Pathology Exam Ii

Front 1

Describe denervation atrophy

Back 1

Any process that affects anterior horn cells in PNS
manifestation- spinal muscular atrophy

Front 2

Most common form of denervation atrophy

Back 2

Werdnig- Hoffman disease
onset- birth to 4 months, death within 3 years due to muscle atrophy

Front 3

Describe muscular dystrophies

Back 3

inherited disorders, often beginning in childhood, characterized clinically by progressive muscle weakness and wasting. Histologically, muscle fibers replaced by fibrofatty tissue

Front 4

Which muscular dystrophy is more severe - DMD or BMD

Back 4

DMD

Front 5

What is most common type of muscular dystropy

Back 5

DMD - 1 per 3500 live male births

Front 6

When is DMD clinically manifested

Back 6

By age of 5, with weakness leading to wheelchair dependness by age of 10-12,death in early 20's

Front 7

Do females have DMD

Back 7

Females are carriers, but they are asymptomatic but often have elevated serum creatin kinase.
-females are at risk of developing dilated cardiomyopathy later in life

Front 8

What is the role of dystrophin in DMD and BMD

Back 8

in DMD there is no or minimal dystrophin
in BMD there is abnormal diminished dystrophin

Front 9

Weakness in DMD begins at

Back 9

Pelvic girdle, and progresses to shoulder

Front 10

Enlargement of calf muscles associated with weakness in DMD is called

Back 10

Pseudohypertrophy - caused by initial increase in muscle fibers and then by replacement with fat and connective tissue

Front 11

Which plasma protein is elevated in DMD

Back 11

Serum creatin kinase is elevated during first decade of life, and then returns to normal

Front 12

Death in DMD results from

Back 12

Respiratory insufficiency, pulmonary infection and cardiac decompensation

Front 13

Boys with BMD develop symptoms earlier or later then DMD

Back 13

Later, with slower progression, and can live normal life span

Front 14

Ion channel myopathies

Back 14

Group of familial disorders characterized by myotonia, relapsing episodes of hypotonic paralysis(induced by cold, vigorous exercise or high carbohydrate meal)

Front 15

Malignant hyperthermia

Back 15

Rare clinical symdrome charaterized by dramatic hypermetabolic state - tachycardia, tachypnea, muscle spasms, triggered by induction of anesthesia
Caused by mutation of gene coding for voltage gated Ca channel

Front 16

Name 3 inflammatory myopathies

Back 16

Dermatomyositis, polymyositis, inclusion body myositis

Front 17

Dermatomyositis

Back 17

Inflammation of both skin and muscle

Front 18

Distinctive skin rash in dermatomyositis

Back 18

Lilac discoloration of upper eyelids with periorbital edema
Also can be accompanied by Grotton lesions - scaling red eruptions at knuckles, elbows, knees

Front 19

Muscular involvement in dermatomyositis

Back 19

Slow onset
Affects proximal muscles first
Dysphagia
40% correlation with cancer

Front 20

Polymyositis differs from DM only in

Back 20

There is no skin involvement in PM, only proximal muscle involvement

Front 21

Inclusion body myositis differs from DM and PM in

Back 21

distal muscle involvement, rather then proximal

Front 22

Ethanol myopathy

Back 22

Binge drinking of alcohol produces acute rhabdomyolysis with accompanying myoglobinuria, which may lead to renal failure

Front 23

Drug induced myopathy

Back 23

Steroid myopathy, characterized by muscle fiber atrophy, predominantly affecting type II fibers

Front 24

Chloroquine myopathy

Back 24

Drug originally used to treat malaria can produce myopathy

Front 25

Myasthenia Gravis

Back 25

Muscle disease caused by immune mediated loss of Ach receptors

Front 26

MG prevalence

Back 26

1 in 100 000

Front 27

What else is often found in patients with MG

Back 27

Thymic hyperplasia in 65% and thymoma in 15 %

Front 28

How does MG present clinically

Back 28

Ptosis and diplopia (double vision), generalized weakness

Front 29

Which drugs are best to treat MG

Back 29

Ach esterase inhibitors

Front 30

Lambert Eaton syndrome

Back 30

Disease of NMJ, paraneoplastic process commonly assoicated with small cell lung CA (60%)
Patients develop proximal muscle weakness along wih autonomic dysfunction

Front 31

Two processes of repair of tissue damage

Back 31

Regeneration and healing

Front 32

Definition of regeneration

Back 32

Growth of cells and tissue to replace lost structures (liver + kidney)

Front 33

Definition of healing

Back 33

Response to

-wound
-inflammation
-necrosis

Front 34

Regeneration requires

Back 34

intact connective tissue scaffold

Front 35

Cell cycle

Back 35

G1 presynthetic
S - DNA synthesis
G2-premitotic
M-mitosis

Front 36

Labile tissue

Back 36

Cells proliferate throughout life, replacing those that are destroyed - surface epithelia - skin, vagina, cervix, lining of glands

Front 37

Quiescent (stable) tissue

Back 37

low level of replication, but can undergo rapid division under certain stimulus, in G0 but can enter G1 - parenchymal cells of liver, kidney, pancreas, fibroblasts

Front 38

Nondividing (permanent) tissue

Back 38

Cells that have left cell cycle and cannot undergo mitosis in postnatal period

Neurons, cardiac and skeletal muscle cells

Front 39

Difference between embryonic and embryonic stem cells

Back 39

EMbryonic are pluripotent

Front 40

Which growth factor causes migration and proliferation of fibroblasts

Back 40

PDGF - platelet deived GF

Front 41

Can liver regenerate?

Back 41

Yes --> even with 70% removed

Front 42

3 processes of formation of scar

Back 42

-emigration and proliferation of fibroblasts
-deposition of ECM
-tissue remodeling

Front 43

Best example of wound healing by first intention

Back 43

surgical incision

Front 44

Within 24 hours

Back 44

neutrophils appear at the margin, formation of thin epithelial layer

Front 45

By day 3

Back 45

replacement of neutrophils by macrophages
appearance of granulation tissue
collagen fibers at margins of incision
epithelial proliferation thickens epidermal layer

Front 46

Day 5

Back 46

incisional space is filled with granulation tissue
maximal neovascularization
Abundant collagen fibers
Epidermis recovers normal thickness

Front 47

2nd week

Back 47

Continued accumulation of collagen and fibroblasts
Increased vasculariy and edema disappear

Front 48

Month

Back 48

Scar has formed, covered by normal epidermis with no inflammation

Front 49

Are dermal appendages recover in wound healing

Back 49

No, permanently lost

Front 50

Healing by 2nd intention

Back 50

-more intense inflammatory reaction
-larger amount of granulation tissue
-wound contraction
-scar formation