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Systems Pathology > Exam 1 > Flashcards

Exam 1 Flashcards

1
Q

Epidemiology is the who are the why?

A

The who

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2
Q

Etiology is the who or the why?

A

The why

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3
Q

What is Klippel-Feil syndrome?

A

Congenital fusion of 2 or more cervical vertebrae

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4
Q

Klippel-Feil Syndrome is idiopathic. What does that mean?

A

Your born with it. It’s not acquired

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5
Q

What pathology is usually seen with Klippel-Feil Syndrome?

A

Sprengel’s Deformity which is a fused scapula. Scapula never descended

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6
Q

How many people have Klippel-Feil Syndrome?

A

1 in 40,000

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7
Q

What is the difference between Type 1 and Type 2 congenital kyphosis?

A

Type 1 is failed development (severe)
Type 2 is failed segmentation (mild)

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8
Q

Why is type I congenital kyphosis severe?

A

The spinal cord is compressed

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9
Q

Which pathologies that we talked about are congenital disorders?

A

Achondroplasia, Cleidocranial dysplasia, osteogenesis imperfecta, osteopetrosis

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10
Q

What does it mean to be a congenital disorder?

A

An often-inherited disorder.

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11
Q

Congenital disorders effect what part of the skeleton?

A

The whole skeleton

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12
Q

What is dysplasia?

A

Mutations that interfere with growth and bone remodeling

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13
Q

What type of disorders is dysplasia seen in

A

Congenital disorders

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14
Q

What is Cleidocranial Dysplasia?

A

Absent/under developed clavicles

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15
Q

Besides absent/ under-developed clavicles, what other symptoms are seen with Cleidocranial dysplasia?

A

Dental abnormalities, delayed closure of cranial sutures, short stature

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16
Q

What causes Cleidocranial dysplasia?

A

Genetic inheritance- abnormality in RUNX2 gene

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17
Q

What is the RUNX2 genen important for?

A

Function during skeletal formation and bone maintenance

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18
Q

What is the most common form of dwarfism and skeletal dysplasia?

A

Achondroplasia

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19
Q

What is the main symptom of achondroplasia?

A

Shortened long bones

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20
Q

Achondroplasia increases the odds of having what?

A

Stenosis of foramen magnum and spinal stenosis

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21
Q

Define stenosis

A

Narrowing

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22
Q

What is the abnormality in the vertebrate most commonly seen with achondroplasia?

A

Bullet vertebrae

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23
Q

What are some pathologies caused by having bullet vertebrae?

A

Hyperlordosis, hyperkyphosis, scoliosis

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24
Q

Frontal bossing is another feature seen in those with achondroplasia. What is frontal bossing?

A

Forehead protrusion

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25
Q

Trident hand is typically seen with what disorder?

A

Achondroplasia

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26
Q

What is the pathogenesis (how) of achondroplasia?

A

Inhibited endochondral growth and decreased cartilage synthesis.

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27
Q

Achondroplasia’s Decreased cartilage synthesis causes what?

A

Short/ bowed legs, frontal bossing, mid face hypoplasia

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28
Q

What is the epidemiology of achondroplasia?

A

Epidemiology is the who if you forgot. People who have the mutated FGFR3 gene.

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29
Q

What is the etiology of achondroplasia?

A

90% of the time, the mutated FGFR3 gene is spontaneous and most commonly from sperm. 10% are autosomal dominant.

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30
Q

What are other names for osteogenesis imperfecta?

A

Brittle bone disease
Type I collagen disease

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31
Q

What is brittle bone disease?

A

A condition resulting in brittle bones and weak connective tissue due to altered type I collagen

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32
Q

What is the lifespan of the 2 types of Osteogenesis?

A

Type 1: normal lifespan
Tyler 2: lethal in utero

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33
Q

What is affected by Type I collagen disease?

A

Bones, eyes, teeth, inner ear bones, skin and joints

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34
Q

What is blue sclara?

A

When the white part of the eyes looks blue. Seen in osteogenesis imperfecta/brittle bone disease/ Type I collagen disease

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35
Q

What are some symptoms seen during childhood in those with type I collagen disease?

