Musculo - Pathology (Part 1) Flashcards Preview

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Flashcards in Musculo - Pathology (Part 1) Deck (179)
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1
Q

Achondroplasia is an autosomal _____ (dominant/recessive) genetic trait.

A

Dominant

2
Q

In achondroplasia, the short limbs are a result of the failure of what type of bone growth?

A

Longitudinal bone growth (this type of bone growth accounts for the growth of the long bones in the limbs)

3
Q

In achondroplasia, _____ (endochondral/membranous) bone growth is not affected.

A

Membranous (this type of bone growth accounts for the formation of the skull, the facial bones, and the axial skeleton)

4
Q

In achondroplasia, the limbs are shorter than normal, but the _____ and _____ are normal.

A

Head; trunk

5
Q

Achondroplasia represents a failure of cartilage maturation resulting from a mutation in what receptor?

A

Fibroblast growth factor receptor

6
Q

Individuals with achondroplasia have _____ (normal/decreased) life span and fertility.

A

Normal

7
Q

True or False? Achondroplasia is associated with advanced paternal age.

A

TRUE

8
Q

A reduction in bone mass despite normal bone mineralization is a description of what disease?

A

Osteoporosis

9
Q

What is type I osteoporosis?

A

Increased bone resorption as a result of decreased estrogen levels

10
Q

Which therapy for osteoporosis is considered controversial due to risk of serious side effects?

A

Estrogen replacement therapy

11
Q

What is type II osteoporosis?

A

Senile osteoporosis; affects men and women older than 70 years of age

12
Q

Which upper extremity fracture is commonly seen in patients with osteoporosis?

A

Colles fracture of the distal radius

13
Q

A known osteoporotic patient presents with acute back pain, loss of height and kyphosis. From what is she suffering?

A

Vertebral crush fractures

14
Q

Name four treatments for osteoporosis.

A

Estrogen, calcitonin, bisphosphonates, and pulsatile parathyroid hormone

15
Q

Name two methods of osteoporosis prophylaxis.

A

Exercise and calcium supplementation before age 30 years

16
Q

A failure of normal bone resorption that leads to thickened, dense bones is a description of which disorder?

A

Osteopetrosis

17
Q

Osteopetrosis is a bone defect caused by the abnormal function of which cell type?

A

Osteoclasts

18
Q

In patients with osteopetrosis, is the serum calcium level high, normal, or low?

A

Normal

19
Q

In patients with osteopetrosis, is the serum phosphate level high, normal, or low?

A

Normal

20
Q

In patients with osteopetrosis, is the alkaline phosphatase level high, normal, or low?

A

Normal

21
Q

A genetic deficiency in carbonic anhydrase II leads to what disease?

A

Osteopetrosis

22
Q

Name three complications of osteopetrosis due to decreased space for bone marrow.

A

Anemia, thrombocytopenia, and infection

23
Q

What neurologic lesions may result from osteopetrosis?

A

Cranial nerve palsies due to narrowed foramina in the skull

24
Q

What is the classic x-ray finding in osteopetrosis?

A

Erlenmeyer flask bones that flare out

25
Q

What is the disorder in which the defective mineralization of osteoid leads to soft bones?

A

Osteomalacia/rickets

26
Q

Vitamin D deficiency in adults leads to decreased levels of what important element of bone formation?

A

Calcium

27
Q

A decreased level of calcium leads to increased levels of what hormone?

A

Parathyroid hormone

28
Q

In osteomalacia/rickets, phosphate levels are _____ (increased/decreased).

A

Decreased (due to secondary hyperparathyroidism induced by low calcium levels)

29
Q

Vitamin D deficiency in childhood causes what disorder?

A

Rickets

30
Q

What condition causes osteitis fibrosa cystica?

A

Hyperparathyroidism

31
Q

In osteitis fibrosa cystica, what are brown tumors?

A

Cystic spaces lined by osteoclasts that are filled with fibrous stroma or blood

32
Q

In patients with osteitis fibrosa cystica, is the serum calcium level high, low, or normal?

