Metabolic Bone Disease

Question 1

What is Paget’s Disease of Bone?

Answer 1

Disorder of bone turnover, increased bone resorption followed by increased formation

Question 2

What does Paget’s lead to?

Answer 2

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

Question 3

Who is at risk of Paget’s disease?

Answer 3

Family history
Those of Anglo-Saxon origins
Possibility of chronic viral infection within osteoclast

Question 4

What are general clinical features of Paget’s?

Answer 4

Isolated elevation of serum Alkaline Phosphatase
Hearing loss
Presents in patients >40 with bone pain
Occasionally presents with bone deformity
Excessive heat over Paget’s bone
Neurological complications such as nerve deafness

Question 5

How is Paget’s treated?

Answer 5

No treatment for asymptomatic unless in skull

If symptomatic IV bisphosphonate therapy - zoledronic acid

Question 6

What is the difference between rickets and osteomalacia?

Answer 6

Rickets is in children before epiphyseal growth plates have sealed and vice versa for adults

Question 7

What causes rickets/osteomalacia?

Answer 7

Severe vitamin D or calcium deficiency leading to insufficient mineralisation
Vitamin D required for calcium and phosphate absorption

Question 8

What is osteogenesis imperfecta?

Answer 8

Genetic disorder of connective tissue characterised by fragile bones from mild trauma and even acts of daily life
Broad clinical range from presenting in 40s to prenatally fatal

Question 9

What is type 1 osteogenesis imperfecta?

Answer 9

Milder form - when child starts to walk and can present in adults

Question 10

What is type 2 osteogenesis imperfecta?

Answer 10

Lethal by age 1

Question 11

What is type 3 osteogenesis imperfecta?

Answer 11

Progressive deforming with severe bone dysplasia and poor growth

Question 12

What is type 4 osteogenesis imperfecta?

Answer 12

Similar to type 1 but more severe

Question 13

What are clinical features of osteogenesis imperfecta?

Answer 13

Growth deficiency
Defective tooth formation
Hearing loss
Blue sclera
Scoliosis
Barrel chest
Ligamentous laxity
Easy bruising

Question 14

How is osteogenesis imperfecta managed?

Answer 14

Surgical - to treat fractures

Medical -prevent fractures with IV bisphosphonates

Question 15

What is osteoperosis?

Answer 15

Metabolic bone disease characterised by low bone mass and micro architectural deterioration of bone tissue, leading to enhanced bone fragility and a consequent increase in fracture risk

Question 16

What happens to bone mass as age increases?

Answer 16

It decreases

Question 17

What happens to bone mass in menopausal/post menopausal women?

Answer 17

Decreases

Question 18

What is the most important aspect of osteoperosis?

Answer 18

Increased risk of fracture

Question 19

What tool can be used to determine risk of fracture?

Answer 19

Q Fracture or FRAX

Question 20

How common is osteoperosis?

Answer 20

50% of women over 50 will have an osteoperotic fracture and 20% of men

Question 21

What are endocrine causes of osteoperosis?

Answer 21

Thyrotoxicosis
Hyper/hypoparathyroidism
Cushings
Hyperprolactinaemia
Hypopituitarism
Early menopause

Question 22

What are rheumatic causes of osteoperosis?

Answer 22

Rheumatoid arthritis
Ankylosing spondylitis
Polymyalgia rheumatica

Question 23

What are Gastroenterological causes of osteoperosis?

Answer 23

Inflammatory diseases - Ulcerative colitis and Crohn’s
Liver diseases - Cirrhosis, primary biliary cholangitis,
Malabsorption - pancreatitis, coeliac disease, ischaemic bowel

Question 24

What medications can cause osteoporosis?

Answer 24

Steroids
Proton pump inhbitors
Enzyme inducting antiepileptic medications
Growth Hormone Releasing Hormone inhibitors
Warfarin

Question 25

How are osteoporotic fractures prevented?

Answer 25

Minimise risk factors
Ensure good calcium and vitamin D status
Fall prevention strategies
Medications

Question 26

What is measured to determine level of treatment for osteoperosis?

Answer 26

Bone mineral density

Question 27

What is considered a normal bone mineral density, and how would someone be treated?

Answer 27

T score >-1

Lifestyle advice

Question 28

What bone mineral density would be indicative of osteoporosis?

Answer 28

T score < -2.5

Question 29

What are side effects of hormone replacement therapy?

Answer 29

Risk of blood clots
Increased risk of breast cancer with extended use
Increased risk of heart disease and stroke

Question 30

What is the main treatment used for osteoporosis?

Answer 30

Bisphosphonates

Question 31

What is generally required in the patient for bisphosphonates to be an option?

Answer 31

Adequate renal function
Adequate calcium and vitamin D status
Good dental health and hygiene advised

Question 32

What is the action of bisphosphonates?

Answer 32

Inhibition of osteoclasts

Question 33

What are possible side effects of bisphosphonates?

Answer 33

Oesophagitis
Iritis/uveitis
Atypical femoral shaft fractures

Question 34

What are treatment options for osteoperosis?

Answer 34

Hormone replacement therapy
Selective oEstrogen Receptor Modulator (SERM)
Bisphosphonates
Denosumab
Teriparatide

Question 35

What are negative effects of SERMs?

Answer 35

Hot flushes if taken close to menopause
Increased clotting risks
Lack of protection at hip site

Question 36

What is denosumab?

Answer 36

Monoclonal antibodies
Subcutaneous injection every 6 months
Reduce osteoclastic bone resorption
Safer in patients with renal dysfunction

Question 37

What are negative effects of denosumab?

Answer 37

Allergy/rash

Symptomatic hypocalcaemia if given when vitamin D deficient

Question 38

What are negative effects of teriparatide?

Answer 38

Injection site rash
Rarely hypercalcaemia
Allergy
Very expensive