A

Fractures, bowing, scoliosis, hearing loss, dwarfism

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36
Q

What is the status of adjusting those with osteogenesis imperfecta?

A

It’s contraindicated

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37
Q

What is the pathogenesis of Brittle Bone disease?

A

Mutated Type 1 Collagen causing a premature breakdown. It’s autosomal dominant.

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38
Q

How common is Type I collagen disease?

A

1 in 20,000

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39
Q

What stands out in x rays of children with osteogenesis imperfecta that have been treated with biphosphonates?

A

Zebra stripes in bone

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40
Q

________ is associated with normal bone strength, but frequently with stenosis of the foramen magnum and lumbar spinal stenosis

A

Achondroplasia

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41
Q

_______ frequently involves localized abnormalities in development of clavicles and teeth.

A

Cleidocranial dysplasia

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42
Q

Individuals born with_______ will be affected by an incomplete formation of one or more vertebral bodies

A

Type I congenital kyphosis

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43
Q

If x-rays look radiolucent, the bone is more dense or less dense?

A

Less dense

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44
Q

X rays that are radiolucent, areas appear darker or lighter?

A

Darker

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45
Q

If someone said areas look brighter/whiter on an x ray it is called what?

A

Radiopaque/radiodensity

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46
Q

High density bone appears ________ on x ray?

A

Bright/white

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47
Q

Marble bone disease is also called what?

A

Osteopetrosis

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48
Q

T or F: bone that is very dense is stronger?

A

False

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49
Q

Why is the bone weak in osteopetrosis?

A

Because it never breaks down old bone. Just building new bone on top of it.

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50
Q

What is skeletal sclerosis?

A

Increased density of bone

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51
Q

What is osteopetrosis

A

Group of genetic disorders causing skeletal sclerosis, bony stenosis, and deranged hematopoiesis.

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52
Q

What disease did we talk about this unit that corresponds with Erlenmeyer flask deformity?

A

Osteopetrosis

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53
Q

Erlenmeyer flask deformity is also called?

A

Fills medulla

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54
Q

What type of fracture is common in osteopetrosis patients?

A

Chalkstick fracture aka pathologic fracture. Bone is so brittle it snaps like chalk

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55
Q

What is the how of osteopetrosis?

A

Decreased osteoclast activity causing an inability to remodel and reabsorb bone leading to thick, dense, brittle bone

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56
Q

Osteopetrosis has a loss of hematopoietic bone marrow causing reduced what?

A

Production of red and white blood cells

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57
Q

What is treatment of osteopetrosis?

A

Decreased calcium intake and/ or stem cell transplant

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58
Q

What is the who and why of osteopetrosis?

A

Inherited

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59
Q

What is acquired severe loss of bone density and mass?

A

Osteoporosis

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60
Q

What does a DEXA scan measure?

A

Bone mineral density (BMD)

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61
Q

Many times, patients don’t know they have osteoporosis until _________.

A

They have a fracture

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62
Q

What is osteopenia?

A

A milder loss of bone density and mass than what’s seen in osteoporosis

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63
Q

Osteopenia and osteoporosis both have decreased __________ and increased ________

A

Bone mass

Porosity

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64
Q

What are the main areas we see osteoporosis in?

A

Trabecular/spongy/cancellous bone.

Vertebral bodies, femoral neck, and calcaneous

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65
Q

What does the DEXA scan determine?

A

Risk for fracture

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66
Q

A DEXA scan greater than 2.5 deviations below is what?

Osteoporosis
Osteopenia

A

Osteoporosis

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67
Q

A DEXA scan between 1-2.5 standard deviations below is what?

Osteoporosis
Osteopenia

A

Osteopenia

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68
Q

What is a T-score?

A

DEXA compared to peak bone mineral density

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69
Q

What is Z- score?

A

DEXA compared to age and gender matched BMD

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70
Q

Is the T core or Z score more commonly used?

A

T score

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71
Q

Localized osteoporosis is caused by what?

A

Disuse

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72
Q

What are the types of osteoporosis affecting the entire skeleton?

