Fa - Pathology 3

Front 1

CO poisoning ~~

(2)

Back 1

1. cherry-red skin

2. *EARLY* HA

Front 2

why is hypoxia so bad?

Back 2

it impairs P'n

=> dec's ATP

- low ATP disrupts key cell functions, like the Na/K pump, Ca2+ pump

Front 3

problem with disrupting Na/K pump:

Back 3

Na+ stays in cells

=> water comes in, cell swells

Front 4

problem with disrupting Ca2+ pump:

Back 4

intracellular Ca2+, which should be low, gets too high

=> act'n of MANY enzymes

Front 5

hallmark of reversible cell injury =

Back 5

cell swelling

Front 6

hallmark of irreversible cell injury =

Back 6

membrane damage / mit. vacuolization

Front 7

hallmark of cell death =

Back 7

loss of nucleus

Front 8

pyknosis =

Back 8

nucleus shrinks

Front 9

karyorrhexis =

Back 9

nucleus broken into pieces

Front 10

karyolysis =

Back 10

nucleus pieces broken into basic building blocks

Front 11

hallmark of coag. necrosis =

Back 11

preserved cell shape, despite loss of nucleus

Front 12

which vitamins are antioxidants?

Back 12

ACE

Front 13

***what does oxidative burst of N's do?

Back 13

converts O2 into O2-, via NADPH oxidase

=> kills org's

Front 14

what is B2-microglobulin all about?

Back 14

provides structural support for MCH Class I

- without it, Class I CAN'T be expressed on cell

Front 15

B2-microglobulin is not ____________ __________ on dialysis

Back 15

not filtered well

=> builds up => deposits in joints

Front 16

if necrosis occurs, the first/main responders are:

Back 16

N's

Front 17

what are TLR's all about?

Back 17

recognize PAMP's of pathogens

- e.g. CD14 = TLR of mP's

- activate NF-xB

Front 18

ALL PG's mediate:

Back 18

vasodilation at the arteriole and inc. vascular permeability at the post-cap venule

Front 19

PGE2 also mediates:

Back 19

***fever and pain***

Front 20

4 chemotactic agents:

Back 20

LTB4

C5a

IL-8

bact. products

Front 21

mast cells are activated by:

(3)

Back 21

1. tissue trauma

2. C3a, C5a

3. cross-linking of cell-surface IgE by antigen

Front 22

C3b =

Back 22

opsonin (flag) for phag. by mP's

Front 23

***Hageman factor = inactive proinflam. factor that activates:

(4)

Back 23

1. coagulation

2. fibrinolytic system

3. Complement

4. kinin system

Front 24

fibrinoid necrosis is m.c.ly seen in:

Back 24

vasculitis

Front 25

7 steps of N' action:

Back 25

1. Margination

2. Rolling

3. Adhesion

4. Transmigration/Chemotaxis

5. Phag.

6. destruction of Phagocytosed products
(via oxidative burst of NADPH oxidase)

7. Resolution
(N's die within tissue => pus)

Front 26

Margination ~~

Back 26

arteriole vasodilation,

=> => N's can go from center of vessel to periphery

at the *post-cap venule*

Front 27

Rolling ~~

Back 27

upregulation of selectins (speed bumps)

=> N's slow down

Front 28

P-selectin is released by:

Back 28

W-P bodies

(also release vWF)

Front 29

E-selections are induced by:

(2)

Back 29

TNF-a

IL-1

Front 30

BOTH selectins bind:

Back 30

Sialyl Lewis X on leukocytes

=> leukocytes slow down along vessel wall

Front 31

Adhesion ~~

Back 31

CAM's of endothelium

being upregulated by TNF-a and IL-1,

integrins of leukocytes being upregulated by C5a, LTB4

=> FIRM adhesion

Front 32

Transmigration ~~

Back 32

crossing post-cap venule endothelium

=> attracted to site of injury by chemotactins

Front 33

Phag. is enhanced by:

(2)

Back 33

IgG, C3b

- otherwise, phag. is blind.

