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Pathophysiology > Immune Function > Flashcards

Immune Function Flashcards

1
Q

We have ___ lines of defense that protects us from the outside world and can signal danger and possibly get rid of the danger

A

3

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2
Q

Our ____ defense is barriers that we were born with

A

Innate

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3
Q

Is our innate defense specific or nonspecific?

A

Nonspecific

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4
Q

What are two examples of anatomical barriers that are part of our innate defense?

A

-Skin
-Mucous membranes

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5
Q

Skin has a ____ temperature than the inside of our bodies which is not conducive to many bacteria and viruses

A

Cooler

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6
Q

Endothelial cells of the skin also have ___ ___ to keep invaders out

A

Tight junctions

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7
Q

The skin also secretes ___ ___ which make it an inhospitable environment for invaders

A

Fatty acids

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8
Q

Tears and saliva also create a biochemical barrier because they are filled with enzymes like ____ that poke holes in bacteria and viruses and also create an acidic environment that invaders do not like

A

Lysozymes

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9
Q

Where are mucus membranes are present?

A

-GI and urinogenital tract
-Urethra
-Bladder
-Mouth and nose
-Lungs
(any warm and wet membranes)

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10
Q

Mucus membrane contains mucus to help ___ invaders and possibly cough them back up if they are in the lungs

A

Trap

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11
Q

There are bacteria in the gut that promote good activity and can secrete ____ to kill off bad bacteria

A

Enzymes

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12
Q

____ that line lungs and other tissues pulse mucous and invaders out of the body

A

Cilia

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13
Q

Endothelial cells secrete ____ and ____ which poke holes in bacteria cell walls

A

Cathelicidins and defensins

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14
Q

____ binds iron which keeps iron away from bacteria that could use it to reproduce

A

Lactoferrin

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15
Q

Biochemical barriers include ____ from cells

A

Secretions

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16
Q

We also have ___ ___ ___ immunity which is why we do not have to worry about getting things like avian flu

A

Species-specific natural immunity

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17
Q

____ is another type of innate, non-specific defense

A

Inflammation

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18
Q

What are three things that are involved with inflammation as an innate defense?

A

-Pattern recognition receptors
-Pathogen-associated molecular patterns
-Damage-associate molecule patterns

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19
Q

Pattern recognition receptors bind to the microbe and stick out from the ___ ___, similar to immunoglobins or antibodies

A

B cell

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20
Q

Pattern recognition receptors recognize ____ put on an invaders surface

A

Proteins

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21
Q

____-___ ___ ___ are exogenous ligands that may be part of the bacteria or virus DNA, RNA, membrane, etc.

A

Pathogen-associated molecular patterns

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22
Q

Pathogen-associated molecular patterns are present on the invaders’ surface and allow the ____ to recognize it

A

Body

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23
Q

Damage-associated molecular patterns are endogenous ligands like nucleic acids, oxidized LDL, etc, that our own body releases when ____

A

Damaged

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24
Q

Damage-associated molecular patterns will be recognized by…

A

Pattern recognition Receptors

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25
Q

Acquired defense is also known as our ____/____ immune system

A

Specific/adaptive

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26
Q

Acquired defense is when the body recognizes specific invaders and develops an immune response to that ___ ___

A

Specific invader

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27
Q

____ launches the acquired response

A

Inflammation

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28
Q

Acquired defense requires a ____ ____

A

First exposure

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29
Q

What are two types of acquired defense?

A

-Cell-mediated (T-cell mediated)
-Humoral-mediated immunity

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30
Q

What are three examples of endogenous immune modifiers?

A

-Age
-Sex
-Genetics

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31
Q

The older you are, the worse your immunity get because you have less lymphocytes, specifically ___ ___

A

T cells

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32
Q

T cells grow in the ___ ____ and this gland begins to atrophy around age 40 so we get fewer T cells and less response to pathogens and less response to pathogens

A

Thymus gland

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33
Q

As you age, other ____ and disease can also decrease your immunity

A

Comorbidities

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34
Q

____ has beneficial effects on the immune system, so women are better protected against pathogens up until menopause

A

Estrogen

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35
Q

____ are an immune modifier

A

Genetics

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36
Q

Many autoimmune diseases are part of a syndrome that have a genetic focus; many genetic disorders occur because there is a lack of ___ ___

A

Immune development

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37
Q

___/___ cells mark the cell as belonging to the host; these proteins say that this is a cell that is ours

A

MHC/HLA

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38
Q

A, B, C, E, F, G, K, L regions are class ___ MHC/HLA

A

I

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39
Q

DM, DO, DP, DQ, DR regions are class ___ MHC/HLA

A

II

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40
Q

We have 2 ____ of MHC/HLA, which is important for transplants because we need a match

A

Haplotypes

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41
Q

MHC/HLA genes are transmitted ____

A

Intact

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42
Q

Siblings have a ___ match with MHC/HLA genes

A

1/4

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43
Q

MHC class I molecule (HLA A, B, and C) participate in antigen presentation to ___ ___ cells

A

Cytotoxic T (CD8+)

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44
Q

MHC class II molecules (DP, DQ, DR) participate in antigen presentation to ___ ___ cells

A

Helper T (CD4+)

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45
Q

What are four examples of exogenous immune modifiers?