A

Primary and secondary

73
Q

Who is more likely to get primary osteoporosis?

A

Those who are senile and/or post menopausal

74
Q

What are 2 more symptoms seen in secondary osteoporosis?

A

Neoplasia, body-wide immobilization

75
Q

Who are we more likely to see secondary osteoporosis in?

A

Those with hyperparathyroidism, nutrient deficiencies, those who use corticosteroids, alcohol, or tobacco

76
Q

Why does menopause contribute to osteoporosis?

A

There is decreased serum estrogen leading to increased osteoclast activity

77
Q

Why does aging contribute to osteoporosis?

A

There is decreased synthetic activity of osteoblast and reduced physical activity

78
Q

How much bone mass is lost each year after mid 20’s

79
Q

Why does osteoporosis affect women due to estrogen levels but doesn’t affect men as much when they have low estrogen levels regularly?

A

Because it’s the fluctuation of estrogen. Not necessarily estrogen levels being low.

80
Q

What type of exercise is beneficial to our osteoporotic patients?

A

Weight training

81
Q

Vitamin D deficiency leads to 2 diseases. What are they?

A

Rickets: childhood version
Osteomalacia: adult version

82
Q

What is rickets

A

Undermineralized bone causing bones to be weak and bow. Patient also has poor growth plates.

83
Q

What is the how of rickets?

A

Failed osteoid formation and/or failed deposition of bone in the growth plates

84
Q

Is rickets or osteomalacia more severe?

85
Q

Why do children get rickets?

A

Deficiency in vitamin D either dietary or UV. Could be due to malabsorption or chronic renal disorders as well.

86
Q

What is osteomalacia?

A

Undermineralized bone causing bone to be weak and prone to fracture

87
Q

What is the pathogenesis of osteomalacia?

A

Failed remodeling of bone and/or Undermineralized matrix accumulation

88
Q

Why do people get osteomalacia?

A

Deficiency in vitamin D either dietary or UV. Could also be due to malabsorption or chronic renal disorders

89
Q

When you hear hyperparathyroidism, what should you think?

A

Taking too much Ca+ from bone

90
Q

What is primary hyperparathyroidism?

A

Autonomous PTH production most commonly caused by an adenoma.

91
Q

What is increased in primary hyperparathyroidism?

A

osteoclast activity

Renal tubular resorption of Ca+

92
Q

What is secondary hyperparathyroidism?

A

Retail failure occurs meaning we can’t regulate Ca+ levels causing hypocalcemia causing an increase in PTH

93
Q

Primary hyperparathyroidism is asymptomatic a lot of times. But what are some symptoms patients may experience?

A

Kidney stones (MC), bone pain, peptic ulcers, depression, anorexia, nausea, demineralization/pathologic fracture

94
Q

What are some skeletal changes we may see in x rays of those with hyperparathyroidism?

A

Salt and pepper skull, multiple myeloma, rugger-Jersey spine, subperiosteal resorption (radial side, middle phalanges)

95
Q

What is treatment of hyperparathyroidism?

A

Depending on if it’s primary or secondary. However mostly just drinking water, physical activity, and avoiding diuretics.

96
Q

Is hyperparathyroidism reversible when PTH levels are normalized?

97
Q

What is the acronym for hyperparathyroidism symptoms?

A

Painful bones, renal stones, abdominal groans, and psychic moans

98
Q

What is the pathogenesis (how) of hyperparathyroidism?

A

Parathyroid adenoma. This causes increases in parathyroid hormone (PTH), increased osteoclast activity, and increases Ca2+ in blood

99
Q

What is a brown tumor?

A

In hyperparathyroidism, cortical and trabecular bone gets resorbed and replaced by loose connective tissue forming a brown tumor

100
Q

What is a skeletal feature of advanced hyperparathyroidism causing the bone to have a cystic appearance?

A

Osteitis fibrosa cystica

101
Q

Who does hyperparathyroidism most commonly effect?

A

Post-menopausal women

102
Q

What is the etiology of hyperparathyroidism?

A

Primary: idiopathic (parathyroid adenoma)
Secondary: kidney failure

103
Q

What is called Shaggy bone?

