Bones 1 Flashcards

1
Q

what are the 3 types of cell in bone ? how many nuclei? basic multicellular unit

A

osteoclasts ( multiple_
osteoblasts (single nucleus)
osteocytes

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2
Q

RANKL located on what cell

A

osteoblasts and marrow stroma cells

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3
Q

RANK is located on what cell? role?

A

osteoclast precursor

- allow OC generation and survival

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4
Q

explain the M-CSF pathway

A
  1. M-CSF secreted by OB

2. M-CSF binds to OC: generation and survival

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5
Q

explain the WNT/Beta-catenin pathway

A
  1. WNT from marrow stromal cell
  2. LRP5 and LRP6 OB receptor bind WNT protein
  3. OB secretes OPG ( blocks RANK)
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6
Q

bone compositition

A

calcium hydroxyapatite

organic matrix with type I collagen

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7
Q

what are the two types of bone? where are they found

A

woven bone: random collagen
lammellar bone: ordered collagen
compact bone
spongy bone

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8
Q

name two bone enzymes

A

osteopontin

alkaline phosphatase

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9
Q

role of osteopontin ( osteocalcin)

A

levels parallel osteblast activity

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10
Q

alkaline phosphatase comes from what organ

A

osteoblasts

liver and placenta

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11
Q

woven bone?
how fast does it grow?
forces?

A

rapid bone growth
resists forces in all directions
pathologic in adults

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12
Q

lamellar bone replaces what

A

woven bone

stronger than woven bone

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13
Q

what process forms bone?

A

intramembranous ossification

  • direct from mesenchyme
  • appositional growth
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14
Q

location of epiphysis

A

distal to growth plate

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15
Q

location of metaphysis

A

beneath growth plate

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16
Q

location of diaphysis

A

center

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17
Q

Dysostosis

A

local problems in migration of mesenchyme and their condensation

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18
Q

dysplasia

A

global defect in regulation of skeletal organogenesis

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19
Q

Cleidocarnial dysplasia

  • inherited
  • defect protein
A

autosomal dominant

-RUNX2 (CBFA1)

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20
Q

clincial presentation for Cleidocranial dysplasia

A

short
abnormal clavicle
supernumery theeth
wormian bone ( extra sutural bone)