A

High

33
Q

In patients with osteitis fibrosa cystica, is the serum phosphorus level high, low, or normal?

A

Low

34
Q

In Pagets disease, is there abnormal activity of osteoblasts, osteoclasts, or both?

A

There is abnormal activity of both osteoblasts and osteoclasts

35
Q

Which disease of the bone is described as abnormal architecture of the bone which is the result of increased osteoblastic and osteoclastic activity?

A

Pagets disease

36
Q

An individual experiencing hearing loss and increased hat size may suffer from what disorder?

A

Pagets disease (hearing loss is a result of auditory foramen narrowing)

37
Q

Patients with Pagets disease are at increased risk of what type of fractures?

A

Chalk stick fractures of long bones

38
Q

Patients with Pagets disease are at increased risk of what type of cardiac disease?

A

High-output congestive heart failure (due to increased atrioventricular shunting)

39
Q

Patients with Pagets disease are at increased risk of what type of cancer?

A

Osteogenic sarcoma

40
Q

In patients with Pagets disease, is the serum calcium level high, low, or normal?

A

Normal

41
Q

In patients with Pagets disease, is the serum phosphorus level high, low, or normal?

A

Normal

42
Q

In patients with Pagets disease, is the parathyroid hormone level high, low, or normal?

A

Normal

43
Q

In patients with Pagets disease, is the serum alkaline phosphatase level high, low, or normal?

A

High

44
Q

True or False? Osteopetrosis is characterized by elevated calcium.

A

False; lab values are normal

45
Q

Describe the effect osteopetrosis has on bones.

A

Bones are thickened and dense

46
Q

In osteomalacia/rickets, serum calcium levels are _____ (increased/decreased), phosphate levels are _____ (increased/decreased) and PTH levels are _____ (increased/decreased).

A

Decreased; decreased; increased

47
Q

In osteitis fibrosa cystica, serum calcium levels are _____ (increased/decreased); phosphate levels are _____ (increased/decreased); ALP levels are ____ (increased/decreased) and PTH levels are _____ (increased/decreased).

A

Increased; decreased; increased; increased

48
Q

What lab value is elevated in Pagets disease?

A

Alkaline phosphatase

49
Q

In polyostotic fibrous dysplasia, bone is replaced by what three substances?

A

Fibroblasts, collagen, and irregular bony trabeculae

50
Q

Which syndrome is a form of polyostotic fibrous dysplasia characterized by unilateral bone involvement, unilateral coast of Maine spots, and precocious puberty?

A

McCune-Albright syndrome

51
Q

What is the endocrine abnormality found in patients with McCune-Albright syndrome?

A

Precocious puberty

52
Q

What are the dermatologic findings in McCune Albright syndrome?

A

Café-au-lait spots and coast of Maine spots

53
Q

Which benign bone tumor is associated with Gardners syndrome?

A

Osteoma

54
Q

_____ _____ is a benign bone tumor most common in men <25 years old and most commonly found in the proximal tibia and femur.

A

Osteoid osteoma

55
Q

Describe the histology of osteoid osteomas.

A

An osteoid osteoma is composed of interlacing trabeculae of woven bone surrounded by osteoblasts

56
Q

How do osteoblastomas differ from osteoid osteomas?

A

Although the two have the same morphology, osteoid osteomas are < 2 cm and found in the proximal tibia and femur, whereas osteoblastomas are larger and found in the vertebral column

57
Q

Giant cell tumors have a peak incidence in patients in what age range?

A

20 to 40 years of age

58
Q

Which bone tumor is described as a locally aggressive benign tumor that is often seen in the distal femur or the proximal tibia?

A

Giant cell tumor

59
Q

Which primary bone tumor has a characteristic double bubble or soap bubble appearance on x-ray?

A

Giant cell tumor

60
Q

What is the histopathologic appearance of giant cell tumors?

A

Spindle-shaped cells and multinucleated giant cells

61
Q

What is another name for giant cell tumor?

