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Snapshot
  • A 60-year-old man presents to his primary care physician with hip pain and hearing loss. He denies any trauma to the hip or any recent changes in his medications. He also notes his hat fits tighter than before. Physical examination is unremarkable. Laboratory testing is significant for an elevated alkaline phosphatase level in the setting of normal serum calcium, phosphate, and parathyroid hormone.
Introduction
  • Clinical definition
    • a progressive metabolic bone disorder of abnormal bone remodeling
      • excessive bone resorption and formation is present
  • Epidemiology
    • incidence
      • second most common metabolic bone disorder (osteoporosis is first)
    • age
      • > 55 years of age
  • Etiology
    • unknown
  • Pathogenesis
    • increased multinucleated osteoclastic and osteoblastic activity leads to a high bone remodeling rate resulting in abnormal bone architecture (lytic and blastic lesions) 
  • Prognosis
    • expansion of untreated Paget lesions
Presentation
  • Symptoms
    • usually asymptomatic
    • bone and/or joint pain
    • bone deformity
      • bowing deformity
    • hearing loss
      • with skull involvement that leads to narrowing of the auditory foramen
  • Physical exam
    • typically unremarkable
Imaging
  • Radiography
    • indication
      • to identify osteolytic, osteoblastic, and sclerotic bone changes
  • Bone scintigraphy
    • indication
      • to determine the extent of Paget disease of bone and to assess for other sites of involvement
Studies
  • Labs
    • normal serum phosphate, calcium, and parathyroid hormone
    • elevated alkaline phosphatase
  • Histology
    • mosaic pattern of lamellar bone with prominent cement lines
Differential
 
Laboratory Abnormalities in Select Bone Disorders
Etiology
Serum Phosphate
Serum Calcium
Serum Alkaline Phosphatase
Parathyroid Hormone
Osteomalacia/rickets  Decreased Decreased Increased Increased
Osteoporosis   Normal Normal Normal Normal
Osteopetrosis  Normal Normal or decreased Normal Normal
Paget disease of the bone  Normal Normal Increased Normal
Osteitis fibrosa cystica  Primary
hyperparathyroidism
• decreased
Secondary
hyperparathyroidism
• increased

Primary
hyperparathyroidism
• increased

Secondary
hyperparathyroidism
• decreased

Primary and secondary hyperparathyroidism
• increased
Primary and secondary hyperparathyroidism
• increased
Hypervitaminosis D Increased Increased Normal Decreased
 
Treatment
  • Medical
    • bisphosphonates
      • indication
        • first-line treatment for Paget disease of bone
  • Surgical
    • orthopedic surgery (e.g., joint/knee arthroplasty)
      • indication
        • to manage symptomatic complications of Paget disease of bone (e.g., osteoarthritis, pathologic and pseudofractures, and spinal stenosis)
Complications
  • Neurologic
    • nerve root compression
    • hearing loss
    • spinal stenosis
  • Orthopedic
    • pathologic fractures
    • secondary osteoarthritis
  • Oncologic
    • osteosarcoma
  • Cardiovascular
    • high-output heart failure
 

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