Metabolic Bone Disease Flashcards

1
Q

Which factors stimulate osteoblast expression of RANK ligand?

A
  • TNF-alpha
  • PTHrP
  • IL-1 and IL-11
  • Vitamin D
  • Glucocorticoids
  • PTH
  • PGE-2
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2
Q

What is Paget’s bone disease and what does it cause?

A
  • Increased bone resorption followed by increased bone formation
  • Leads to bigger, less compact, disorganised bone which is more vascular and more susceptible to deformity and fracture
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3
Q

Describe the aetiology of Paget’s disease

A
  • Genetics: large proportion are familial
  • Distribution restricted to those of anglo-saxon origins
  • Environmental: chronic viral infection within osteoclasts
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4
Q

What are the symptoms of Paget’s disease?

A
  • Patients > 40
  • Bone pain
  • Occasionally bone deformity
  • Excessive heat over Pagetic bone
  • Neurological complications e.g. nerve deafness
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5
Q

How does Paget’s disease present?

A
  • Isolated elevation of serum alkaline phosphatase
  • Bone pain and local heat
  • Bone deformity or fracture
  • Hearing loss
  • Rarely: osteosarcoma in affected bone
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6
Q

How can Paget’s disease be treated?

A
  • Only treat asymptomatic if in skull or other areas requiring surgical intervention
  • Not to be done on raised alkPhosphatase alone
  • IV bisphosphonate therapy - one off IV zoledronic acid
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7
Q

Which bone diseases are caused by severe nutritional vitamin D or calcium deficiency which causes insufficient mineralisation?

A
  • Rickets in children

- Osteomalacia in adults (whose epiphyseal lines are closed)

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8
Q

What are the clinical features of rickets?

A
  • Stunted growth
  • Large forehead
  • Odd curve to spine or back
  • Odd shaped ribs or breast bones
  • Large abdomen
  • Odd shaped legs
  • Wide joints at elbow and wrist
  • Wide ankles and wide bones in the leg
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9
Q

What is osteogenesis imperfecta?

A

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

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10
Q

List the four most common types of osteogenesis imperfecta and how they present

A
  • Type I: mild (when child starts to walk/can present in adults)
  • Type II: lethal by age 1
  • Type III: severe bone dysplasia and poor growth
  • Type IV: similar to type I but more severe
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11
Q

What are the signs and symptoms of osteogenesis imperfecta?

A
  • Growth deficiency
  • Defective tooth formation
  • Hearing loss
  • Blue sclera
  • Scoliosis
  • Barrel chest
  • Ligamentous laxity
  • Easy bruising
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12
Q

How can osteogenesis imperfecta be managed?

A
  • Surgically: to treat fractures
  • Medically: IV bisphosphonates (to prevent fractures)
  • Socially: educational and social adaptations
  • Genetic counselling
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13
Q

What is the definition of osteoporosis?

A

A 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

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14
Q

When would a DXA bone scan be diagnostic of osteoporosis (T score)?

A

A result of < -2.5 SDs below the young adult mean in a post menopausal woman

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15
Q

What is the risk of fracture related to in osteoporosis?

A
  • Age
  • Bone mass density
  • Falls
  • Bone turnover
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16
Q

Which tool is used to assess fracture risk?

A

FRAX - WHO fracture risk assessment tool

17
Q

What does the Q fracture score use to calculate risk and what are the drawbacks?

A
  • Aged 30-85, men/women
  • CVS risks, falls and TCA
  • No ability to add BMD and some of the variables/risks can’t be altered by osteoporotic medications
18
Q

List the endocrine causes of osteoporosis

A
  • Thyrotoxicosis
  • Hyper and hypoparathyroidism
  • Cushings
  • Hyperprolactinaemia
  • Hypopituitarism
  • Early menopause
19
Q

List the rheumatic causes of osteoporosis

A
  • Rheumatoid arthritis
  • Ankylosing spondylitis
  • Polymyalgia rheumatica
20
Q

List the GI causes of osteoporosis

A
  • Inflamm diseases: UC and Crohns
  • Liver: PBC, CAH, alcoholic cirrhosis and hep C
  • Malabsorption: chronic pancreatitis, coeliac disease, whipples disease, short gut syndromes and ischaemic bowel
21
Q

List the medications which can cause osteoporosis

A
  • Steroids
  • PPIs
  • Enzyme inducting anti-epileptics
  • Aromatase inhibitors
  • GnRH inhibitors
  • Warfarin
22
Q

How can osteoporotic fractures be prevented?

A
  • Minimise risk factors
  • Ensure good calcium and vitamin D status
  • Falls prevention strategies
  • Medications
23
Q

When should osteoporosis be treated?

A
  • T score below -2.5
  • Osteopenia: T score -1 to -2.5 with previous fracture
  • 20% 10 year risk at any site over the next 10 years
24
Q

When should a patient be referred for a DXA scan?

A

FRAX or QFracture score of > 10% fracture risk at any site over the next 10 years

25
Q

Which medications can be used to treat osteoporosis?

A
  • HRT
  • Selective Oestrogen Receptor Modulator (SERMS)
  • Bisphosphonates (main treatment option)
26
Q

What are the potential side effects of HRT?

A
  • Increased risks of blood clots
  • Increased risk of breast cancer with extended use
  • Increased risk of heat disease and stroke if used after large gap from menopause
27
Q

What are the negative effects of SERMS?

A
  • Hot flushes if taken close to the menopause
  • Increased clotting risks
  • Lack of protection at hip site
28
Q

Give two examples of biphosphonates

A
  • Alendronate

- Risedronate

29
Q

Give an example of a SERMS

A

Raloxifene

30
Q

What are the potential side effects of bisphosphonates?

A
  • Oesophagitis
  • Iritis/uveitis
  • ONJ?
  • Atypical femoral shaft fractures?
  • Need a drug holiday after ten years
31
Q

What is denosumab and how is it used?

A
  • Monoclonal antibody against RANKL
  • Reduces osteoclastic bone resorption
  • SC injection every 6 months
  • Safer in patients with significant renal impairment
32
Q

What are the potential side effects of denosumab?

A
  • Allergy/rash
  • Symptomatic hypocalcaemia ( if given when vitD deplete)
  • ?ONJ
  • ? Atypical femoral shaft fractures
33
Q

What is teripatide?

A

A synthetic version of the parathyroid hormone which regulates calcium metabolism

34
Q

What are the potential side effects of teriparatide?

A
  • Injection site irritation
  • Rarely hypercalcaemia
  • Allergy
  • (Cost)