[PATH] Bones, Joints and Soft Tissue (Part 1 BONE) [Martin] Flashcards

1
Q

This image depicts what type of process?

A

Endochondral ossification

(in a calcaneus)

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2
Q

Root word association:

Osteoporosis =

Osteomalacia =

Osteopetrosis =

Osteogenesis imperfecta =

Osteitis deformans =

Osteodystrophy =

A

Osteoporosis = Porous; thinned out bone

Osteomalacia = Weakened, soft bone

Osteopetrosis = Peter=rock, stone bone

Osteogenesis imperfecta = Imperfect origin of bone

Osteitis deformans = Deforming bone-itis

Osteodystrophy = Bad growing bone

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3
Q

What are the (3) components of bone matrix?

A

Type I collagen

Osteoid

Mineral component

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4
Q

Hardness of bones is due to?

A

Hydroxyapatite

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5
Q

What are the (2) histologic forms of bone?

A

Woven

Lamellar

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6
Q

Characteristics of woven bone?

A

Disorganized

Produced rapidly ; fetal development and fracture repair

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7
Q

Characteristics of lamellar bone?

A

Slow production

Parallel collagen

Strong

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8
Q

Function of osteoblasts

A

Synthesize, transport and assemble matrix

*Regulates mineralization

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9
Q

Function of osteocytes?

A

Inactivate osteoblasts

Control calcium and phosphate levels

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10
Q

What are osteoclasts?

Function?

A

Multinucleated macrophages derived from circulating monocytes

Bone resorption

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11
Q

What type of bone is associated with:

Endochondral ossification?

Intramembranous ossification?

A

Endochondral ossification = Long bones

Intramembranous ossification = Flat bones

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12
Q

When is peak bone mass achieved?

A

Early adulthood

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13
Q

RANK

Where is it expressed?

Function?

A

Where is it expressed = Osteoclast precursors

Function = Breakdown

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14
Q

RANK-L

Where is it expressed?

Function?

A

Where is it expressed = Osteoblasts and marrow stromal cells

Function = Breakdown

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15
Q

OPG

Where is it expressed?

Function?

A

Where is it expressed = “Decoy” receptor made by osteoblasts that can bind RANKL and prevent it’s interaction with RANK

Function = Build

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16
Q

A product produced by osteocyte inhibits the WNT/Beta-catenin pathway. What is it?

A

Sclerostin

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17
Q

What are the common building circulating hormones/factors?

A

Estrogen

Testosterone

Vit D

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18
Q

What are the common breakdown circulating hormones/factors?

A

PTH

IL-1

Glucocorticoids

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19
Q

What is M-CSF?

A

Monocyte colony stimulating factor

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20
Q

Function of M-CSF?

A

M-CSF receptor on osteoclast precursors STIMULATES tyrosine kinase cascade that is crucial for generation of osteoclasts

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21
Q

What are the (3) primary developmental disorders of bone?

A

Osteogenesis imperfecta

Osteopetrosis

Mucopolysaccharidoses

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22
Q

What is commonly associated with developmental disorders of bone?

A

Transcription factor defects: specifically homeobox genes

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23
Q

Define:

Dysplasia

A

Global disorganization of bone &/or cartilage

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24
Q

Define:

Syndactyly/Craniosynostosis

A

Abnormal fusion of bones

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25
Q

What are wormian bones?

A

Extra bones within cranial sutures

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26
Q

What is an excellent way to determine the origin of a dwarfism pathology?

Can you give examples?

A

Look at the chest cavity!

Normal trunk length with achondroplasia

Small chest cavity with thanatophoric dysplasia

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27
Q

Achondroplasia

Gene mutation?

Presentation?

Mortality?

A

Gene mutation = Autosomal dominant FGFR3 gain of function

Presentation = Short extremities, normal trunk length, enlarged head, bluging forehead, depression root of nose

Mortality = NO change in longevity, intelligence or reproductive status

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28
Q

Thanatophoric dysplasia

Gene mutation?

Presentation?

Mortality?

A

Gene mutation = FGFR3 gain of function

Presentation = Small chest cavity, bell-shaped abdomen, micromelic shortening of limbs

Mortality = Die at birth or soon after

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29
Q

Osteogenesis imperfecta:

Which type has the best prognosis?

Worst?

A

Best = Type I

Worst = Type 2

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30
Q

Prognosis of Type II osteogenesis imperfecta?

A

Uniformly fatal in utero

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31
Q

Buzz word association for osteogenesis imperfecta type 2?

A

“Accordion-like” shortening of the limbs

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32
Q

What are the major clinical features of osteogenesis imperfecta?

A

Blue sclera

Skeletal fragility

Dentinogenesis imperfecta (small teeth)

Hearing impairment

Joint laxity

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33
Q

What is the most common type of osteogenesis imperfecta?

A

Type I

Also the mildest type of OI

34
Q

Why is type II typically fatal?

A

Respiratory problems

(They have underdeveloped lungs from small thoracic cage)

35
Q

What is the difference in sclera appearance b/w Type III and Type IV OI?

A

Type III = Blue, purple or gray tint

Type IV= White

*Remember; The order of best prognosis to worst is (1–> 4 –>3–>2) because fuck, lets make this difficult. But yeah, remember that type 4 is less severe, so the sclera are going to be normal in color.

36
Q

Osteopetrosis

Aka?