A

Pager disease

104
Q

Osteitis deformans is another name for what?

A

Paget Disease

105
Q

What is Paget Disease?

A

Disordered bone remodeling in the axial skeleton. Rare in extremities.

106
Q

What are the symptoms of Osteitis Deformans?

A

80% is asymptomatic. Otherwise, bone pain, bowing, nerve compression, and an increased fracture risk. 1% experience osteosarcoma (poor prognosis)

107
Q

How is Paget Disease diagnosed?

A

Increase in alkaline phosphatase in serum. This is a byproduct of osteoblast activity.

108
Q

What type of fracture is common with Paget disease?

A

Chalkstick fracture

109
Q

What disease causes a mosaic jigsaw puzzle pattern in bone?

A

Paget disease (osteitis deformans)

110
Q

You take x-rays of a patient’s spine. One vertebrae appears very radiopaque. What is this called?

A

Ivory vertebrae sign

111
Q

What are the causes of ivory vertebra sign?

A
  1. Paget disease
  2. Metastatic cancer (prostate)
  3. Lymphoma
112
Q

What is the pathogenesis of Paget disease?

A

Disordered bone remodeling due to excessive osteoclast activity and reactionary increase of osteoblast activity

113
Q

What’s the 1st stage of Paget Disease?

A

Regional osteoclastic activity

114
Q

What is the second stage of osteitis deformans?

A

Osteoblastic activity causing disorganized excessive bone. It’s greater mass and poor quality gives the shaggy appearance

115
Q

What is the 3rd stage of Paget Disease?

A

Sclerotic Phase (osteosclerotic)

116
Q

What’s the epidemiology of Paget disease?

A

Older Caucasian males. 2% less than 50 yrs old. 10% more than 80yrs old

117
Q

What is the etiology of Paget Disease?

A

Genetic and geographic

118
Q

What are the most common places affected by Osteitis deformans?

A

Pelvis, sacrum, skull

119
Q

When do people most commonly get diagnosed with osteitis deformans?

120
Q

What 3 countries is Paget disease found more in?

A

Europe
New Zealand
Australia

121
Q

_______ is an endocrine disorder MC from a tumor that affects the entire body but causes reduced bone mineral density in the skeleton.

A

Hyperparathyroidism

122
Q

______ is a group of inherited conditions that are characterized by reduced osteoclast activity

A

Osteopetrosis

123
Q

When do most individuals reach peak bone mass?

124
Q

Which site is MC location for a pathological fracture due to osteoporosis?

A

Femoral neck

125
Q

What is a closed fracture?

A

Intact with overlying tissue

126
Q

What is a compound (open) fracture?

A

Skin is ruptured.

*At risk for infection

127
Q

What is a comminuted fracture?

A

Bone is fragmented or splintered

128
Q

What is a displaced fracture?

A

When the distal segment is misaligned

129
Q

What is a pathological fracture?

A

Fracture at site of any pathology

130
Q

What is a stress fracture?

A

Caused by repetitive use developing slowly. Found in lower leg/foot

131
Q

If optimal healing is taking place, what should happen to a fracture during the first week?

A

Soft tissue callus forms. noncalcified

132
Q

If optimal healing is taking place, what should happen to a fracture between weeks 2 and 3?

A

Development of woven bone

133
Q

If optimal healing is taking place, what should happen to a fracture between weeks 6-8?

A

Endochondral ossification giving us a bony callus

134
Q

______ fractures are at the highest risk of developing an infection post injury.

135
Q

_______ fractures are characterized by an underlying pathology that weakened the bone to the point of fracture following trivial trauma.

A

Pathological

136
Q

What fracture goes straight through the growth plate?

A

Type I Salter-Harris fracture

137
Q

What is type II Salter Harris fracture?

A

When it extends through the growth plate and the metaphysis

138
Q

What is the most common Salter Harris fracture?

139
Q

How many Salter Harris fractures are type II?

140
Q

What type of Salter Harris fracture goes through the growth plate and epiphysis?

141
Q

10% of Salter Hardis fractures are what type?

