
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Pathophysiology
Ultimately, formation exceeds resorption but the new woven bone is WEAKER than normal bone - this leads to deformity and increased fracture risk
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There is increased osteoclastic bone resorption followed by formation of WEAKER NEW BONE, increased local bone blood flow and fibrous tissue
Treatment
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Disease activity monitored by symptoms and measurement of serum alkaline phosphatase or urinary hydroxyproline
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