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18 Cards in this Set
- Front
- Back
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what are the 3 causes of paget's dz?
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idopathic
genetic (AD) viral |
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what genes are involved in paget's dz? what do these genes do?
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RANK ligand signaling--> mediates osteoclast formation.
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what age group is mostly affected by Paget's dz? At >50, which gender has a slight predominance?
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>45 yoa, Men
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what neutralizes RankL?
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osteoprotegerin
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what is the primary cellular abnormality in paget's disease?
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increased osteoclastic bone resoprtion.
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what 5 cytokines enhance osteoclast formation?
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IL-6, RANKL, TGF-B, ILGF, and FGF
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what is the pathogenesis of paget's disease? what can this lead to?
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increased osteoclastic bone resorption --> intense exuberant formation of new bone of poor quality --> vascularity of abnormal bone increase warmth--> possible CHF in elderly w/ polyostotic bone
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what percentage of the population has paget's? what happens to the incidence rate after age 50?
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2-3%, incidence doubles w/ each decade >50
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How do paget's pts present clinical?
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most- asymptomatic
some--> bone pain (skull, spine, pelvis, femur, tibia), skeletal deformity, fractures, high output cardiac failure, nerve compression syndromes |
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what are the five possible complications of paget's disease?
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1) secondary arthritis
2) hyperuricemia and gout 3) Vascular steal and high output CHF 4) sarcomatous degeneration 5) osteosarcoma |
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what is the main cause of death in Paget's?
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sarcomatous degeneration
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how do you diagnose paget's via biochem?
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biochemical markers of bone turnover:
-Alk PO4ase (new bone formation) -urinary hydroxyproline -x-linked N-telopeptides of type 1 collagen |
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how do you diagnose paget's via radiologic abnormalities?
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-bone scan
-xrays |
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what are the three stages of radiologic abnormalities in paget's?
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1) osteolytic
2) cortical thickening, coarse travecular markings and both lytic and sclerotic lesions 3) sclerotic lesions often assoc w/ increase in bone width |
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what is the DOC for paget's disease?
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Pamidronate (IV)- may be DOC
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what are the 9 indications for tx for paget's dz?
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Elevated Alk PO4ase 2x normal
skeletal deformity pain prevention of OA osteolytic lesions hearing loss radiculopathy hypercalcemia or high output CHF |
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what is a "mosiac pattern" in reference to paget's dz?
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areas of increased lucency & sclerosis
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what is "osteoporosis circumscripta" in reference to paget's dz?
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demarcated zones of lucency in frontal areas
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