Tl Phagocytic Leukodeficiency Catos

Front 1

What does it mean for an enzyme to be activated in the coagulation cascade?

Back 1

Serine is in the catalytic state, and has cleaved the peptide bond.

Front 2

What is prothrombin activated by, and what does it become?

Back 2

Prothrombin is activated by Factor Xa and becomes Thrombin.

Front 3

What does Thrombin do?

Back 3

Thrombin activates Fibrinogen to become Fibrin

Front 4

What does factor IXa do?

Back 4

It activates factor X to become factor Xa

Front 5

What activates prothrombin to become thrombin

Back 5

Factor Xa

Front 6

What role does tPA play in regards to clots

Back 6

The injured endothelium releases tPA, which changes plasminogen to plasmin. Plasmin breaks down fibrin fibers to dissolve the clot.

Front 7

What shape is a hemoglobin dissociation curve?

Back 7

Sigmoidal

Front 8

What effect will an increase or decrease in pH have on a hemoglobin dissociation curve?

Back 8

An increase will shift the curve to the left, and a decrease will shift it to the right.

Front 9

What will an increase or decrease in DPG or temp do to a hemoglobin dissociation curve?

Back 9

An increase will shift the curve to the right, and a decrease will shift it to the left.

Front 10

What proteins are important for osmotic pressure?

Back 10

Albumin, Lipoproteins, Transferrin, Prothrombin

Front 11

Describe and give an example of a left-shift to the O2 heme dissociation curve.

Back 11

The curve shifts to the left when examining the lungs, bc they will have a hight affinity for oxygen. This affinity, in a sense, allows O2 to be "saved" for tissues that need it most, and therefore are willing to put out more energy to acquire it.

Front 12

What do catalase-positive microorganisms do?

Back 12

Destroy thier own hydrogen peroxide.

Front 13

How do macrophages and lymphocytes behave differently in inflammation?

Back 13

Macrophages kill first
Lymphocytes consider the AA sequence of the pathogen.

Front 14

What is "at fault" in Chronic Granuloma Disease?

Back 14

NADPH Oxidase

Front 15

What are the three lines of defense against pathogens?

Back 15

Physical/Chemical
Inflammation (phagocytes)
Immune Response (Lymphocytes)

Front 16

How do T-cells kill a cell infected with virus?

Back 16

Stop replication of the virus by forming holes so big that the cell dies.

Front 17

What three cell types are involved in early (acute) cellular response to inflammation?

Back 17

Neutrophils
Eosinophils
Basophils/Mast cells

Front 18

What three cell types are involved in late (chronic) cellular response to inflammation?

Back 18

Monocytes
Lymphocytes
Epitheloid Giant Cells

Front 19

What type of granules contain myeloperoxidase?

Back 19

Primary of azurophilic

Front 20

How do granulocytes localize to the site of injury or infection?

Back 20

Rolling
Adhesion(selectins and integrins)
Diapedese (transedothelial migration)

Front 21

How and where does opsinization occur, and how does it assist in phagocytosis?

Back 21

Microorganisms in serum are coated with serum proteins (opsins) typically IgG and IgM or C3a.
Opsinization enhances phagocytosis.

Front 22

How does NADPH oxidase kill cells?

Back 22

O2 + NADPH --> NADP + 2O2
O2 gets catabolized into several oxygen species that are lethal for microorganisms.

Front 23

How are the phagocytes protected from being killed along with the microorganism?

Back 23

Confinement in phagocytic vacuoles, superoxide dismutase.

Front 24

What protein do granules released to the surface contain?

Back 24

Perforin (5-10 min later, cell bursts)

Front 25

Neutropenia
Leukopenia

Back 25

Less than 1,500 neutrophils per cubic mm
Leukocyte count below 4,000

Front 26

Outline Granulopoiesis

Back 26

Granulocyte Progenitor Cells
Granulocyte Precursor Cells
Myeloblast
Promyelocyte
Myelocyte
Metamyelocyte
Band Cells
Mature Cells
Neutrophils
Basophils
Eosinophils

Front 27

In Neutrophils, what do the primary and secondary granuals contain?

Back 27

Primary: Myeloperoxidase, lysozyme, acid hydrolases, cationic proteins (defensins)
Secondary: Lactoferrin, lysozyme.

Front 28

List three types of congenital neutropenia:

Back 28

Cyclic Neutro
Severe Congenital Neutro
Scwachman Diamond Syndrome

Front 29

List three immunodeficiencies caused by defects of adhesion.

Back 29

LAD Type 1
LAD Type 2
Rac 2 Deficiency

Front 30

Most of the diseases studied in this paper are autosomal recessive. List the ones that are not:

Back 30

Cyclic Neutro -Auto Dom
LAD 2
Rac 2
Late onset IF gamma -auto dom
Earlier onset Chronic Gran- X

Front 31

What is the pathway involved in IF gamma and IL 12? What about the feedback mechanism?

Back 31

The pathogen induces dendritic cells and macrophages to make IL12, which induces Tcells and NK cells to make IF gamma. The IF gamma induces macrophages and neutrophils to make TNF alpha and NADPH oxidase, which makes H2O2
The feedback mechanism is due to T-cells making IL10 which inhibits IF gamma, T-cells and IL12.

Front 32

Beginning with O2, NADPH, and NADPH oxide, what is the pathway to bleach.

Back 32

Superoxide is made, which combined with H ions makes H202. H2O2 combined with Cl- and MPO makes hypochlorous acid (bleach)

Front 33

Oral ulcers, gingivitis, stomatitis and cellulitis are associated with:

Back 33

Cyclic Neutropenia

Front 34

Skeletal abnormalities, exocrine pancreatic insufficieny, and pancytopenia are associated with:

Back 34

Scwachmann-Diamond

Front 35

Meningitis is assiciated with:

Back 35

Severe Congenital Neutropenia

Front 36

Infections of the oral/genital mucosa, skin, GI and respiratory tract are associated with:

Back 36

LAD Type 1

Front 37

Growth retardation, dysmorphic features and neurological deficits are associated with:

Back 37

LAD Type 2

Front 38

Recurrent perirectal abscesses are associated with:

Back 38

Rac 2

Front 39

In interleukin 12, interferon gamma defects, the defect is in signaling, and is more severe if:

Back 39

Autosomal recessive, onset in infancy.

Front 40

Mycobacterium TB, BCG, and t-cell deficiency is associated with:

Back 40

IF gamma IL 12

Front 41

Catalase positive infections are assiciated with:

Back 41

Chronic Granulomas

Front 42

Abscesses in lung, liver and brain; soft-tissue infections, facial acne, inflamed nares, and GI/UG obstruction are associated with:

Back 42

Chronic Granulomas

Front 43

What is myeloperoxidase deficiency?

Back 43

The most common defect in formation of fx neutrophil granuals. Often in conjunction with Diabetes Mellitus.

Front 44

Bleeding problems due to problems in the clotting cascade, and skin/lung infections are associated with:

Back 44

Neutrophil specific granule deficiency.

Front 45

Partial albinism, easy bruising, mild MR, and peripheral nerve defects are associated with:

Back 45

Cheiak Higashi