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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

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2
Q

Musculoskeletal pathology includes diseases of what three body components?

A

bone
joints
skeletal muscle

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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

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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

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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

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6
Q

What percentage of the skeleton is turned over each year?

A

10%

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7
Q

True or False: Osteoblasts are multinucleated.

A

False, osteoCLASTS are multi-nucleated

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8
Q

Which signal in bone turnover will increase osteoclast precursor differentiation?

A

RANK binding to RANKL

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9
Q

What are the two discussed Hereditary Diseases of Bone?

A 
Osteogenesis Imperfecta (OI): Brittle Bone
Osteopetrosis: Marble Bone
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10
Q

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

A

Osteogenesis Imperfecta

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11
Q

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

A

Brittle Bone

Dominant

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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

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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

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14
Q

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

A

True, Brittle Bone = OI = Type I

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15
Q

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

A

osteopetrosis (marble bone disease)

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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)

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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)

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18
Q

How can osteopetrosis lead to blindness or deafness?

A

thickening bone leads to compression of cranial foramina

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19
Q

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

A

bone marrow transplantation

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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

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21
Q

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

A

True

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22
Q

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

A

bone mass

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23
Q

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

A

25

50

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24
Q

True or False: Men and women get osteoporosis

A

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

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25
What are two endocrine disorders that could cause osteoporosis?
Hyperparathyroidism | Diabetes, type I
26
___________ is a neoplastic condition that is characterized by abnormal plasma cells that accumulate in the bone marrow. This could undoubtedly lead to osteoporosis.
Multiple Myeloma
27
True or False: Women typically start with a higher peak bone mass but lose bone more quickly.
False, women start with a lower peak bone mass
28
How does age relate to osteoporosis?
- decreased replication of osteoprogenitor cells - decreased activity of osteoblasts - decreased activity of growth factors - reduced physical activity
29
Menopause contributes to osteoporosis via the increase in osteoclasts/IL1/IL6/TNF/RANK-RANKL and the decrease in ______.
estrogen
30
Where are the most common sites affected by osteoporosis?
Weight-bearing bones: - vertebral bodies - pelvis - femoral neck
31
Osteoporosis may negatively impede on the ______ system due to the bending of the vertebral column, also known as ______.
respiratory system | kyphoscoliosis
32
Osteoporotic fractures affect more women than ____, _____, and _____ combined.
heart attack stroke breast cancer
33
_____ percent of osteoporosis patients who suffer a hip fracture will die within the year.
20%
34
What is the gold standard for diagnosing osteoporosis?
``` DEXA Scan (dual X-ray absorptiometry) ```
35
What are the treatment modalities for osteoporosis?
- diet/exercise modification - calcium supplementation with vitamin D - bisphosphonates - hormone therapy (estrogen replacement) - monoclonal antibody
36
_____ disease is the second most common acquired bone condition. What is the alternate name for this condition?
Paget Disease | aka- osteitis deformans
37
Which hereditary bone condition is most similar to Paget Disease?
osteopetrosis | -both are characterized by dense bone that is structurally weak and prone to fractures
38
What is the cause of Paget Disease? Who is effected by it?
unknown etiology, but thought to involve paramyxovirus infection usually affects adults >40
39
True or False: Most cases of Paget disease are asymptomatic.
True, they are detected only via routine radiographs and blood work
40
If symptoms are present, what are they?
``` 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
There are three phases of Paget Disease and they are ____, _____, and _____.
1. Lytic 2. Mixed 3. Sclerotic
42
______ is a symptom of Paget that is characterized by an overgrowth of the facial and cranial bones
Leontiasis ossea (Lion Face)
43
The mosaic appearance of bone occur during which phase of Paget Disease? Is this the first, second, or third phase?
Sclerotic | 3rd phase
44
True or False: Patients with Paget's Disease have a slight increased risk for development of osteosarcoma.
True, 1% lifetime (slight)
45
What is the treatment for Paget's Disease?
calcitonin bisphosphonates analgesics for pain
46
Which type of hyperparathyroidism is most severe?
primary
47
Which type of hyperparathyroidism is most common? What is its common etiology?
secondary | -commonly a complication of end-stage renal disease or vitamin D deficiency
48
What are the actions of Parathyroid Hormone (PTH)?
Overall: Increase in serum calcium - osteoclast activation - increased calcium reabsorption by kidneys - increased vitamin D synthesis (increases GI absorption of calcium)
49
In addition to osteoporosis, what is the bone condition that results from hyperparathyroidism?
Osteitis Fibrosa Cystica (cyst-like brown tumors of the bone)
50
What is the treatment for primary hyperparathyroidism?
remove the affected gland/tumor
51
What is the treatment for secondary hyperparathyroidism?
renal transplant | vitamin D supplementation
52
Osteomyelitis is an _______ of the bone and ______.
infection | marrow
53
Osteomyelitis infections are usually ______.
bacterial
54
What are the three pathways by which bacteria can colonize bone (osteomyelitis)?
1. Hematogenous Spread 2. Contiguous Infections 3. Implantation (following surgery or fracture)
55
Which osteomyelitis-related organism is most likely to spread hematogenously?
Staph. aureus
56
Osteomyelitis in the oral cavity is most likely to spread via which mechanism?
Contiguous infection (i.e. Odontogenic)
57
What are the signs/symptoms of osteomyelitis?
pain/tenderness | overlying erythema and/or swelling
58
Sequestrum is _______ bone.
non-vital
59
Involucrum is ______ bone.
new (vital)
60
Chronic osteomyelitis is characterized by _____ sitting inside ______.
nonvital (sequestrum) sitting inside a rim of new bone (involucrum)
61
What is the treatment for osteomyelitis?
drainage antibiotics (specific to the particular organism involved) surgical debridement

SP16- Gen Path Exam 3 (12 decks)

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