Pagets disease of the bone Flashcards

1
Q

underlying problem

A

increased bone turnover due to overactive osteoblasts and osteoclasts

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2
Q

is the excessive turnover coordinated? result

A

no

patchy areas of high density and low density

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3
Q

what happens to the bones?

A

enlarged and misshapen

prone to pathological fractures

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4
Q

what bones are affected?

A

axial

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5
Q

presentation

A

bone pain
bone deformity
fractures
hearing loss

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6
Q

Key investigations

A

x-ray

bloods - alkaline phosphatase, calcium and phosphate

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7
Q

x-ray findings

A

bone enlargement and deformity
cotton wool appearance of skull
v shaped defects in long bones
osteoporosis circumscripta

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8
Q

what blood marker is increased?

A

alk phos

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9
Q

main treatment

A

bisphosphonates

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10
Q

other treatment

A

NSAIDs
calcium and vitamin D
surgery
physio, orthotics, aids

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11
Q

monitoring

A

serum ALP

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12
Q

Two key complications

A

osteogenic sarcoma - osteosarcoma

spinal stenosis and SCC

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13
Q

osteosarcoma

A

poor prognosis

increased focal bone pain, bone swelling or #

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14
Q

spinal stenosis

A

narrowing of canal
MRI scan treat with bisphosphonates
surgery

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15
Q

neurological symptoms

A
hearing loss
chronic facial pain 
hydrocephalus 
peripheral nerve entrapment
spinal stenosis
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16
Q

only confirmatory test

A

bone biopsy

17
Q

Risk factors

A

FH
age over 50
male