pagets disease

Question 1

what is Paget’s disease

Answer 1

Focal disorder of bone remodelling
(areas of patchy bone due to improper osteoblast/clast function)

Question 2

commonly affected areas

Answer 2

skull
spine
pelvis

Question 3

symptoms

Answer 3

• bone / joint pain
• warm
• changes in bone shape
• peripheral neuropathy
• balance problems
• mostly asymptomatic

Question 4

treatments

Answer 4

bisphosphonate
- oral risedronate
- IV zoledronate

supportive therapies
surgery
Ca + VitD

Question 5

pathophysiology

Answer 5

increased and uncontrolled bone turnover
- increased osteoclastic activity
- increased osteoblastic activity

Question 6

typical presentation

Answer 6

• older male
• bone pain
• raised ALP
• pelvis, lumbar, skull, femur
• bowling of tibia and bossing of skull

Question 7

invetsigations

Answer 7

bloods
- raised ALP
- Ca + phosphate normal

markers of bone tunover
xray - osteolysis early disease + thickened skull + cotton wool skull

Urinary hydroxyproline: protein constituent of bone collagen = good marker of progression

Question 8

markers of bone turnover

Answer 8

PINP
NTx
CTx
urinary hydroxyproline

Question 9

whats shown on an xray

Answer 9

osteolysis in early disease
thickened skull

Question 10

complications

Answer 10

• deafness
• bone sarcoma
• fractures
• skull thickening
• high output cardiac failure