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21
Q

what goes wrong in achondroplasia

A
  • growth plate defect from paracrine cell defect

- reduced chondrocyte proliferation in growth plate

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22
Q

achondroplasia is a defect in what gene? what does it cause

A

FGFR3 point mutation

-inhibits cartilage growth

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23
Q

how is achondroplasia passed on to generations

A

paternal allele

autosomal dominant

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24
Q

what growth is still present for achondroplasia? what does this cause

A

appositional and intramembranous bone formation

-thick cortical bone

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25
clinically what does achondroplasia look like
``` short proximal limbs normal turnk enlarged head depression of root nose normal reproductive and intelligence ```
26
what is the most common lethal dwarfism
Thanatophoric dwarfism
27
thanatrophoric dwarfism is a mutation in what
FGFR3
28
clinical presentation of thanatophoric dwarfism
cloverleaf leaf | bell-shaped abdomen
29
LRP5 is a receptor that activates what
WNT/beta-catenin in OB | - produces OPG (Blocks RANKL)
30
what disease has an inactive LRP5
osteoporosis pseudoglioma syndrome - skeletal fragility - loss of vision
31
Marble bone disease
osteopetrosis
32
what goes wrong in osteopetrosis
reduced osteoclast bone resorption - cannot acidify pit - defect in RANKL LRP5 gain of function
33
in osteopetrosis bone deposition replaces what
medullary cavity | - no room for hematopoiesis
34
what does osteopetrosis look like on X-ray
Erlenmeyer flask
35
how is osteopetrosis passed down
autosomal dominant
36
brittle bone disease
osteogenisis imperfecta
37
Osteogensis imperfecta what goes wrong
type 1 collagen disease - mutation of alpha 1 and 2 chains extreme skeletal fragility
38
what is the most common type of autosomal dominant bone disease
osteogenisis imperfecta
39
clinical features of Autosomal dominant osteogensis imperfecta
blue sclera hearing loss dentin defect
40
Type 2, 9, 10, 11 collagen disease has a defect in what?
hyaline cartilage | -
41
Mucopolysaccharidoses is a defect in what
enzymes degrading dermatan sulfate, heparan sulfate, keratan sulfate - abnormal hyaline cartilage - malformed bone
42
what is osteoporosis
increase bone porosity | decrease mall
43
primary osteoporosis
senile, postmenopausal
44
secondary osteoporosis
drugs, diabetes,
45
compare osteoporosis and osteopenia
osteopenia: decrease bone mass osteoporosis: osteopenia to point of risk of fracture
46
senile osteoporosis what happens to the osteoblasts? cortex?
reduce metabolism | cortex is thinned
47
when is skeletal mass peak for senile osteoporosis
young adult
48
in postmenopausal osteoporosis what happens when estrogen is decreaed
increase inflammatory cytokines increase RANKL decrease OPG OC>OB
49
rickets
child vitamin D deficiency
50
osteomalacia
adult vitamin D deficiency
51
renal osterodystrophy
chronic renal disease - increase or decreased OC/OB activity - decrease Vitamin D conversion
52
what happens to OC and OB in hyperparathyroidsim
more OC
53
what does the X-ray look like for hyperparathyroidsim
bone loss radial aspect of middle phalange of index and middle finger -osteopenia
54
Paget disease involve what bone
axial skeleton | proximal femur
55
what are the 3 stages of paget disease
osteolytic stage mixed stage osteosclerotic stage
56
osteolytic stage
loss of bone mass
57
mixed stage
osteolytic and osteoblastic
58
osteosclerotic stage
jigsaw puzzle-like cement lines of lamellar bones
59
paget disease has an increase in what enzyme? what values stay the same
alkaline phosphatase | normal Ca and PO4
60
Paget disease: pain overgrowth can lead to
cranial nerve palsy | heavy skull
61
what happens to the skin in Paget disease
warm skin over affected bone with hypervasculatiry | -high-output cardiac failure
62
treatment for Paget disease
Calitonin and biphosphonates
63
bony callus
woven bone | -overtime can bear weight
64
Soft tissue callus
easily disrupted hematoma fibrin osteoprogentor cells activated
65
peak bone mass
early adulthood
66
how can a genetic factor cause osteoprosis
vit D receptor polymorphism
67
is an X-ray and serum levels of calcium, phosphorous, alkaline phosphate helpful in diagnosing osteoporosis
NO
68
osteitis fibrosa cystica
severe form of parathyroidsm
69
pseudoarthrosis
non union
70
osteonecrosis
infarction of bone marrow
71
most common cause of osteonecrosis
corticosteroid
72
in osteonecrosis what is dead bone/fat replaced by
Ca soaps
73
name 5 things that can cause avascular necrosis
``` corticosteroid pregnancy infection dysbarism sickle cell disease ```
74
subchondral infarct what doe sit look like
wedge-shaped subchondral bone
75
osteomyelitis
inflammation almost always from infection
76
how does bacteria reach bone
hematogenous direct implantation
77
X-ray for osteomyelitis
lytic bone lesion with surrounding sclerosis
78
most common bacteria in pyogenic osteomyelitis
s. aureus
79
most common bacteria in pyogenic osteomyelitis for infants
group B | H. influencza
80
where does pyogenic osteomyeltissi occur in bone in neonate children adult
neonate: metaphysis/epophysis children: metaphysis adult: epiphysis and subchondral bone
81
sequestrum
dead piece of bone
82
brodie abscess
small intraosseous abscess often in cortex walled off by reactive boone
83
involucrum
reactive surrounding bone
84
process of pyogenic osteomyelitis
sequestrum chronic inflammation : brodie abcxess involucrum
85
congential syphilis
at enchondral ossification centers and periosteum
86
acquired syphilis of bone? what type of bone and clinical presentsion
bone in tertiary phase | saddle nose, saber shin ( tibia)