Biochem disorders of bone Flashcards

1
Q

Give examples of bisphosphonates

A

Alendronate
Risedronate
Etidronate

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2
Q

What is desunomab

A

Monoclonal antibody which reduces osteoclastic activity

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3
Q

What does strontium do?

A

Increases osteoblast replication and reduces resorption

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4
Q

What is Zoledronic acid?

A

Once yearly IV bisphosphpnate (but v expensive)

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5
Q

Risks of HRT

A

Inreased breast and endometrial cancer rates

DVT

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6
Q

Risks of Raloxifene (oestrogen receptor modulator)

A

DVT

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7
Q

Qualitive defect of bone

A

Osteomalacia

  • due to deficient mineralisation of osteoid
  • INADEQUATE AMOUNTS OF CALCIUM AND PHOSPHATE
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8
Q

Psuedofractures (Looser’s zones)

A

Osteomalacia

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9
Q

Patients may complain of bone pain, have deformities of soft bones, sustain pathological fractures easily and have symptoms of hypocalcemia

A

Osteomalacia

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10
Q

Low calcium
Low phosphate
High serum alk phos

A

Osteomalacia

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11
Q

Blood biochem in osteoporosis

A

Normal

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12
Q

Brown tumours

A

Caused by hyperparathyroidism (lytic lesions in bone)

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13
Q

Osteitis fibrosa cystica

A

Caused by hyperparathyroidism

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14
Q

Treatment for high serum calcium

A

EMERGENCY
IV fluids
Bisphosphonates
Calcitonin

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15
Q

Treatment of hyperparathyroidism

A

Removal of adenomatous glands
Treatment of underlying cause (vitamin D supplementation)
Emergency treatment for very high calcium

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16
Q

What is renal osteodystrophy

A

Typical bone changes due to CKD
(reduced phosphate excretion and inactive activation of vitamin D –> sclerosis of bones and calcification of soft tissues)

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17
Q

Type 1 osteoporosis

A

Post menopausal

Colles fracture and vertebral insufficiency fracture most likely

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18
Q

Type 2 osteoporosis

A

Osteoporosis of old age

Fracture of vertebra and femoral neck most common

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19
Q

Causes of hypophosphatemia

A

Refeeding syndrome

Alcohol excess

20
Q

Principal cause of osteomalacia

A

Insufficienct calcium from the intestine because of lack of dietary calcium or a deficiency of or resistance to the action of vitamin D, or phosphate deficiency caused by increased renal losses

21
Q

Drugs associated with poor bone quality

A

Long term anticonvulsant use

22
Q

Treatment for pagets

A

Bisphosphonates (inhibit osteoclasts) or calcitonin if extensive lytic disease

23
Q

Defect of which type of collagen in osteogenesis imperfecta?

A

Type 1

24
Q

Osteogenesis imperfecta method of inheritence

A

Autosomal dominant

25
Q

Blue sclera and loss of hearing

A

Osteogenesis imperfecta

26
Q

Sofield procedure

A

Multiple osteotomies and intramedullary stabilisation for progressive deformity caused by OSTEOGENESIS IMPERFECTA

27
Q

Prominent forehead widened nose
Lax joints
Normal mental development

A

Achondroplasia

28
Q

Marfan inheritence

A

Autosomal dominant

29
Q

Tall stature, disproportionately long limbs and ligamentous laxity
High arched paalte
Scoliosis
Pectus excavatum
Eye problems (lens dislocation, retinal detachment)
Aortic aneurysm
Cardiac valve incompetence

A

Marfan syndrome

30
Q

Which dislocation common in Down syndrome?

A

Patella

Atlanto-axial instability in the C-spine

31
Q

Profound joint hypermobility, vascular fragility with ease of bruising, joint instability and scoliosis

A
Ehlers Danlos 
(autosomal dominant)
32
Q

Diagnosis is by raised serum creatinine phosphokinase and abnormalities on muscle biopsy

A

Duchenne muscular dystrophy

33
Q

Hypoplastic bones

A

Short

34
Q

If you have limb malformations caused in gestation, when would they have occurred?

A

between 4th and 6th week of gestation

35
Q

What is syndactyly?

A

COMMONEST congenital malformation
-two digits are fused
(usually separated age 3/4)

36
Q

Polydactyly

A

Extra digit

37
Q

Fibular hemimelia

A

Absence of fibula

  • leads to shortened limb
  • bowing of tibia
  • ankle deformity
38
Q

Most common congenital fusion

A

Tarsal coalition

39
Q

Erbs palsy

A

Damage to C5 and C6 during pregnancy

loss of motor innervation of the deltoid, supraspinatus, infraspinatus, biceps and brachialis

40
Q

What does erbs palsy look like

A

Humerus is internally rotated (unopposed subscapularis)
“waiter’s tip posture”
Physiotherapy
Prognosis good

41
Q

What is Klumpe’s palsy?

A

Lower brachial plexus palsy (C8 and T1)
Paralysis of intrinsic hand muscles +/- wrist flexors an POSSIBLE horner’s syndrome
Fingers typically flexed
(hand looks a bit like spider man but not quite)
No specific treatment
Prognosis poor

42
Q

Babinski sign

A

UMN

43
Q

Drugs which can help reduce spacticity

A

Baclofen

Botox

44
Q

Meningocele

A

This is in spina bifida

–> menignocele not usually associated with neurological sequelae

45
Q

Myelomeningocele

A

Usually has neurological deficit

46
Q

Spina bifida and the feet

A

Can cause high arched foot!!!

47
Q

A viral infection affecting motor anterior horn cells in the spinal cord or brainstem resulting in a lower motor neurone deficit

A

Polio