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E - MSK > Metabolic Bone Disease > Flashcards

Metabolic Bone Disease Flashcards

1
Q

What is Paget’s disease

A

Disorder of bone turnover

There is increased bone resorption followed by increased bone formation leading to disorganised bone

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2
Q

What is bone like in Paget’s (4)

A

Bigger
Less compact
More vascular
More susceptible to deformity and fracture

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3
Q

What percentage of Paget’ is familial - what is the gene involved

A

15-30%
Loci of SQSTMI
Anglo-Saxon origins

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4
Q

Presentation of Paget’s (5)

A

Bone pain - blood gives deep pulsating pain
Bone deformity
Excessive heat over Pagetic bone
Neurological complications - i.e. nerve deafness
Development of osteosarcoma in affected bone (rare)

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5
Q

What age group does Paget’s occur in

A

Over 40s

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6
Q

What scan is used for Paget’s - what does it show

A

Isotope bone scan shows area of dense bone

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7
Q

What bone and liver enzyme is affected by Paget’s and how

A

Serum alkaline phosphatase is elevated

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8
Q

differential of Paget’s

A

metastatic cancer

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9
Q

When is treatment for Paget’s indicated

A

If in skull or area requiring surgical intervention - do not treat based on a raised AlkPhos alone

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10
Q

What medication is used for Paget’s

A

IV bisphosphonate

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11
Q

what is a type of bisphosphonate used for Pagets

A

Zoledronate injection (one off)

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12
Q

What is the difference between rickets and osteomalacia

A

Ricket’s is before the epiphyseal plates have fused

Osteomalacia is after the epiphyseal plates have fused

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13
Q

Clinical presentation of rickets (4)

A

Stunted growth
Bandy legs (wide)
Large skull (fontanelles do not fuse)
Failure to thrive

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14
Q

Clinical presentation of osteomalacia (5)

A 
No visible deformity 
Microfractures 
Bone pain 
Muscle weakness
Increased fall risk
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15
Q

Who is most at risk of osteomalacia

A

Institutionalised

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16
Q

what causes rickets or osteomalacia

A

Severe nutritional vitamin D or calcium deficiency causes insufficient mineralisation.
Vit D stimulates calcium and phosphate absorption from the gut which then becomes available for bone mineralisation.
So less vit D means less Calcium and phosphate so less bone mineralisation

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17
Q

Apart from reduced bone mineralisation, what else in the body is affected by low vitamin D states

A

Muscle function

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18
Q

What is osteogenesis imperfecta

A

Genetic disorder of connective tissue characterised by fragile bones from mild trauma and even acts of daily life

19
Q

Who does OI affect

A

Broad range from prenatally fatal to those presenting in 40s with “early osteoporosis

20
Q

Where is the defect which causes OI

A

Defects in type I collagen

21
Q

What are the 4 most common types of OI

A

T1 - milder form, occurs when child starts to walk, can present in adults

T2 - lethal by age 1

T3 - progressive deforming with severe bone dysplasia and poor growth

T4 - similar to T1 but more severe

22
Q

Other clinical features of OI (7)

A

Growth deficiency
Defective tooth formation (dentinogenesis imperfecta)
Hearing loss
Blue sclera
Scoliosis / Barrel chest
Ligamentous laxity (hyper mobility brighten score)
Easy bruising

23
Q

Management of OI (4)

A

Surgery to treat fractures
IV Bisphosphonates (to prevent fracture)
Educational and social adaptations
Genetic counselling for parents and next generation

24
Q

What is osteoporosis

A

A metabolic bone disease characterised by low bone mass and deterioration of bone tissue resulting in bone fragility and increased fracture risk

25
Q

What tools are used to assess fracture risks (2) - what do they assess and what are the difference between these tools

A

Both assess 10 year probability of bone fracture risk
FRAX - uses clinical risk factors and BMD at the femoral neck
Q Fracture - does not have ability to add BMD

26
Q

If a patient has significant risk of fracture result from FRAX/Q Fracture, what should they be referred for

A

DEXA scan

risk >10%

27
Q

Who should be referred for a DEXA scan for osteoporosis regardless of their fracture risk percentage

A

If on oral steroids

If suffered a low trauma fracture

28
Q

What does DEXA scan measure

A

Bone mineral density which is then compared to the BMD of a healthy adult and someone who is same age and sex as patient.
The difference is calculated as a SD and called a T score

29
Q

What T score is defined as osteoporosis

A

Below -2.5 SD

30
Q

What bones are good to scan in DEXA and why

A

L1-L4 as spine has more cortical bone than trabecular bone so commonly affected by osteoporosis
L1-4 also doesn’t have other bones around it to interfere with the scan

31
Q

Endocrine causes of osteoporosis (6)

A 
Thyrotoxicosis 
Hyper and Hypoparathyroidism 
Cushing's
Hyperprolactinaemia 
Hypopituitarism 
Low sex hormone levels
32
Q

Rheumatic causes of osteoporosis (3)

A

RA
Ankylosing Spondylitis
Polymyalgia Rheumatica (steroids used making bones thin)

33
Q

GI causes of osteoporosis (11)

A 
IBD 
Primary biliary cirrhosis
Alcoholic cirrhosis 
Congenital adrenal hyperplasia 
Viral cirrhosis (hep C)
Cystic Fibrosis (causes malabsorption)
Chronic pancreatitis 
Coeliac disease
Whipple's disease 
Short gut syndromes 
Ischaemic bowel
34
Q

Medications which cause osteoporosis

A 
Steroids 
PPI 
Enzyme inducing anti-epileptic medications
Aromatase inhibitors 
GnRH agonists 
Warfarin
35
Q

Non pharmacological ways to prevent osteoporosis

A

Minimise risk factors
Ensure good calcium and Vitamin D status
Falls prevention strategies

36
Q

Medications to treat osteoporosis (5)

A

Oral bisphosphonates (main)
HRT
Selective Oestrogen Receptor Modulator-Raloxifene (SERMS)
Denosumab s/c injection (6m)
Teriparatide (human parathyroid hormone) injection

37
Q

What needs to be checked for bisphosphonates

A

Renal function
Calcium and Vit D status
Good dental health and hygiene - notify dentist

38
Q

Side effects of bisphosphonates (4)

A

Osteonecrosis of Jaw
Oesophagitis
Iritis / uveitis
Atypical femoral shaft fractures

39
Q

Side effects of HRT

A

Blood clots risk
Breast cancer risk increased
Heart disease and stroke risk increased if used after large gap from menopause

40
Q

Side effects of SERMS

A

Hot flushes
Increased clotting risks
Leg cramps

41
Q

What is denosumab and how does it help in osteoporosis

A

Monoclonal antibody against RANKL
Reduces osteoclastic bone resorption
Safer in patients with significant renal impairment

42
Q

Side effects of denosumab

A

Allergy / rash
Symptomatic hypocalcaemia if given when via D depleted
ONJ
Atypical femoral shaft #

43
Q

Teriparatide side effects

A

injection site irritation
Hypercalcaemia (rare)
allergy
COST

E - MSK (29 decks)

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