questions 8

Paget Disease of Bone

Topic updated on 09/07/17 6:32pm

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
  • 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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AVERAGE 4.0 of 4 RATINGS

Qbank (3 Questions)

TAG
(M3.OR.2) A 67-year-old male presents to the emergency department after a motor vehicle collision. The patient is complaining only of left hip pain which has been ongoing for the past 6-8 months. Examination of the left hip reveals mild crepitus, appropriate range of motion, adequate distal sensation, and palpable posterior tibial and dorsalis pedis pulses. A CT scan is ordered as part of the trauma protocol for a high energy motor vehicle collision (Figure A). Labs demonstrate alkaline phosphatase of 876 U/L (40-120) and normal vitamin D, calcium, and PTH levels. What is this patient's most likely diagnosis? Topic Review Topic
FIGURES: A          

1. Hip arthritis secondary to degenerative changes
2. Hip arthritis secondary to traumatic event
3. Osteogenic sarcoma
4. Paget's disease of bone
5. Multiple myeloma

PREFERRED RESPONSE ▶
TAG
(M3.OR.3) A 67-year-old male presents with right hip pain. He is also concerned because he had to go up a notch on his favorite alumni hat. He is accompanied by his wife who complains that he is constantly turning up the volume on their television, and that she sometimes has to shout to get his attention. Labs reveal serum alkaline phosphatase 875 U/L (40-120), with bone fraction 190 U/L (15-40). Urinary pyridine and deoxypyridine are 87 nmol/mmol creatinine (12.8-25.6) and 12.5 nmol/mmol creatinine (2.3-5.5), respectively. Vitamin D, calcium and PTH levels are normal. If this patient's hearing loss is related to the same overall disease process, which of the following are true statements? Topic Review Topic

1. The associated hearing loss is reversible with appropriate treatment
2. The course of the hearing loss cannot be slowed or modified
3. Hearing loss can be slowed with bisphosphonates
4. Hearing loss is an uncommon presentation of this condition
5. None of the above are true

PREFERRED RESPONSE ▶
TAG
(M2.OR.2) A 55-year-old male presents with left hip pain and stiffness. Laboratory studies show a normal serum calcium, increased serum alkaline phosphatase, and increased urinary N-telopeptide. Radiographs are shown in Figures A and B. A biopsy of the left femur is performed and is shown in Figure C. This patient is at an increased risk of all of the following EXCEPT: Topic Review Topic
FIGURES: A   B   C      

1. Hearing loss
2. Sarcoma
3. Frontal bossing
4. Plexiform-type neurofibromas
5. Cranial nerve palsies

PREFERRED RESPONSE ▶
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