Pagets disease Flashcards
Where is a high incidence of pagets disease
UK, Aus, NZ (not common in NA)
What is the pathophysiology of pagets disease
- increased osteoclastic activity (creates lytic areas in bone)
- Osteoblastic response (disordered bone trabeculae)
- increased HR and CO due to highly vascularized tissue
Distribution of pagets in body
Likes axial skeleton (~30-60%) Long bones (25-30%)
Is pagets more commonly monostotic or polyostotic
m/c polyostotic and more common on r side of body
Clinical s/s of pagets and how commonly is it asymptomatic
80-90% asymptomatic
- may have neuro compromise if in spine
- May have dull pain
- expansion of bone
Clinical features of pagets
- Increased bone size
- Increased cardiac output
- may have ear ossicle involvement causing deafness
4 phases of paget on xray
- Lytic- well marginated lucent lesions
- Mixed- Lytic areas + thick cortices, coarse trabeculea, expansion of bone + softening
- Sclerotic- purely opaque, expanded, bone softening
- Maligant- cortical destruction, soft tissue mass (usually lytic)
Lab findings of pagets
- Increased alkaline phophatase
- Increased urinary hyroxyprolene
When will a bone scan be cold in pagets
in malignant pagets (usually is hot on bone scan)
Tx of pagets (meds + management)
Diphosphonates + mithramycinates arest the proccess
-Supportive bracing of abnormal bone due to softening
What is another name for the mixed phase of pagets
cotton wool appearence
What is tam oshanter sign
When the shape of the skull becomes flatteneded due to bone softening
What will you see in the mixed phase of pagets
Thickening of trabecule/ unorganized
expansion of bone
mixed lytic areas
What is a picture frame vert and what phase does it mean
Cortical thickening of vert body; some expansion
-signals mized phase
What could ivory vert be due to and how to differentiate it
blastic mets
hodkins
pagets: Expansion, >40
