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MyPGMEE Authors: Dr. Mudit Khanna, Dr. Sushant Bhanja, Dr. Punit Bhojani and Dr. Ashwin Singh Parihar.
HYT – PAGET’S DISEASE
1. Increased bone turnover (Therefore elevated serum ALP and urine Hydroxyproline levels) with abnormal internal architecture leading to thick, but brittle bones.
2. High incidence in Europe, America, relatively rare in Asian countries, M=F, common in >50 yrs age
3. Familial involvement +
4. Link with viral infection suggested as probable etiology
5. Both Osteoblastic and Osteoclastic activities are elevated
6. Most common sites – Pelvis, Tibia
7. Most patients – ASYMPTOMATIC, incidental diagnosis on X-ray for other cause or elevated S. ALP levels
8. Clinical Features – Dull aching pain, deformities of extremity bones that later complicate as fractures, esp. anterior bowing of tibia, skin over the deformed bone appears warm (Osteitis deformans is therefore a synonym used for Paget’s disease), enlarged skull, Platybasia.
9. Cranial nerve compression – 5th, 7th, 8th CN, Spinal nerve root compression
10. Increased incidence of otosclerosis, Steal syndromes
11. X-ray – Osteoporosis circumscripta, flame shaped osteolytic lesion in diaphyseal region
12. Biochemical – S.Calcium and S.Phosphate – Normal. RAISED S.ALP, U.hydroxyproline
13. Risk of malignant transformation is around 1% with very poor prognosis.
14. Specific treatment to be considered if – Persistent bone pain, repeated fractures, high output cardiac failure, hypercalcemia due to immobilization, neurological complications
15. Treatment options – Calcitonin, Bisphosphonates
MyPGMEE Authors: Dr. Mudit Khanna, Dr. Sushant Bhanja, Dr. Punit Bhojani and Dr. Ashwin Singh Parihar.
HYT – PAGET’S DISEASE
1. Increased bone turnover (Therefore elevated serum ALP and urine Hydroxyproline levels) with abnormal internal architecture leading to thick, but brittle bones.
2. High incidence in Europe, America, relatively rare in Asian countries, M=F, common in >50 yrs age
3. Familial involvement +
4. Link with viral infection suggested as probable etiology
5. Both Osteoblastic and Osteoclastic activities are elevated
6. Most common sites – Pelvis, Tibia
7. Most patients – ASYMPTOMATIC, incidental diagnosis on X-ray for other cause or elevated S. ALP levels
8. Clinical Features – Dull aching pain, deformities of extremity bones that later complicate as fractures, esp. anterior bowing of tibia, skin over the deformed bone appears warm (Osteitis deformans is therefore a synonym used for Paget’s disease), enlarged skull, Platybasia.
9. Cranial nerve compression – 5th, 7th, 8th CN, Spinal nerve root compression
10. Increased incidence of otosclerosis, Steal syndromes
11. X-ray – Osteoporosis circumscripta, flame shaped osteolytic lesion in diaphyseal region
12. Biochemical – S.Calcium and S.Phosphate – Normal. RAISED S.ALP, U.hydroxyproline
13. Risk of malignant transformation is around 1% with very poor prognosis.
14. Specific treatment to be considered if – Persistent bone pain, repeated fractures, high output cardiac failure, hypercalcemia due to immobilization, neurological complications
15. Treatment options – Calcitonin, Bisphosphonates
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