Front 34

Chediak-Higashi synd. =

Back 34

AR prot. trafficking defect - railroad system is knocked out

- mPs' phagosomes can't be brought to lysosomes

Front 35

chronic granulomatous dz ~~

Back 35

1. X-linked

OR

2. AR

defect in NADPH oxidase

Front 36

m.c.c. of chronic granulomatous dz = S. aureus, but most imp. for exams is:

Back 36

Pseudomonas cepacia

Front 37

5 bugs that cause chronic granulomatous dz:

Back 37

1. S. aureus

2. Pseudomonas cepacia

3. Serratia marcescens

4. Nocardia

5. Aspergillus

Front 38

S. aureus,
Pseudomonas cepacia,
Serratia marcescens,
Nocardia,
and
Aspergillus

cause chronic granulomatous dz b/c:

Back 38

they are CATALASE+

- catalase destroys H2O2

=> MPO has nothing to work with - can't produce HOCl to destroy them

(and already lacking NADPH oxidase)

Front 39

screen for CGD =

Back 39

Nitroblue tetrazolium test

Front 40

if NADPH oxidase is intact/effective, Nitroblue test will become:

Back 40

blue

- if not, will remain colorless

Front 41

remember that with MPO deficiency, the Nitroblue test will:

Back 41

still turn blue,

b/c there's no problem with NADPH oxidase

Front 42

mP's are _____ - independent

Back 42

O2

- use lysozymes

Front 43

resolution of inflammatory process and healing occur when mP's release:

(2)

Back 43

TGF-B

IL-10

Front 44

resolution is put off and inflammation continues when mP's release:

Back 44

IL-8

=> calls in additional N's

(chemotactin like LTB4, C5a)

Front 45

how are CD4+ cells activated?

(2)

Back 45

1. APC presents antigen to MHC II

2. B7 of APC binds CD28 of CD4+

Front 46

Th1 releases:

(2)

Back 46

1. IL-2
(CD8+ activator)

2. IFN-y
(mP activator)

Front 47

Th2 helps B-cells by releasing:

Back 47

1. IL-4
(induces class switching to IgG, IgE)

2. Il-5
(eosinophil chemotaxis and act'n, maturation of B-cells to plasma cells, and class switching to IgA)

3. IL-10
(inhibits Th1 phenotype)

Front 48

how are CD8+ cells activated?

(2)

Back 48

1. APC MCH I presents antigen to TCR

2. IL-2 from Th1 binds CD8+

Front 49

what do naive B cells express?

Back 49

IgM, IgD

Front 50

how are B-cells activated?

(2)

Back 50

1. B-cell presents antigen to CD4+ via MHC II

2. CD40 r' on B-cell binds CD40L on CD4+

(=> Th2 => secretes Il-4, IL-5 => class switching, maturation to plasma cells)

Front 51

differentiation b/w caseating and non-caseating granulomas:

Back 51

caseating granulomas have central necrosis

(~ TB, fungal inf's)

Front 52

***3 steps of granuloma formation:***

Back 52

1. mP's present antigen to CD4+'s

2. mP's secrete IL-12, inducing Th1 subtype

3. Th1's secrete INF-y, which converts mP's into epithelioid histiocytes and giant cells

Front 53

5 features of DiGeorge syndrome:

Back 53

1. 22q11 microdeletion

2. failure of 3rd and 4th pharyngeal pouches to dev.

3. **lack of thymus**

4. **lack of PTH glands**

5. check heart abnormalities

Front 54

3 etiologies of SCID:

Back 54

1. cytokine r' defects

2. ***adenosine deaminase deficiency**

3. MHC II deficiency
(no CD4+ act'n = impaired func. of B-cells AND CD8+'s)

Front 55

adenosine deaminase deficiency explained:

Back 55

adenosine deaminase prevents buildup of adenosine and deoxyadenosine, which are toxic to lymphocytes

=> deficiency = loss of lymphocytes

Front 56

tx for SCID =

Back 56

stem cell transplant

Front 57

***X-linked A-gammaglobulinemia =

Back 57

COMPLETE lack of Ig in the blood

- b/c naive B-cells can't mature

Front 58

***X-linked A-gammaglobulinemia is caused by:

Back 58

mutated B-TK
(Bruten TK)

- nec. for B-cells to become plasma cells

Front 59

***presentation of X-linked A-gammaglobulinemia:

Back 59

***bacterial, enterovirus, and Giardia inf's***

6 mths after birth

(mom's AB's protect for a while)

- avoid live vaccines

Front 60

CVID ~~

(common variable imm. def.)