A

Osteoclastoma (not to be confused with osteoblastoma)

62
Q

What is the most common primary benign bone tumor?

A

Osteochondroma

63
Q

At what age do men usually develop osteochondromas?

A

When they are < 25 years of age

64
Q

From where do osteochondromas most commonly originate?

A

Metaphysis of long bones

65
Q

Osteochondromas rarely transform into which type of malignancy?

A

Chondrosarcoma

66
Q

What is another name for osteochondroma?

A

Exostosis

67
Q

Histologically, what benign bone tumor can be described as mature bone with a cartilaginous cap?

A

Osteochondroma (exostosis)

68
Q

What is a benign cartilaginous neoplasm that is found in the intramedullary bone?

A

Enchondroma

69
Q

Where are enchondromas generally found?

A

Distal extremities

70
Q

What is the second most common primary malignant bone tumor after multiple myeloma?

A

Osteosarcoma

71
Q

The incidence of osteosarcoma peaks in males in what age range?

A

10 to 20 years of age

72
Q

Where are osteosarcomas most commonly found?

A

In the metaphysis of the long bones, especially around the knee

73
Q

What four factors predispose to osteosarcoma?

A

Pagets disease of bone, bone infarcts, radiation, and familial retinoblastomas

74
Q

What is Codmans triangle?

A

X-ray findings of elevation of the periosteum, which is classic for osteosarcoma

75
Q

Which malignant primary bone tumor is described as having anaplastic small blue cells?

A

Ewings sarcoma

76
Q

Patients of what sex and age are predisposed to Ewings sarcoma?

A

Males younger than 15 years of age

77
Q

Ewings sarcoma is very aggressive with early _____, but it is responsive to chemotherapy.

A

Metastases

78
Q

What is the classic radiographic appearance of bone in Ewings sarcoma?

A

It has an onion-skin appearance

79
Q

Where are the four most common sites of Ewings sarcoma?

A

The diaphysis of the long bones, the pelvis, the scapula, and the ribs

80
Q

What genetic change is associated with Ewings sarcoma?

A

11;22 Translocation

81
Q

What is the name of a malignant cartilaginous tumor?

A

Chondrosarcoma

82
Q

Chondrosarcomas are most common among men who are between what ages?

A

30 to 60 years of age

83
Q

Where are chondrosarcomas usually located?

A

In the pelvis, spine, scapula, humerus, tibia, and femur

84
Q

A chondrosarcoma may be of primary origin or may occur as a result of what?

A

An osteochondroma with malignant transformation

85
Q

How does a chondrosarcoma look on gross exam?

A

As an expansile glistening mass within the medullary cavity

86
Q

Benign _____ and malignant _____ are found in the intramedullary part of bone.

A

Enchondromas; chondrosarcomas

87
Q

Benign _____ _____ and malignant _____ _____ are found in the diaphysis of bone.

A

Osteoid osteomas; Ewings sarcoma

88
Q

Osteoclastomas (giant cell tumors) are found in the _____ (epiphysis/metaphysis/diaphysis/intramedullary) part of long bones.

A

Epiphysis

89
Q

Benign _____ and malignant _____ are found in the metaphysis of bone.

A

Osteochondromas; osteosarcomas

90
Q

Soap bubble tumors are also known as?

A

Giant cell tumor

91
Q

Which type of tumor causes the formation of Codmans triangle?

A

Osteosarcoma

92
Q

In osteoarthritis, the wear and tear of joints leads to the destruction of what?

A

Articular cartilage

93
Q

What are Heberdens nodes?

A

Calcific spurs of the articular cartilage at the distal interphalangeal joint characteristic of osteoarthritis

94
Q

What are Bouchards nodes?

A

Calcific spurs of the articular cartilage at the proximal interphalangeal joint

95
Q

Pain in weight-bearing joints after use that improves with rest is a classic presentation of which type of arthritis?

A

Osteoarthritis

96
Q

Systemic symptoms such as fever and malaise are _____ (present/absent) in osteoarthritis.

