Clinical features:
Paget’s disease is suspected when there is bone pain, with bowing of tibia or kyphosis. Although it's symptomatic in about only 25%.
Pain is localized in about one third of patients.
Headache and deafness may indicate skull involvement.
Pathological fractures may occur
Laboratory findings:
Marked elevation of serum alkaline phosphatase
Raised serum procollagen type-I N-terminal propeptide (PINP)
Increased urinary N-telopeptide of type 1 collagen cross-links.
Hypercalcemia
Imaging
Osteolytic skull lesions (osteoporosis circumscripta).
Long bone lytic lesions.
Areas of bone sclerosis may occur mixed with lyric bone lesions.
In patients with normal radiography, technetium pyrophosphate bone scans can detect bone lesions with active turnover.
Treatment:
Asymptomatic patients usually need only follow up, unless significant radiological changes are evident.
Bisphosphonates:
Zoledronic acid is the most effective and the most commonly used.
Dose : 5 mg single dose intravenously
Other bisphosphonates:
Risedronate: 30 mg orally, daily for 2 months. Alendronate: 40 mg orally, daily for 6 months.
Calcium and vitamin D supplements to prevent hypocalcemia induced by bisphosphonates.
Response to therapy is shown by reduction of serum alkaline phosphatase and symptomatic improvement.
No comments:
Post a Comment