Back 60

**low** Ig's due to B-cell *OR* CD4+ defects

Front 61

CVID carries increased risk for:

(2)

Back 61

1. AI dz

2. lymphoma

Front 62

IgA deficiency ~~

Back 62

increased risk of mucosal inf's

Front 63

***Hyper-IgM syndrome*** is due to:

Back 63

**mutated CD40 or CD40L**

=> dec. act'n of B-cells or Th2 = dec. class switching

=> IgM predominates

Front 64

Hyper-IgM synd. results in:

Back 64

recurrent pyogenic inf' s

Front 65

Wiscott-Aldrich synd. is caused by:

Back 65

mut. in WASP gene

(X-linked - only present in MALES)

=> COMBINED B and T cell deficiency

Front 66

triad of Wiscott-Aldrich synd =

Back 66

1. thrombocytopenia

2. eczema

3. recurrent inf's
(esp. pyogenic - no humoral response against capsule)

Front 67

***if C5-C9 are deficient, you're at an increased risk for:

Back 67

Neisseria inf's

Front 68

C1 inhibitor deficiency ~~

Back 68

hereditary angioedema

(ESP. periorbital edema)

- due to overact'n of Complement

Front 69

Hyper-IgM synd. results in:

Back 69

recurrent pyogenic inf' s

Front 70

heart complication of SLE:

Back 70

Libman-Sacks endocarditis

Front 71

Libman-Sacks endocarditis =

Back 71

vegs on BOTH sides of a valve

- unique to SLE

Front 72

removal of drug in DILE =>

Back 72

remission

Front 73

a subset of SLE pts will develop antiphospholipid AB's; specific ones are:

Back 73

1. anticardiolipin AB's
(=> FP VDRL)

2. lupus anticoagulant AB
(=> falsely-elevated PTT)

Front 74

complication of lupus anticoagulant AB =

Back 74

hypercoaguability

- **req's lifelong anticoagulation**

Front 75

Sjogren's syndrome =

Back 75

AI destruction of lacrimal and salivary ducts

Front 76

features of Sjogren's syndrome:

(5)

Back 76

1. dry eyes
("dirt")

2. dry mouth

3. recurrent dental carries
(lack of saliva = teeth not washed)

4. ~~ Rheumatoid Arthritis

5. **inc. risk for B-cell lymphoma**

Front 77

B-cell lymphoma in Sjogren's syndrome:

Back 77

UNILATERAL, enlargING parotid gland

- Sjogren's parotid enlargements are bilateral

Front 78

scleroderma ~~

Back 78

act'n of fibroblasts and deposition of collagen

Front 79

diffuse Scleroderma ~~

(2)

Back 79

1. EARLY visceral involvement

2. esophagus commonly affected

Front 80

CCREST =

Back 80

Calcinosis

anti-Centromere AB

Raynaud's phenomenon

Esophageal dysmotility

Sclerodactyly
(esp. of fingers, hands)

Telangiectasias
(spider-like caps)

Front 81

calcinosis =

Back 81

Ca2+ deposition into any soft tissue

Front 82

sclerodactyly =

Back 82

tightness of skin

Front 83

mixed CT dz ~~

Back 83

mixed features of SLE, scleroderma, and polymyositis

- **anti- U1 RNP**

Front 84

2 quiescent tissues:

Back 84

1. liver

2. proximal renal tubule

- can reenter cell cycle, but it takes a bit

Front 85

granulation tissue eventually becomes scar; mechanism =

Back 85

type III collagen removed by collagenases,

replaced with type I

Front 86

collagenases require ________ as a cofactor

Back 86

ZINC

Front 87

FGF ~~

(2)

Back 87

angiogenesis, skeletal development

Front 88

primary intention = form of cut. healing in which:

Back 88

wound edges are BROUGHT TOGETHER

=> MINIMAL scar formation

Front 89

m.c.c. of delayed wound healing =

Back 89

inf.

- other causes =

Vit. C deficiency,
copper deficiency,
zinc. deficiency

Front 90

copper is required for wound healing b/c it's:

Back 90

a cofactor for lysyl oxidase, nec. to link lysine and hydroxylysine of collagen