(2 names)

A

Marble bone disease

Albers-Schonberg disease

37
Q

What is the deficiency in osteopetrosis?

A

Carbonic anhydrase 2 (CA2)

38
Q

How does osteopetrosis appear on Xray?

Buzz word association?

A

Erlenmeyer flask

39
Q

Inheritance pattern for osteopetrosis?

Populations?

A

Autosomal recessive

Mediterranean and Arab race

40
Q

Common clinical features of osteopetrosis?

A

Cranial nerve deficits:

Optic atrophy, deafness, facial paralysis

41
Q

What is seen on histology with osteopetrosis?

A

No medullary cavity

42
Q

What type of disease is mucopolysaccharidoses?

A

Lysosomal storage disease

43
Q

What are examples of aquired bone disorders (5)?

A

Osteopenia/osteoporosis

Pagets

Osteomalacia/rickets

Hyperparathyroidism

Renal osteodystrophy

44
Q

Define osteopenia

Define osteoporosis

A

Osteopenia = 1.0 - 2.5 standard deviations below the mean

Osteoporosis = At least 2.5 SD below the mean peak bone mass in young adults

45
Q

If your patient has a bone mass that is less than 2.5 SD below the mean, but you still diagnose them with osteoporosis, what MUST have happened?

A

Atraumatic

or

Vertebral compression fractures

46
Q

What population is at risk of never obtaining their full potential bone mass due to eating disorders?

A

Adolescent girls

(Insufficienct calcium intake during period of rapid growth restricts their peak bone mass)

47
Q

What is a major hormonal influence on osteoporosis?

A

Menopause

(estrogen deficiency)

48
Q

Describe the appearance of osteoporotic bone

A

(Right)

Loss of horizontal trabeculae

49
Q

What is the method of diagnosis for osteoporosis?

A

DEXA-scan

50
Q

Risk factors for osteoporosis?

A

Caucasian, light colored eyes and skin

Nutrition (too much phosphorus-soda)

Smokers

51
Q

What spinal changes are associated with loss of height in osteoporosis?

A

Lumbar lordosis & kyphoscoliosis

52
Q

Paget disease

aka?

A

Osteitis deformans

53
Q

Paget disease

Characteristsics?

Gene?

A

Polyostotic (involves multiple bones)

70yo average age

Caucasians

SQSTM1 gene

54
Q

Paget disease

Buzz word association with Xray appearance?

A

“Cotton Wool”

55
Q

What is the paget disease morphology?

A

Mosaic pattern of lamellar bone

Jigsaw-like

56
Q

What type of fractures are associated with paget disease?

A

Chalk stick type fractures

57
Q

What are the lab values associated with paget disease?

A

Elevated ALK PHOSPHATASE

NORMAL = Calcium and phosphorous

58
Q

Name a unique clinical feature of paget disease

A

Hyeprvascularity of paget disease

Warms the overlying skin

–> Can lead to high output heart failure

59
Q

Rickets and osteomalacia are manifestations of?

A

VITAMIN D DEFICIENCY

Or it’s abnormal metabolism

60
Q

What is the histology associated with hyperparathyroidism?

A

Train tracks

61
Q

Define:

Simple fracture

A

Overlying skin intact

62
Q

Define:

Compound fracture

A

Bone communicates with the skin surface

63
Q

Define:

Comminuted fracture

A

Bone is fragmented

64
Q

Define:

Displaced fracture

A

Ends of the bone at the fracture site are not aligned

65
Q

Define:

Stress fracture

A

Slowly developing fracture that follows a period of increased physical activity in which bone is subjected to repetitive loads

nice.

66
Q

Define:

Greenstick fracture

A

Extending only partially through the bone, common in infants when bones are soft

67
Q

Define:

Pathologic fracture

A

Involving bone weakened by an underlying disease process such as a tumor

68
Q

What is osteonecrosis?

A

Infarction of the bone and marrow

(pretty common)

69
Q

What causes most cases of osteonecrosis?

A

Fractures

or

Corticosteroid tx

70
Q

What region is typically not affected in osteonecrosis?

Why?

A

Cortex is not affected

Due to collateral flow

71
Q

Buzz word association with osteonecrosis?

A

“Creeping substitution”

72
Q

What is osteomyelitis?

A

Inflammation of the bone and marrow

Typically following infection

73
Q

What are most cases of osteomyelitis caused by?

A

Pyogenic bacteria

&

Mycobacteria

74
Q

How does pyogenic osteomyelitis typically spread in:

Kids?

Adults?

A

Kids = Hematogenous

Adults = Complication of open fractures, surgical procedures and diabetic infections

75
Q

Most common cause of pyogenic osteomyelitis?

A

Staph aureus (80-90%)

76
Q

Osteomyelitis:

Name the associated organism with:

Sickle cell patients

A

Salmonella

77
Q

Osteomyelitis:

Name the associated organism with:

MAC deficiency

A

Neisseria

78
Q

What is:

Sequestrum?

Involucrum?

A

Sequestrum = Dead bone following abscess

Involucrum = New bone

79
Q

What is the histology associated with mycobacterial osteomyelitis?

A

Caseous necrosis and granulomas

80
Q

What is Pott disease?

A

Aka Tuberculous spondylitis

Permanent compression fractures

81
Q

What are the associated bone deformities seen with skeletal syphilis?

A

Saber shin

Saddle nose