A

Absent; osteoarthritis is not associated with systemic symptoms

97
Q

What pathologic changes of bone are found in osteoarthritis?

A

Histologically, subchondral cysts, sclerosis, thickened capsule, and slight synovial hypertrophy are found in osteoarthritis

98
Q

Name three risk factors for the development of osteoarthritis.

A

Age, obesity, and joint deformity

99
Q

In osteoarthritis of the knees, cartilage loss begins where, leading to what finding?

A

Medial aspect of the knee, leading to a bowlegged appearance

100
Q

What kind of problem is rheumatoid arthritis: mechanical or autoimmune?

A

Autoimmune

101
Q

_____ deviation of the fingers is associated with rheumatoid arthritis.

A

Ulnar

102
Q

What dermatologic finding is seen in rheumatoid arthritis?

A

Subcutaneous nodules

103
Q

What joint finding is pathognomonic in rheumatoid arthritis?

A

Pannus formation in the joints

104
Q

In the hand, which two joints are most affected in rheumatoid arthritis?

A

Metacarpophalangeal and proximal interphalangeal joints

105
Q

Which type of joint is affected by rheumatoid arthritis?

A

Synovial joints

106
Q

Is rheumatoid arthritis more prevalent in males or females?

A

Females

107
Q

Eighty percent of patients with rheumatoid arthritis have what serologic finding?

A

Rheumatoid factor (anti-immunoglobulin G antibodies)

108
Q

Rheumatoid factor is an antibody against what type of molecule?

A

Immunoglobulin G

109
Q

Do the symptoms of rheumatoid arthritis improve or worsen with use?

A

Morning stiffness is associated with rheumatoid arthritis and improves with use

110
Q

Is the joint involvement in rheumatoid arthritis unilateral or symmetric?

A

Symmetric

111
Q

Rheumatoid arthritis is characterized by what symptoms other than arthritis?

A

Morning stiffness improving with use, symmetric joint involvement, and systemic symptoms (fever, fatigue, pleuritis, pericarditis)

112
Q

What are the histologic findings in rheumatoid arthritis?

A

Bone and cartilage erosion, increased synovial fluid, and pannus formation

113
Q

What physical exam finding is characterized by flexed distal interphalangeal joints and overextended proximal interphalangeal joints?

A

Swan-neck deformity

114
Q

What physical exam finding is characterized by overextended distal interphalangeal joints and flexed proximal interphalangeal joints?

A

Boutonniere deformity

115
Q

Rheumatoid arthritis has a strong association with which human leukocyte antigen complex?

A

Human leukocyte antigen DR4

116
Q

Name the finger joint spared in rheumatoid arthritis.

A

Distal interphalangeal joints

117
Q

_____ cysts are associated with rheumatoid arthritis.

A

Bakers cysts, which are located behind the knee

118
Q

What serologic test is less sensitive but more specific for rheumatoid arthritis than rheumatoid factor?

A

Anticyclic citrullinated peptide

119
Q

sjögrens syndrome is characterized by which triad of signs and symptoms?

A

Xerophthalmia (dry eyes and conjunctivitis), xerostomia (dry mouth and dysphagia), and arthritis

120
Q

Individuals with sjögrens syndrome have an increased risk of what type of cancer?

A

B-cell lymphoma

121
Q

Which serologic findings are characteristic of sjögrens syndrome?

A

Antiribonucleoprotein antibodies; also known as anti-Ro and anti-La or SS-A and SS-B

122
Q

sjögrens syndrome typically affects which demographic?

A

Females 40 to 60 years of age

123
Q

A 55-year-old woman complains of a myriad of symptoms including dry eyes, dry mouth, nasal and vaginal dryness, chronic bronchitis, and reflux esophagitis. She denies any arthritic symptoms. What diagnosis can explain this constellation of symptoms?

A

Sicca syndrome

124
Q

Individuals with sjögrens syndrome are at increased risk for which dental condition?

