paget's%20bone%20disease
PAGET'S BONE DISEASE
Paget's bone disease, also known as osteitis deformans, is characterized by a significant increase in bone resorption and turnover in localized parts of the skeleton causing enlargement and thickening of the bone that is disordered and architecturally unstable.
The prevalence increases with age, with men and women affected equally.
Genetic factors and/or viral infection may play a role in the etiology.
May affect one bone (monostotic) or several bones (polyostotic).
By decreasing frequency, involved bones may include pelvic bone and sacrum, spine, skull and femur, tibia, humeri and clavicles.

Paget's%20bone%20disease Management

Follow Up

Frequency and extent of follow-up will depend upon severity of disease

  • Monitor serum alkaline phosphatase (ALP) every 3-6 months to determine the biochemical response to bisphosphonate therapy
    • Once the value of serum ALP plateaus after 3-6 months of assessment, it can be measured 1-2x/year as a marker of bone activity
  • A more specific bone formation/resorption marker other than ALP (ie amino-terminal propeptide of type 1 collagen [P1NP], βC-terminal propeptide of type-1 collagen [βCTx], N-terminal propeptide of type 1 collagen [NTx] assay) may be measured for patients with untreated monostotic Paget’s disease
  • Serial imaging tests (eg bone scan) are not generally useful in routine monitoring of treatment response
    • Bone scan may be helpful in monitoring treatment response after 6-12 months of treatment in patients with monostotic disease
  • If treatment failure is apparent consider re-treatment 6 months after the initial therapy for the following:
    • Symptom relapse or persistence
    • >25% above nadir of serum ALP in asymptomatic patient
  • Long-term evolution increases the risk for osteosarcoma on pagetic bone
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