Paget's Flashcards
what is the typical presentation of paget’s?
Bone pain (gradual), nerve compression (sciatica, hearing loss), bone bossing, kyphosis, warm skin over painful area (don’t fit into shoes or hats) - norm U&Es but high ALP (serum CTX and P1NP)
Define Pagets
A chronic bone disorder that is characterised by focal areas of increased bone remodelling, resulting in overgrowth of poorly organised bone. This unbalanced process may lead to osseous deformities, altered joint biomechanics, nerve compressions, and pathological fractures.
what is the inheritance pattern of paget’s?
autosomal dominant
outline the aetiology/ risk factors of Paget’s?
unknown
family history= autosomal dominant
viral infection?
more than 55 years old
outline the pathophysiology of Paget’s?
Localised area of metabolic hyperactive bone
Abnormally large osteoclasts with XS nuclei => large resorption pits and cavities
osteoblast activity is increased so that the newly formed bone don’t have time to organise properly
Newly formed bone is less resistant and more elastic => deformities + microfractures
what are the 3 phases of the evolution of paget’s?
- An initial, short-livedburst of multinucleate osteoclastic activitycausing bone resorption
- A mixed phaseof both osteoclastic and osteoblastic activity, with increased levels of bone turnover leading to deposition of structurally abnormal bone
- A final chronic sclerotic phase, during which bone formation outweighs bone resorption.
Summarise the epidemiology of Paget’s disease of bone
Majority are sporadic cases
Some have a FHx
Mean age of onset is 55yrs
what are the presenting symptoms and signs of Paget’s?
Asymptomaticin most patients – usually incidental finding
Pelvis, long bones, and skullare most common sites of abnormal bone
May cause back pain
Fractures
Frontal bossing– increase the size of their headwear
Prognathism- affected patients note progressive enlargement of the mandible
Increased local temp
If skull involvement => facial pain and hearing loss
what are the appropriate investigations for Paget’s and what do the results show?
X-rays showing lytic changes, occasionally fractures, scleroticpicture (later stages)
Bone scans – but non-specific- highlights areas of stress and microfractures
Total ALP – may be elevated or normal
Bone-specific ALP– much more specific and is used as an index for treatment response
P1NP and CTX- used as a marker for bone formation and index for treatment response- initally elevated, treatment may normalise values
Vit D levels should be normal– used to differentiate this from osteomalacia and fibrous dysplasia
LFTs- monitor for liver disease causing increase in ALP