Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

SP16- Gen Path Exam 3 > Hereditary and Acquired Bone Diseases > Flashcards

Hereditary and Acquired Bone Diseases Flashcards

1
Q

The musculoskeletal system provides _____, _____, and ______ to the human body.

A

form
stability
movement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Musculoskeletal pathology includes diseases of what three body components?

A

bone
joints
skeletal muscle

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

True or False: Bone physiology is a tightly regulated and complex metabolism that relies heavily on calcium, osteoclasts, progenitor cells, and osteoblasts.

A

True

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the actions of OPG (osteoprotegerin) in bone physiology?

A

it is a decoy receptor for RANKL and inhibits/controls the activity of osteoclasts by limiting differentiation of precursors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

______ has become the target for osteoporosis therapy due to its actions that block formation of bone.

A

sclerostin

i.e. osteoporosis therapy = sclerostin ANTAgonist

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What percentage of the skeleton is turned over each year?

A

10%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

True or False: Osteoblasts are multinucleated.

A

False, osteoCLASTS are multi-nucleated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Which signal in bone turnover will increase osteoclast precursor differentiation?

A

RANK binding to RANKL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are the two discussed Hereditary Diseases of Bone?

A 
Osteogenesis Imperfecta (OI): Brittle Bone
Osteopetrosis: Marble Bone
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

________ is a group of hereditary bone disorders with abnormal synthesis of Type I Collagen.

A

Osteogenesis Imperfecta

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

OI is known as ______ disease and has an autosomal _____ inheritance pattern.

A

Brittle Bone

Dominant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

How does OI resemble dentinogenesis imperfecta?

A

Opalescent teeth: clinically, the two diseases cannot be deciphered based on the look of the teeth; however, OI and DI differ based on involved genes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Why do patients with OI often display a “blue sclera?”

A

decreased type I collagen

less collagen in the eyes = pigmented coroid layer shows through the thinned sclera

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

True or False: Brittle Bone disease involves a reduction in Type I collagen.

A

True, Brittle Bone = OI = Type I

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

_______ is a group of rare hereditary bone disorders with defective bone remodeling (osteoclast dysfunction).

A

osteopetrosis (marble bone disease)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What happens to the bone structure in patients with osteopetrosis?

A

new bone continues to deposit, but due to osteoCLAST dysfunction there is no removal of old bone
= bone gets thicker and more dense but it is structurally unsound (like a piece of chalk)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Why are osteopetrosis patients more prone to infections?

A

the bone marrow is replaced by osseous tissues which leads to a decrease in RBC, WBC, and Platelets (anemia, leukopenia, thrombocytopenia, pancytopenia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

How can osteopetrosis lead to blindness or deafness?

A

thickening bone leads to compression of cranial foramina

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What type of treatment is often given to patients wtih osteopetrosis?

A

bone marrow transplantation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is the most commonly acquired metabolic bone disease in the the USA? What is the second most common?

A
  1. Osteoporosis

2. Paget’s Disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

True or False: Postmenopausal women are at the greatest risk for osteoporosis.

A

True

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Osteoporosis is defined as the increased porosity of the skeleton due to reduced _______.

A

bone mass

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

____% of women will sustain fractures by age 65. _____% by age 90.

A

25

50

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

True or False: Men and women get osteoporosis

A

True (more common in females 4F:1M)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What are two endocrine disorders that could cause osteoporosis?

A

Hyperparathyroidism

Diabetes, type I

26
Q

___________ is a neoplastic condition that is characterized by abnormal plasma cells that accumulate in the bone marrow. This could undoubtedly lead to osteoporosis.

A

Multiple Myeloma

27
Q

True or False: Women typically start with a higher peak bone mass but lose bone more quickly.

A

False, women start with a lower peak bone mass

28
Q

How does age relate to osteoporosis?

A
  • decreased replication of osteoprogenitor cells
  • decreased activity of osteoblasts
  • decreased activity of growth factors
  • reduced physical activity
29
Q

Menopause contributes to osteoporosis via the increase in osteoclasts/IL1/IL6/TNF/RANK-RANKL and the decrease in ______.

A

estrogen

30
Q

Where are the most common sites affected by osteoporosis?

A

Weight-bearing bones:

  • vertebral bodies
  • pelvis
  • femoral neck
31
Q

Osteoporosis may negatively impede on the ______ system due to the bending of the vertebral column, also known as ______.

