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SP16- Gen Path Exam 3 > Bone Tumors, Joints, and Skeletal Muscle Diseases > Flashcards

Bone Tumors, Joints, and Skeletal Muscle Diseases Flashcards

1
Q

______ is the replacement of normal bone by fibrous CT and abnormal bony trabeculae.

A

Fibrous Dysplasia

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2
Q

True or False: Fibrous Dysplasia occurs in elderly adults.

A

False, mostly adolescents and young adults

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3
Q

Which is more common, monostotic or polyostotic fibrous dysplasia?

A

monostotic (70%)

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4
Q

Polyostotic disease is often associated with _______ spots and occasionally with ______.

A

cafe-au-lait spots

edocrine disorders

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5
Q

Jaffe-Lichenstein syndrome is the polyostotic diseases with _______.

A

cafe-au-lait spots

“Jaffe Cafe”

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6
Q

McCune-Albright syndrome includes _____ and ____.

A

both cafe-au-lait spots and endocrine disorders

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7
Q

What is meant by the “ground glass appearance?”

A

ill-defined radiolucency

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8
Q

True or False: Many patients with Fibrous Dysplasia will stabilize at puberty and therefore do not need treatment.

A

True, just watch them

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9
Q

If fibrous dysplasia does not stabilize at puberty, what kind of treatment is initiated?

A 
anti-resorptive therapy
surgical reduction (wait until after skeletal maturity)
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10
Q

What are the clinical and radiographic features of osteosarcoma (bone tumor)?

A

Radiographic: “sunburst” appearance and “Codman’s
Triangle” (SarComa = SC = Sunburst + Codmans)

Clinical: painful enlarging mass

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11
Q

What is the standard treatment for osteosarcoma?

A

Aggressive surgery and chemotherapy

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12
Q

True or False: Osteosarcoma is the most common primary malignant tumor of bone.

A

True

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13
Q

Any bone can be affected by osteosarcoma. However, the ________ region of ______ bones in the extremities is the most frequent site. 60% of osteosarcomas occur in the _____.

A

metaphyseal
long
knee

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14
Q

What is Codman’s Triangle?

A

an acute angle between the neoplastic bone and the cortex

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15
Q

What is the mean age affected by osteosarcoma?

A

18 years old

most patients are less than 20

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16
Q

__________ is the most common disorder of the joints and is a very frequent, if not inevitable, part of aging

A

Osteoarthritis (degenerative joint disease)

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17
Q

What is the fundamental feature of osteoarthritis?

A

degeneration of the articular cartilage

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18
Q

The process of osteoarthritis may be _____ or _____.

A

primary (arises without obvious predisposing factors)

secondary (arises in a previously deformed joint)

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19
Q

True or False: Osteoarthritis is primarily an inflammation of the joints.

A

False, (in contrast to RA) osteoarthritis may become secondarily inflamed as the process progresses

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20
Q

True or False: The pathogenesis of osteoarthritis is unknown.

A

True,
though the most important factors appear to be:
1. aging
2. mechanical effects (Although osteoarthritis is not exclusively a wear-and-tear process, there is little doubt that mechanical stresses on the joints play a major role in its development)

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21
Q

Normal articular cartilage is bathed in _______ and ensures a friction-free joint surface

A

synovial fluid

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22
Q

How does sound cartilage assist the joints?

A

cartilage spreads the load across the joint surface, helping the bone absorb shock and weight without being crushed

23
Q

Describe the progression of osteoarthritis.

A
  1. cartilage cap develops vertical fissures
  2. fissures extend through the full thickness to the subchondral bone
  3. portions of the cartilage are eroded
  4. exposure of underlying bone
  5. Fragments of dislodged bone and cartilage often float free in the joint space and form “joint mice”
  6. entire surface of the bone becomes exposed
  7. thickening/polishing to an ivory-like appearance
  8. small bony outgrowths termed osteophytes arise at margins
  9. further inflammation of the surrounding synovial membrane
24
Q

True or False: Fusion of the joint does not occur with osteoarthritis.

A

True, another contrast to RA

25
Q

Initial symptoms of osteoarthritis typically develop after the age of ____ and include _____, joint stiffness and deep, aching pain, especially in the morning.

A

50

crepitus

26
Q

What is crepitus?

A

grating or popping sensation

27
Q

True or False: Aside from complete inactivity, there is no way to prevent or halt the progression of osteoarthritis.

