Paget's Disease of Bone

Question 1

What is Paget’s disease?

Answer 1

Paget’s disease is a chronic condition involving cellular remodeling and deformity of one or more bones, often resulting in weakened bone structure and increased fracture risk

Question 2

What is the aetiology of Paget’s disease?

Answer 2

Cause is uncertain

• Family history
• Genetic Factors
• Viral infectious
• Rare if under 50
• Males

Question 3

Which bones are most commonly affected in Paget’s disease?

Answer 3

(1) long bones
(2) pelvis
(3) lumbar spine
(4) skull

Question 4

What are the three stages of Paget’s disease?

Answer 4

Osteolytic - bone resorption (large osteoclasts)

Mixed - both osteoclastic and osteoblastic activity

Osteosclerotic - abnormal bone formation (weaker than normal bone).

Question 5

What is the result of the abnormal bone process in Paget’s disease?

Answer 5

Thick excess bone with abnormal reversal lines, creates a mosaic pattern. The bone becomes soft and porous, leading to increased fracture risk

Question 6

What are the common clinical features of Paget’s disease?

Answer 6

(1) Often asymptomatic

(2) Pain from micro-fractures or nerve compression

(3) Bone enlargement and deformity (e.g., sabre tibia, platybasia)

(4) Increased metabolic activity, leading to symptoms like the warmth of the skin and high-output heart failure

(5) Hearing loss (if the skull is involved)

Question 7

What biochemistry findings are typical in Paget’s disease?

Answer 7

Elevated alkaline phosphatase (ALP) with normal liver function tests (LFTs)

Question 8

What is the first-line treatment for Paget’s disease?

Answer 8

The first-line treatment is analgesia for pain management

Question 9

What is the second-line treatment for Paget’s disease?

Answer 9

Bisphosphonates

• to reduce osteoclastic activity

Question 10

What are the major complications of Paget’s disease?

Answer 10

Secondary malignancy in the affected bone, such as osteosarcoma and fibrosarcoma

Question 11

What is the main investigation done?

Answer 11

XRAY

Question 12

Paget’s disease is characterised by increased bone [Blank] and increased bone [Blank] ?

Answer 12

Formation and resorption

Question 13

In Paget’s disease, bone turnover is [Blank]

Answer 13

High

Question 14

What best describes osteoid?

Answer 14

Un-mineralised bone matrix

Question 15

What is the most common intracellular protein in eukaryotic cells?

Answer 15

Actin

Question 16

Describe what “motor neurone pool” means

Answer 16

The total motor neurone projection to a single muscle

Question 17

How often should ALP levels be monitored?

Answer 17

every 6-12 months

Question 18

Frontal bossing, hearing reduction and pain in the lower limbs are consistent with what disease?

Answer 18

Paget’s disease

Question 19

What ethnicity is at greatest risk of Paget’s disease?

Answer 19

Central European

Question 20

What are the complications of Paget’s Disease of Bone?

Answer 20

(1) Deafness
(2) Osteoarthritis
(3) Nerve compression
(4) Fractures
(5) Limb length discrepancy
(6) Osteosarcoma

Question 21

There is a marked difference in the characteristics of adult bone and bone that is first formed in development.
Which term describes bone first formed in development?

Answer 21

Woven bone

Question 22

A 78-year-old female patient is on an acute ward being treated for an infective exacerbation of COPD. One night, she slips on the floor whilst mobilising to the toilet.
Upon examination, she complains of severe pain in her left hip. She is unable to weight bear. An X-ray of her pelvis and hip is performed which reveals a fracture.

What radiological results would support a diagnosis of Paget’s disease?

Answer 22

Bony enlargement of something because Paget’s disease is characterised by disordered bone growth

Question 23

A 55-year-old male presents to his GP practice with an increasingly prominent forehead. He also complains of reduced hearing and as he walks downhill he notices that his legs become increasingly painful. On examination, he has no focal neurology in his lower limbs.

What is a suitable first-line investigation?

Answer 23

Blood tests:

(1) Full blood count
(2) urea and electrolytes
(3) bone profile
(4) liver function