A

Caries, due to decreased production of saliva

125
Q

Gout occurs from the precipitation of _____ _____ crystals into the joints as a result of hyperuricemia.

A

Monosodium urate

126
Q

Thiazide diuretics inhibit the excretion of _____ _____, leading to increased risk of gout.

A

Uric acid

127
Q

Are joints affected by gout in a symmetric or asymmetric pattern?

A

Asymmetric

128
Q

What is the name of the painful metatarsophalangeal joint found in the big toe of a patient with gout?

A

Podagra

129
Q

In patients with gout, tophi often form on which areas of the body?

A

External ear, olecranon bursa, Achilles tendon

130
Q

The crystals in gout are _____ (positively/negatively) birefringent and _____ (needle/rhomboid)-shaped.

A

Negatively; needle

131
Q

Is gout more common in men or women?

A

Men

132
Q

What are two common triggers of acute gout?

A

Consumption of alcohol (alcohol metabolites compete with uric acid for excretion sites in kidney, causing reduced uric acid secretion and buildup in blood) or a large meal

133
Q

Name four treatments for gout.

A

Allopurinol, probenecid, colchicine, and nonsteroidal antiinflammatory drugs

134
Q

Which inborn errors of metabolism can predispose to gout?

A

Lesch Nyhan and Von Gierkes disease

135
Q

Pseudogout is caused by the deposition of _____ _____ crystals within the joint space.

A

Calcium pyrophosphate

136
Q

In pseudogout, are the crystals positively or negatively birefringent?

A

Weakly positively birefringent

137
Q

Pseudogout tends to affect people who are more than ____ years old.

A

50

138
Q

Are men or women more affected by pseudogout?

A

Both are equally affected

139
Q

While in gout crystals are yellow when _____ (perpendicular/parallel) to the light of the microscope, in pseudogout crystals are yellow when _____ (perpendicular/parallel) to the light of the microscope.

A

Parallel; perpendicular

140
Q

Pseudogout tends to affect predominantly the _____ (large/small) joints.

A

Pseudogout affects large joints (classically the knee)

141
Q

Name three organisms that commonly cause septic arthritis.

A

Staphylococcus aureus, Streptococcus, and Neisseria gonorrhoeae

142
Q

Which organism is a common cause of septic arthritis leading to red and painful joints presenting in a monoarticular, migratory, asymmetric pattern?

A

Neisseria gonorrhoeae (remember: STD = Synovitis (eg, knee), Tenosynovitis (eg, hand), and Dermatitis (eg, pustules)

143
Q

Name two diseases that can cause chronic infectious arthritis.

A

Tuberculosis and Lyme disease

144
Q

Seronegative spondyloarthropathies are a form of autoimmune arthritis that occurs without a positive _____ _____ in the blood.

A

Rheumatoid factor

145
Q

In seronegative spondyloarthropathies, there is a strong association with which human leukocyte antigen?

A

Human leukocyte antigen B27

146
Q

Do seronegative spondyloarthropathies affect more men or more women?

A

Men

147
Q

What is the classic triad of clinical signs and symptoms in ankylosing spondylitis?

A

Ankylosis (stiff spine), uveitis, and aortic regurgitation

148
Q

What is the classic triad of clinical signs and symptoms in Reiters syndrome?

A

Urethritis, ophthalmologic findings (conjunctivitis and anterior uveitis), and arthritis (remember: Cant see, cant pee, cant climb a tree)

149
Q

What are two triggers for Reiters syndrome?

A

Gastrointestinal infection and chlamydial infection

150
Q

The physical exam finding of dactylitis (sausage fingers) and radiologic finding of pencil-in-cup deformity are associated with which type of arthritis?

A

Psoriatic arthritis

151
Q

True or False? Most individuals with psoriasis are affected with psoriatic arthritis as well.

A

False; fewer than one third of patients with psoriasis are affected by psoriatic arthritis

152
Q

Psoriatic arthritis commonly presents in a(n) _____ (symmetric/asymmetric) pattern.