A

respiratory system

kyphoscoliosis

32
Q

Osteoporotic fractures affect more women than ____, _____, and _____ combined.

A

heart attack
stroke
breast cancer

33
Q

_____ percent of osteoporosis patients who suffer a hip fracture will die within the year.

A

20%

34
Q

What is the gold standard for diagnosing osteoporosis?

A 
DEXA Scan
(dual X-ray absorptiometry)
35
Q

What are the treatment modalities for osteoporosis?

A
  • diet/exercise modification
  • calcium supplementation with vitamin D
  • bisphosphonates
  • hormone therapy (estrogen replacement)
  • monoclonal antibody
36
Q

_____ disease is the second most common acquired bone condition. What is the alternate name for this condition?

A

Paget Disease

aka- osteitis deformans

37
Q

Which hereditary bone condition is most similar to Paget Disease?

A

osteopetrosis

-both are characterized by dense bone that is structurally weak and prone to fractures

38
Q

What is the cause of Paget Disease? Who is effected by it?

A

unknown etiology, but thought to involve paramyxovirus infection

usually affects adults >40

39
Q

True or False: Most cases of Paget disease are asymptomatic.

A

True, they are detected only via routine radiographs and blood work

40
Q

If symptoms are present, what are they?

A 
bone enlargement (dentures affected: mx>mnd.)
fractures
"growing" pains (rapid turnover)
cranial nerve compression
bowing of leg bones (Simian Stance)
increased head size (change in hat size)
41
Q

There are three phases of Paget Disease and they are ____, _____, and _____.

A
  1. Lytic
  2. Mixed
  3. Sclerotic
42
Q

______ is a symptom of Paget that is characterized by an overgrowth of the facial and cranial bones

A

Leontiasis ossea (Lion Face)

43
Q

The mosaic appearance of bone occur during which phase of Paget Disease? Is this the first, second, or third phase?

A

Sclerotic

3rd phase

44
Q

True or False: Patients with Paget’s Disease have a slight increased risk for development of osteosarcoma.

A

True, 1% lifetime (slight)

45
Q

What is the treatment for Paget’s Disease?

A

calcitonin
bisphosphonates
analgesics for pain

46
Q

Which type of hyperparathyroidism is most severe?

A

primary

47
Q

Which type of hyperparathyroidism is most common? What is its common etiology?

A

secondary

-commonly a complication of end-stage renal disease or vitamin D deficiency

48
Q

What are the actions of Parathyroid Hormone (PTH)?

A

Overall: Increase in serum calcium

  • osteoclast activation
  • increased calcium reabsorption by kidneys
  • increased vitamin D synthesis (increases GI absorption of calcium)
49
Q

In addition to osteoporosis, what is the bone condition that results from hyperparathyroidism?

A

Osteitis Fibrosa Cystica (cyst-like brown tumors of the bone)

50
Q

What is the treatment for primary hyperparathyroidism?

A

remove the affected gland/tumor

51
Q

What is the treatment for secondary hyperparathyroidism?

A

renal transplant

vitamin D supplementation

52
Q

Osteomyelitis is an _______ of the bone and ______.

A

infection

marrow

53
Q

Osteomyelitis infections are usually ______.

A

bacterial

54
Q

What are the three pathways by which bacteria can colonize bone (osteomyelitis)?

A
  1. Hematogenous Spread
  2. Contiguous Infections
  3. Implantation (following surgery or fracture)
55
Q

Which osteomyelitis-related organism is most likely to spread hematogenously?

A

Staph. aureus

56
Q

Osteomyelitis in the oral cavity is most likely to spread via which mechanism?

A

Contiguous infection (i.e. Odontogenic)

57
Q

What are the signs/symptoms of osteomyelitis?

A

pain/tenderness

overlying erythema and/or swelling

58
Q

Sequestrum is _______ bone.

A

non-vital

59
Q

Involucrum is ______ bone.

A

new (vital)

60
Q

Chronic osteomyelitis is characterized by _____ sitting inside ______.

A

nonvital (sequestrum) sitting inside a rim of new bone (involucrum)

61
Q

What is the treatment for osteomyelitis?

A

drainage
antibiotics (specific to the particular organism involved)
surgical debridement

SP16- Gen Path Exam 3 (12 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions