Paget's disease of bone

Question 1

Which sites are affected in paget’s disease?

Answer 1

pelvis, femur, lumbar spine, skull, tibia

Question 2

What are the risk fx for paget’s?

Answer 2

smoking, viral infection, family hx, genetic mutations

Question 3

Name a virus associated with paget’s?

Answer 3

measles

Question 4

What is the pathogenesis of paget’s?

Answer 4

pathogenic changes in osteoclasts which leads to increased bone resorption. Increased activity of osteoblasts which lay down weaker woven bone to account for activity of osteoclasts

Question 5

What is a typical presentation of paget’s?

Answer 5

70% of patients are asymptomatic and are picked up by raised ALP or XR changes. Common complaint is bone pain + bone overgrowth syx e.g. nerve compression- hearing loss with skull involvement

Question 6

Which features of examination are characteristic of paget’s disease?

Answer 6

bowed tibia or shortening of legs

Question 7

Name three complications of paget’s

Answer 7

nerve root compression, hearing loss, spinal stenosis, OA, fractures, osteosarcoma

Question 8

Which investigation is the most specific and sensitive for paget’s?

Answer 8

bone biopsy

Question 9

Which features of cells seen in bone biopsy are synonymous with paget’s?

Answer 9

osteoclasts with increased nuclei and disorganised matrix and lamellar bone formation

Question 10

What is the management for paget’s?

Answer 10

analgesia, biphosphonates,vit D/Ca2+ supplements,, physio, OT, orthoritcs, corrective surgery, joint replacement

Question 11

Which blood markers are monitored in paget’s?

Answer 11

ALP

Question 12

Three investigations for Paget’s disease?

Answer 12

Blood tests- LFTs, bone profile
Bone scan: hot spots
X-ray

Question 13

Two features on xray of paget’s disease?

Answer 13

  Bone enlargement 
  Sclerosis 
  Patchy cortical thickening 
  Deformity 
  Wedge-shaped lytic lesions 
  Osteoporosis circumscripta 
  Well-defined lytic skull lesions

Question 14

What would be the levels of ALP, Ca, and PO4 in paget’s disease?

Answer 14

raised ALP

Ca and PO4 normal

Question 15

What is a classical feature on radiographs of Paget’s?

Answer 15

blade of grass sign/flame sign

Question 16

What is the malignant progression of Paget’s disease?

Answer 16

Paget’s sarcoma

Question 17

Which disease increases the risk of forming osteosarcomas?

Answer 17

Paget’s disease