A

Asymmetric and patchy involvement

153
Q

A woman has a rash on her cheeks that involves the bridge of the nose but spares the area below the nasolabial sulcus. What is the name of this rash, and what disease is it most commonly associated with?

A

Malar rash; systemic lupus erythematosus

154
Q

Which demographic is most commonly affected by lupus?

A

Females 14 to 45 years of age

155
Q

Systemic lupus erythematosus is most common and severe in which ethnic group: whites, African-Americans, or Asians?

A

African-Americans

156
Q

Which form of endocarditis is associated with systemic lupus erythematosus?

A

Nonbacterial verrucous endocarditis

157
Q

What abnormal finding could a patient with systemic lupus erythematosus have on chest x-ray?

A

Hilar adenopathy

158
Q

True or False? Raynauds phenomenon is associated with systemic lupus erythematosus.

A

TRUE

159
Q

What is the histopathologic appearance of renal disease in systemic lupus erythematosus?

A

Wire loop lesions, due to immune complex deposition in the glomeruli

160
Q

What are two common causes of death in patients with systemic lupus erythematosus?

A

Renal failure and infection

161
Q

Patients with systemic lupus erythematosus may be falsely diagnosed with which disease based on serologic testing?

A

False-positive rapid plasma reagin or venereal disease research laboratory tests for syphilis occur as a result of the presence of antiphospholipid antibodies

162
Q

What are the eleven findings used to make a clinical diagnosis of systemic lupus erythematosus?

A

For the characteristics of systemic lupus erythematosus, remember IM DAMN SHARP: Immunoglobulins (anti-double-stranded DNA, anti-Smith, antiphospholipid), Malar rash, Discoid rash, Antinuclear antibody, Mucositis (oropharyngeal ulcers), Neurologic disorders, Serositis (pleuritis, pericarditis), Hematologic disorders, Arthritis, Renal disorders, Photosensitivity

163
Q

In false-positive rapid plasma reagin or venereal disease research laboratory syphilis tests, the antiphospholipid antibody cross-reacts with what substance?

A

Cardiolipin

164
Q

Which serologic findings (antibodies) can be positive in patients with lupus?

A

Antinuclear antibodies, anti-double-stranded DNA, anti-Smith antibodies, and antihistone antibodies

165
Q

Serositis in systemic lupus erythematosus refers to inflammation of which membranes?

A

Pleuritis and pericarditis

166
Q

In patients with drug-induced lupus, which antibodies are detected?

A

Antihistone antibodies

167
Q

Which antibody is specific for lupus but is not prognostic?

A

Anti-Smith antibodies

168
Q

Which antibody is specific for lupus and predicts a poor prognosis?

A

Anti-double-stranded DNA

169
Q

Which antibody is sensitive for lupus but is not specific?

A

Antinuclear antibodies

170
Q

In which diseases can a positive antinuclear antibody result be found on serologic testing?

A

Systemic lupus erythematosus, sjögrens syndrome (and sicca), and scleroderma

171
Q

What specific population is most affected by sarcoidosis?

A

African-American females

172
Q

What is the underlying pathology in sarcoidosis?

A

Widespread noncaseating granulomas

173
Q

What enzyme levels are elevated in the serum in patients with sarcoidosis?

A

Serum angiotensin-converting enzyme levels

174
Q

What type of lung disease is associated with sarcoidosis: obstructive or restrictive?

A

Restrictive

175
Q

What type of lymphadenopathy is associated with sarcoidosis?

A

Bilateral hilar lymphadenopathy

176
Q

What type of skin pathology is associated with sarcoidosis?

A

Erythema nodosum

177
Q

What is a common finding on the chest x-ray of a patient with sarcoid?

A

Bells palsy

178
Q

What two histopathologic findings are seen in the epithelial granulomas of sarcoidosis?

A

Schaumann bodies and asteroid bodies

179
Q

Inflammation of which structures of the head is seen in sarcoidosis?

A

Ocular and parotid gland inflammation