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80 Cards in this Set
- Front
- Back
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Paget's occurs in what type of patients?
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older, of euro descent, >50
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this metabolic bone disease occurs in patients >50 of euro descent
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paget's
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3 phases of paget's disease, activity during
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early resorp - hyperactive o-clast
vascular phase - normal function, bone marrow replaced sclerotic phase - oclast down, oblast up |
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early resorptive phase in which oclasts are hyperactive,
a vascular phase in which bone marrow is being replaced a sclerotic phase in which oblasts are depositng scleortic bone what is this disease? |
paget's
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what is radio appeareance of pagets, and when do you notice it?
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in phase 3 (sclerotic) you see COTTON WOOL appeareance
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what disease has "cotton wool" appearance?
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paget's in stage 3 - sclerotic
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what are the serious complications of paget's diseae?
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progression to o-sarcoma or o-necrosis
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what disease must be monitored as it could turn into o-necrosis or o-sarcoma?
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paget's
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paget's
serum level... ca? phosph? alk phosph? |
ca/p normal
alk phosph elevated |
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Paget's occurs in what type of patients?
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older, of euro descent, >50
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in what disease do older patients say there teeth are starting to drift apart?
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paget's
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this metabolic bone disease occurs in patients >50 of euro descent
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paget's
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3 phases of paget's disease, activity during
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early resorp - hyperactive o-clast
vascular phase - normal function, bone marrow replaced sclerotic phase - oclast down, oblast up |
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early resorptive phase in which oclasts are hyperactive,
a vascular phase in which bone marrow is being replaced a sclerotic phase in which oblasts are depositng scleortic bone what is this disease? |
paget's
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what is radio appeareance of pagets, and when do you notice it?
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in phase 3 (sclerotic) you see COTTON WOOL appeareance
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what disease has "cotton wool" appearance?
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paget's in stage 3 - sclerotic
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what are the serious complications of paget's diseae?
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progression to o-sarcoma or o-necrosis
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what disease must be monitored as it could turn into o-necrosis or o-sarcoma?
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paget's
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paget's
serum level... ca? phosph? alk phosph? |
ca/p normal
alk phosph elevated |
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in what disease do older patients say there teeth are starting to drift apart?
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paget's
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what are 2 possible causes of paget's?
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NF-kappa B rank
Measels (PMV) |
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nf-kappa b rank and
paramyxovirus (measles) 2 possible causes of what? |
paget's
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tx of pagets?
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tx the symptoms, or bisphosphonates if secer
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what disease is this?
benign, shows by 5 yrs, regresses @ puberty, auto dominant? |
cherubism
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cherubism is auto___, and has ___% in M, __% in F, who have the loss of ____gene.
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dominant
100% in M 50-75% F Loss of SH3BP2 gene |
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loss of the SH3BP2 gene is assoc with...
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cherubism
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hwen does cherubism appear?
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by 5 yrs
regress by pubert |
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what disease has a radio of "soap-bubble" appearence?
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cherubism
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radio appearance of cherubism?
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soap bubble appearance
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what disease, histologicaly, looks like central giant cell granuloma? with MN giant cells?
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cherubusm
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what does cherubism look like histologically?
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central giant cell granuloma w/ mn giant cells
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what disease is "marble bone"
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osteopetrosis
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osteopetrosis is ____o-clasts
paget's disease is ____oclasts |
petrosis - defective
pagets - hyperactive |
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3 types of o-petrosis?
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auto recessive - infancy - fatal
intermediate auto-rec - non fatal, by 10 yrs auto dominant - adult - norma llife span |
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what is the worst kind of o-petrosis, auto dom or recessive?
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recessive
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the bone in osteopetrosis is 2 things...
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thicker and weaker
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what are oral problems of o-petrosis and why?
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bone fails to resorb so you get lack of eruption or premature exfoliation due to a bad PDL
also hypoplastic teeth |
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what is a serious complication of osteopetrosis?
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osteomyelitis
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osteomyelitis is a serious complication of what?
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osteopetrosis
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What looks like "bone in bone" radiographically?
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osteopetrosis
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cleidocranial dysplasia is auto___, is more common in (M/F) and most common in (race)
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dominat
m=f all race equal |
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this disease has lack of collarbones and open soft spots
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cleidocranial dysplasia
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which genes are involved in cleidocranial dysplasia?
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RunX2, CbFA1
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runx2, cbfa1 genes involved in...
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cleidocranial dysplasia
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which syndrome of bone as issues with MR?
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crouzons
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what is wrong in patients with crouzons?
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the skull fuses prematurely = increased pressure
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what idsease is this
premature skull fusion, increased brain pressure = MR |
crouzons' syndorme
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genes in Crouzons
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FGFR2/3
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FGFR2/3 involved in
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crouzons'
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buldging eyes, hypoplastic midface are charactersistc of...
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crouzons
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crouzon's apperance
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droopy nose
buldging eyes hypoplastic midface |
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radio apperance of crouzons?
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hammerhead-silver apperacnce
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what disease has a radiograph showing hammerhead silver appearance?
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crouzons
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treacher collin's syndrome affects what structures?
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1st and 2nd branchial arches
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what disease effects 1st and 2nd branchial arches?
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treacher collins
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What disease involves the TcOF1 gene?
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treacher collins
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treacher collins involves what gene?
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tcof1
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what disease gives pts a convex profile, prominent nose, retrusivechin, HEARING LOSS< downward eyes
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treacher collins
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% of overall
CP - CL - CL + CP - |
CP - 30%
CL - 25% CL + CP = 45% |
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CL + CP
live births? race? % unitlateral? % part of a syndrome? |
1 in 700-1000 live births
as>wh>black 80% unilateral 30% part of a syndrome |
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what abnormality is this
1 in 700-1000 live births as>wh>black 80% unilateral 30% part of a syndrome |
cl + CP
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CP only
live births? sex? race? |
1 in 1500-3000 births
F>M race all equal |
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what abnormality is this?
1 in 1500-3000 births F>M race all equal |
CP only
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van der woude syndrome, related to what facial abnormality?
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CL + CP
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x-linked, related to what facial abnormality?
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CP only
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ectodermal dysplasia, related to what facial abnormality?
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CL and CP
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what syndrome was first discovered on margarita island, is assoc with CP and CL, and has pts who are missing hteir middle digits?
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ectodermal dysplasia
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PVRL1 gene is assoc with what syndrome?
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ectodermal dysplasia
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This syndrome results in CP, involves the TBOX22 gene, and also gives patients ankyloglossia
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x-linked CP
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whta abnormality involves the TBOX22 gene?
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x-linked cp
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x-linked CP involves what gene?
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TBOX22
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ectodermal dysplasia involves what gene?
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PVRL1
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van der woude involves what gene?
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IRF6
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IRF6 is involved in what facial abnormality?
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van der woude
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3 vitamins assoc with cl/cp?
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folate
riboflavin vit a |
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3 drugs assoc with cl/cp
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retinoids (accutane)
methotrexate (cancer) dilantin (seizure) |
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what syndrome has associated paramedin lip pits?
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van der woude
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2 things with van der woude
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paramedian lip pits and cl/cp
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what syndrome has associated paramedin lip pits?
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van der woude
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2 things with van der woude
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paramedian lip pits and cl/cp
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