Paget's Disease of Bone Flashcards
What is Paget’s disease?
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
What is the aetiology of Paget’s disease?
Cause is uncertain
- Family history
- Genetic Factors
- Viral infectious
- Rare if under 50
- Males
Which bones are most commonly affected in Paget’s disease?
(1) long bones
(2) pelvis
(3) lumbar spine
(4) skull
What are the three stages of Paget’s disease?
Osteolytic - bone resorption (large osteoclasts)
Mixed - both osteoclastic and osteoblastic activity
Osteosclerotic - abnormal bone formation (weaker than normal bone).
What is the result of the abnormal bone process in Paget’s disease?
Thick excess bone with abnormal reversal lines, creates a mosaic pattern. The bone becomes soft and porous, leading to increased fracture risk
What are the common clinical features of Paget’s disease?
(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)
What biochemistry findings are typical in Paget’s disease?
Elevated alkaline phosphatase (ALP) with normal liver function tests (LFTs)
What is the first-line treatment for Paget’s disease?
The first-line treatment is analgesia for pain management
What is the second-line treatment for Paget’s disease?
Bisphosphonates
- to reduce osteoclastic activity
What are the major complications of Paget’s disease?
Secondary malignancy in the affected bone, such as osteosarcoma and fibrosarcoma
What is the main investigation done?
XRAY
Paget’s disease is characterised by increased bone [Blank] and increased bone [Blank] ?
Formation and resorption
In Paget’s disease, bone turnover is [Blank]
High
What best describes osteoid?
Un-mineralised bone matrix
What is the most common intracellular protein in eukaryotic cells?
Actin
Describe what “motor neurone pool” means
The total motor neurone projection to a single muscle
How often should ALP levels be monitored?
every 6-12 months
Frontal bossing, hearing reduction and pain in the lower limbs are consistent with what disease?
Paget’s disease
What ethnicity is at greatest risk of Paget’s disease?
Central European
What are the complications of Paget’s Disease of Bone?
(1) Deafness
(2) Osteoarthritis
(3) Nerve compression
(4) Fractures
(5) Limb length discrepancy
(6) Osteosarcoma
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?
Woven bone
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?
Bony enlargement of something because Paget’s disease is characterised by disordered bone growth
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?
Blood tests:
(1) Full blood count
(2) urea and electrolytes
(3) bone profile
(4) liver function