142
Q

What type of Salter Harris Fracture goes through the metaphysis, growth plate, and the epiphysis?

143
Q

T or F: Type 4 Salter Harris fracture has the worst prognosis and is the least common.

A

False. Type 5

144
Q

What is a type 5 Salter Harris fracture?

A

The growth plate is crushed

145
Q

What type of Salter Harris fracture is most frequently missed on x ray?

146
Q

10% of hip injuries result in what?

A

Avascular necrosis aka osteonecrosis

147
Q

What is ostsonecrosis aka avasuclar necrosis?

A

Ischemic necrosis of bone

148
Q

Is osteonecrosis asymptomatic or painful?

149
Q

Where are common places to see osteonecrosis aka avascular necrosis?

A

Hip, knee, shoulder, wrist, ankle

150
Q

Osteonecrosis can cause bone to collapse/fracture which causes _______.

A

Osteoarthritis

151
Q

What is the how of osteonecrosis?

A

Ischemia. Caused by mechanical trauma to vascularure, extravascular pressure, or thrombotic occlusion

152
Q

How do many osteonecrotic injuries heal?

A

Via creeping substitution

153
Q

Who is osteonecrosis most common in?

A

30-50 year olds

154
Q

What are the risk factors of osteonecrosis?

A

Vascular injury
Prolonged corticosteroid use
Systemic vascular pathology
Susceptibility to thrombosis
25% are idiopathic

155
Q

What is osteomyelitis?

A

Bone-marrow inflammation from an infection

156
Q

What is the how of osteomyelitis?

A

Infection
1. Hematogenous (MC in children)
2. Adjacent infection (MC in adults)
3. Implantation (MC In adults)

157
Q

What are the 2 types of osteomyelitis?

A
  1. Pyrogenic osteomyelitis
  2. Mycobacterial osteomyelitis
158
Q

What’s the most common bacteria found in pyogenic osteomyelitis?

A

Staphylococcus aureus

159
Q

How do we diagnose pyogenic osteomyelitis?

A

Radiography and biopsy

160
Q

What is involucrum?

A

Pyogenic Osteomyelitis

The part of the bone that surrounds the infected bone

161
Q

What is the sequestrum?

A

Pyogenic osteomyelitis

Entrapped necrotic bone

162
Q

What is mycobacterial osteomyelitis?

A

Tuberculosis is the cause of the bone infection. There is severe destruction and caseous granulomas

163
Q

How is mycobacterial osteomyelitis aka skeletal tuberculosis spread?

A

Hematogenous (mc) or lymphatic

164
Q

What part of the body does mycobacterial osteomyelitis affect?

A

Long bones and vertebrae

165
Q

Path Disease is a possible neuro deficit of what?

A

Mycobacterial osteomyelitis

166
Q

Which disease is a common risk factor for development of avascular necrosis of bone?

A

Disorders that increase the risk for clotting

167
Q

_______ is the term used for the entrapped necrotic bone in a patient with pyogenic osteomyelitis

A

Sequestrum

168
Q

What is arthritis?

A

Inflammation of a joint

169
Q

What is osteoarthritis?

A

Degeneration of articular cartilage

170
Q

What is the most common joint disorder?

A

Osteoarthritis

171
Q

Is primary or secondary the most common form of osteoarthritis?

172
Q

If a child has osteoarthritis, is it primary or secondary?

173
Q

What causes secondary osteoarthritis in adults?

A

Trauma, obesity, disease/deformity

174
Q

What are the common locations of osteoarthritis?

A

Cervical and lumbar spine, knees, hips, DIP joint, 1st MCP joint, 1st TMT joint

175
Q

What are 2 things we see in advanced osteoarthritis?

A

Subchondral cysts and sbudnagion

176
Q

Define ankylosis

177
Q

Spinal osteoarthritis usually occurs with _______.

A

Degenerative disc disease

178
Q

Define spondylosis

A

Spinal pain from degenerative changes

179
Q

What is degenerative disc disease?

A

Facet degeneration stressing the discs > the degenerating discs stresses facets

Systems Pathology (2 decks)

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