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Bone > Dougherty Part 1 > Flashcards

Dougherty Part 1 Flashcards

1
Q

RANK receptor is found on what and what does it allow for

A

osteoclast precursor

allow for osteoclast generation and survival

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2
Q

RANKL is found on what

A

osteoblast and marrow stroma cells

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3
Q

M-CSF is secreted by what

A

osteoblast

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4
Q

MCSF receptor is found on what and allows for what

A

osteoclast

allows for osteoclast generation and survival

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5
Q

WNT is secreted from what

A

marrow stromal cells

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6
Q

LRP5 and LRP6 are found on what and bind what and do what

A

osteoblast
bind WNT protein
activate beta-catenin
secrete osteoprotegrin (OPG)

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7
Q

what does osteoprotegrin (OPG) do

A

blocks RANK

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8
Q

from what and when is osteoprotegrin (OPG) secreted

A

from osteoblasts when WNT has bound LRP5 and LRP6

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9
Q

the organic matrix of bone composition is mostly made up of what

A

Type 1 collagen

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10
Q

what are the types of bone

A 
woven bone (random collagen deposition)
lamellar bone (ordered collagen deposition)
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11
Q

what are the types of lamellar bone

A 
compact bone
spongy bone (calcinous)
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12
Q

levels of what parallel osteoblast activity

A

osteopontin (osteocalcin)

unique to bone

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13
Q

alkaline phosphatase is secreted from what

A

osteoblasts

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14
Q

osteopontin activity is high in what

A

growing kids

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15
Q

what type of bone resists forces in all directions

A

woven bone

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16
Q

what bone has rapid growth and is found in healing fracture

A

woven bone

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17
Q

woven bone in an adult is what

A

pathologic

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18
Q

what is intramembranous ossification

A

bone formation direct form mesenchyme

appositional growth

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19
Q

what is dysostosis

A

local problems in migration of mesenchyme and their condensation

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20
Q

dysplasia is what

A

global defect in regulation of skeletal organogenesis

all bones are affected

21
Q

cleidocranial dysplasia has what pattern of inheritance and the defect

A

autosomal dominant

RUNX2 (CBFA1) transcription factor defect

22
Q

what is seen in cleidocranial dysplasia

A

short stature
abdominal clavicles
supernumerary teeth
Wormian bone

23
Q

Wormian bone is found in what

A

cleidocranial dysplasia

24
Q

achondroplaisa is a defect in what and has what inheritance pattern

A

FGFR3 point mutation- gain of function that inhibits cartilage growth
autosomal dominant
most common from paternal allele

25
Q

achondroplaisa is what type of defect

A

growth plate defect from paracrine cell defect

26
Q

what is seen in achondroplasia

A 
short stature
short proximal limbs
normal trunk length
enlarged head with building forehead
depression root of nose
normal longevity, intelligence, and reproductive status
27
Q

what is the defect in thanatophoric dwarfism is a defect in what

A

FGFR3 mutation- gain of function

28
Q

what is seen in thanantophoric dwarfism

A 
cloverleaf skull
micromelic short bowed limbs
frontal bossing with macrocephaly
small underdeveloped chest with bell-shaped abdomen
diminished chondrocyte proliferation
29
Q

a gain of function in LRP5 is seen in what, with what inheritance pattern, and what happens

A

Osteopetrosis Type 1
autosomal dominant
cannot up regulate osteoclasts

30
Q

Osteoporosis pseudoglioma syndrome is due to what

A

inactive LRP5

31
Q

what is seen in osteoporosis pseudoglioma syndrome

A

skeletal fragility

loss of vision

32
Q

what is “marble bone disease”

A

osteopetrosis

33
Q

osteopetrosis is due to what

A

reduced osteoclast bone resorption:
cannot acidly pit (carbonic anhydrase II (CA2) deficiency
defect in RANKL
LRP5 gain of function

34
Q

erlenmeyer flask deformity (bulbous long bones) is seen in what

A

osteopetrosis

35
Q

what is seen in osteopetrosis

A

bone deposition replaces medullary cavity (extra medullary hematopoiesis)
bulbous long bones (erlenmeyer flask deformity)
narrow neural foramina
brittle bones

36
Q

what is the spectrum of osteopetrosis

A

autosomal dominant benign to autosomal recessive “malignant”

37
Q

osteogenesis imperfecta aka brittle bone disease is due to what

A

Type 1 collagen

mutation of alpha1 or alpha 2 chains

38
Q

Osteogenesis Imperfecta Type I is what inheritance pattern and what is seen

A

autosomal dominant
normal stature with less fractures after puberty
blue sclerae from translucency of sclera
hearing loss
dentinogenesis imperfect from dentin defect

39
Q

osteoporosis is what

A

increased bone porosity and decreased mass

40
Q

what causes primary metabolic bone disease

A

senile
postmenopausal
idiopathic

41
Q

what in menopause leads to osteoporosis

A

increased expression of RANK, RANKL

42
Q

secondary metabolic bone disease is caused by what

A 
drugs
diabetes
endocrine disorders
malignancy liver
GI disease
43
Q

what is the difference b.w osteopenia and osteoporosis

A

osteopenia- decrease bone mass

osteoporosis- osteopenia to the point of risk of fracture

44
Q

what is the low turnover variant of osteoporosis

A

senile osteoporosis

45
Q

what is the high turnover variant of osteoporosis

A

postmenopausal osteoporosis

46
Q

postmenopausal osteoporosis is caused by what

A

decreased estrogen leads to increased inflammatory cytokines
this increases RANKL and decreases OPG
osteoclast activity increases more than osteoblast

47
Q

how do you detect osteoporosis

A

Dual-energy absorptiometry

48
Q

in osteoporosis the cortex of the bone resembles what

A

cancellous bone

Bone (19 decks)

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