Metabolic Bone Disease

Question 1

What cells create pre-osteoclasts?

Answer 1

Myeloid progenitor cell

Question 2

What cells make pre-osteoblasts?

Answer 2

Mesenchymal progenitor cell

Question 3

Factors that stimulate osteoblasts expression of RANK ligand?

Answer 3

PGE2, PTH, glucocorticoids, vitamin D, IL-11, IL-1, PTHrP, TNF-a

Question 4

What is Paget’s disease of the bone?

Answer 4

Localised disorder of bone turnover, there is increased bone resorption followed by increased bone formation

Question 5

What does the disorder of bone turnover in Paget’s disease lead to?

Answer 5

Disorganised bone that is bigger, less compact, more vascular and more susceptible to deformity and fracture

Question 6

Does Paget’s disease have a genetic or environmental trigger?

Answer 6

Genetic component- Loci of SQSTMI

Environmental trigger- ?chronic viral infection within the osteoclasts

Question 7

Symptoms of Paget’s disease?

Answer 7

Bone pain, 
Bone deformity or fracture,
Local heat, 
Neurological complications eg hearing loss,
Osteosarcoma ?

Question 8

Sign of Paget’s disease?

Answer 8

Local heat,
Bone deformity or fracture,
Isolated elevation of serum alkaline phosphatase

Question 9

Should you treat asymptomatic Paget’s disease?

Answer 9

Not unless in skull or in area requiring surgical intervention

Question 10

treatment of symptomatic Paget’s disease?

Answer 10

Intravenous Bisphosphonates therapy,

One off IV zoledronic acid

Question 11

What deficiency causes rickets and osteomalacia?

Answer 11

Vitamin D or Calcium

Question 12

Rickets signs?

Answer 12

Wide bones, 
Wide ankles, 
Odd-shaped legs, 
Large abdomen, 
Wide joints at elbow and wrist,
Odd shaped ribs and breast bones, 
Odd curve to spine or back,
Large forehead, 
Stunted growth

Question 13

Osteomalacia signs?

Answer 13

Bone pain and tenderness, fractures

Question 14

Define osteogenesis imperfecta

Answer 14

Genetic disorder of connective tissue, causing fragile bones

Question 15

Osteogenesis imperfecta pathology

Answer 15

Defects in type 1 collagen

Question 16

The first 4 types of osteogenesis imperfecta?

Answer 16

Type 1- milder, child can walk so presents in adults,

Type 2- lethal by age 1

Type 3- progressive deforming with sever bone dysplasia and poor growth

Type 4- similar to type 1 but more severe

Question 17

Signs/ symptoms of osteogenesis imperfecta

Answer 17

Fragile bones, 
Growth deficiency,
Defective tooth formation (dentigenesis imperfecta), 
Hearing loss, 
Blue sclera, 
Scoliosis, 
Barrel chest, 
Ligamentous laxity, 
Easy bruising

Question 18

Osteogenesis imperfecta investigations

Answer 18

Examination,

The Beighton score

Question 19

Management of Osteogenesis Imperfecta

Answer 19

Surgical (for fractures),
IV bisphosphonates,
Genetic counselling,
Social adaptations