A

True

28
Q

Gout is a disorder caused by tissue accumulation of excessive amounts of ______

A

uric acid

29
Q

the high uric acid in body fluids leads to precipitation of _______ that trigger a chain of events that culminate in joint injury

A

urate crystals

30
Q

Elevation of the serum uric acid may result from ________ or _______ or ______. In most cases, the process is due to _____?

A

overproduction of uric acid
reduced excretion of uric acid
both
most cases: an idiopathic overproduction of uric acid

31
Q

How do urate crystals cause pain associated with gout?

A
  1. released crystals activate complement and cause chemotaxis of neutrophils.
  2. Dying neutrophils and reacting macrophages release destructive lysosomal enzymes and inflammatory mediators.
32
Q

Although any joint can be affected by gout, which is most common?

A

the great toe is affected in close to 90% of cases

33
Q

What is the difference between acute and chronic gouty arthritis?

A

Acute: arises upon precipitation of urate crystals in the synovial fluid and membranes

Chronic: arises secondary to repeated episodes of acute attacks, crystals become encrusted on the surfaces

34
Q

What is a “tophus?”

A

Urate crystals that become encrusted on the articular surface and penetrate deeply into surrounding bone and soft tissue = DIAGNOSTIC for gout

35
Q

True or False: Gout exhibits a female predominance and appears in postmenopausal years.

A

False, Gout exhibits a male predominance and rarely produces symptoms prior to 30 years of age

36
Q

What are the two discussed diseases of skeletal muscle?

A

Muscular Dystrophy

Myasthenia Gravis

37
Q

_______ is a heterogenous group of heritable diseases with spontanteous, progressive degeneration of muscle fibers

A

Muscular Dystrophy

38
Q

The most common and devastating form of muscular dystrophy is __________.

A

Duchenne muscular dystrophy (DMD)

39
Q

DMD is an _________ disorder.

A

x-linked recessive

40
Q

Which structural protein is affected by DMD?

A

there is an absence of DYSTROPHIN (key structural protein in skeletal and cardiac muscle that attaches fibers to the membrane)

41
Q

Absence or abnormality of dystrophin is associated with impaired _______ activity and a variety of other derangements in both skeletal and cardiac muscle

A

contractile

42
Q

True or False: DMD only occurs in males.

A

False, could occur in females if there is a mutation

43
Q

The affected muscles of DMD undergo progressive degeneration. This process begins in the _____ and _______with eventual spread to the ________.

A

pelvis
shoulder girdles

extremities

44
Q

When do initial signs of DMD appear? What are the signs?

A

in childhood (around 3-5 years old)

difficulty in standing, walking, and getting out of a chair

45
Q

Despite muscular weakness with DMD, the legs are often enlarged. Why?

A

pseudohypertrophy (replacement of lost muscle with fibrofatty tissue)

46
Q

By their ______, most patients with DMD are confined to wheel chairs. Death usually ensues before age ___ due to cardiac involvement, weakness of muscles necessary for respiration, or from pneumonia secondary to aspiration of food associated with difficulties in swallowing.

A

teens

30

47
Q

How is DMD diagnosis confirmed after initial signs?

A

family history
elevated serum muscle enzymes
electromyography
biopsy

48
Q

Myasthenia gravis is an _______ disease which results in destruction of ______ in the neuromuscular junction

A 
autoimmune
acetycholine receptors (70-90% reduction)
49
Q

How does myasthenia gravis manifest clinically?

A

weakness and prominent fatigability of voluntary muscles
(the most active muscles generally are the most severely affected: muscles involved in eye movement, facial expression, chewing, swallowing and respiration)

50
Q

True or False: Myasthenia Gravis can arise at any age and exhibits a female predominance

A

True

51
Q

There is a strong association between Myasthenia Gravis and _______ abnormalities.

A

THYMUS
~65% = thymic hyperplasia
~15% = thymoma

52
Q

In Myasthenia Gravis patients, the most common sites of initial involvement are the muscles of the _____ and the muscles controlling _________.

A 
eyelids (ptosis: drooping)
eye movements (diplopia: double vision)
53
Q

How is myasthenia gravis treated?

A

The natural course of the condition is improved by:

  • thymectomy in cases of thymus hyperplasia
  • removal of the tumor in those with a thymoma
  • medications (anticholine esterase and corticosteroids)
54
Q

In the past, _______ was a major cause of death for patients with myasthenia gravis; due to improved therapies, the current 5-year survival is ____.

A

respiratory compromise

95%

SP16- Gen Path Exam 3 (12 decks)

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