A

-Stress
-Sunlight
-Medications
-Illness

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46
Q

Stress causes a release of ____ which depresses the immune system by depressing all cell synthesis and decreasing immunoglobulin production

A

Cortisol

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47
Q

Sunlight is a ____ genetic modifier

A

Positive (helpful)

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48
Q

Sunlight helps the body make usable ___ ___ which helps with better white blood cell formation

A

Vitamin D

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49
Q

____ often suppress the immune system (cortisol)

A

Medications

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50
Q

What types of illnesses suppress the immune system?

A

-Diabetes
-CVD
(most chronic or acute diseases are bad for immune function)

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51
Q

What are 5 components of the immune system?

A

-Marrow
-Spleen
-Thymus
-Lymphatic tissue
-Specialized cells

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52
Q

The marrow is where ____ are born

A

Lymphocytes (T and B cells)

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53
Q

The spleen stores ___ that can turn into macrophages which are helpful for immune function

A

Monocytes

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54
Q

The ___ includes areas that are exposed to immune cells

A

Thymus

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55
Q

The lymphatic tissue contains…

A

MALT (Mucosa-associated lymphoid tissue); Nodes

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56
Q

What are examples of Mucosa-associated lymphoid tissue?

A

-Adenoids
-Tonsils
-Peyer’s Patch
-Appendix

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57
Q

What are 4 types of antigen-presenting cells?

A

-B cells
-Macrophages
-Dendritic cells
-Other

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58
Q

____ are small white blood cells that are not immunocompetent

A

Lymphocytes

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59
Q

Lymphocytes are in the ___ and ___ of the fetus

A

Liver and spleen

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60
Q

Lymphocytes are in the ___ ___ of children and adults

A

Bone marrow

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61
Q

Lymphocytes circulate to become committed either in the ___ ___ to the thymus or the ___ ___ to the bone marrow, and are then activated

A

T line; B line

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62
Q

There is potentially a hybrid of B and T cells called an ___ ___ which has characteristics of both and are found in people with Type 1 Diabetes

A

X lymphocyte

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63
Q

Clonal selection refers to cell ___ and ____ after first exposure (this allows for specific recognition and memory for the next time we are exposed)

A

Proliferation and differentiation

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64
Q

What are the three steps of the initial response of the immune system?

A

-Exposure and breach of barriers
-Inflammation
-Cell-mediated response (T cells)

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65
Q

If we did not have inflammation, we would get sick ___ (inflammation helps keep the response down)

A

Immediately

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66
Q

Within the cell-mediated response, the first step is…

A

T cell formation

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67
Q

Roles of T cells:

A

-Attack and destroy diseased cells
-Orchestrate, regulate, and coordinate the overall immune response
-Responsible for cell-mediated immunity
-Influences humoral immunity

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68
Q

T cells mature in the thymus where they are exposures to other ___ ___

A

Host cells

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69
Q

T cells are stimulated by ____

A

Hormones

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70
Q

T cells develop both ___ and ___ markers

A

CD4 and CD8

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71
Q

____ refers to clonal deletion and negative section which means that T cells will die in the thymus if they are too reactive to our own cells (if some of these to make it out, we hope that helper T cells would kill them)

A

Autoreactivity

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72
Q

Cytotoxic T cells (CD8) recognize MHC class ___ molecules

A

I

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73
Q

Cytotoxic T cells help rid the body of cells that have been infected by…

A

-Viruses
-Cancer cells
-Damaged cells

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74
Q

Cytotoxic T cells are also responsible for the ____ of tissue and organ grafts

A

Rejection

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75
Q

Examples of cytotoxic T cells:

A

-Killer cells (filled with perforin)
-Granzymes (causes apoptosis)

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76
Q

Helper T cells (CD4) recognize MHC class ___ molecules

A

II

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77
Q

____ helper T cells act on invaders and infected cells

A

Effector

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78
Q

____ helper T cells maintain antigen recognition/memory for the future

A

Memory

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79
Q

____ helper T cells control the immune response; they dampen the response to prevent autoimmune diseases (turn off the response when the pathogen is cleared)

A

Regulatory

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80
Q

__ __ cells do not recognize a specific antigen to kill a cell

A

Natural killer

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81
Q

Natural killer cells have ____ receptors and ____ receptors

A

Activating; inhibitory

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82
Q

Natural killer cells release…

A

-Perforin
-Granzyme
-Cytokines

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83
Q

To stop cells from killing healthy cells, natural killer cells scan the body looking for ____

A

Invaders

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84
Q

Natural killer cells are ___ independent

A

MHC

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85
Q

T cell accessory cells are produced by the ____ cell line and include monocytes, macrophages, eosinophils, neutrophils, mast cells, and basophils

A

Myeloid

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86
Q

T cell accessory cells can all become ____

A

Phagocytic

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87
Q

T cell accessory cells can be…

A

-Complement
-Cytokines
-Interleukins

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88
Q

____ are “nonself” molecules

A

Antigens

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89
Q

Antigens are present on…

A

-Bacteria, viruses, fungi, parasites
-Pollens
-Foods
-Venom
-Drugs
-Vaccines
-Transplanted tissues

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90
Q

____ are proteins produced by the body to attack and fight off antigens (they are specific to each antigen)

A

Antibodies

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91
Q

Antibodies are also known as _____

A

Immunoglobulins

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92
Q

An ___ is the region of the antigen that our body can recognize in our immune system (if there is prior exposure)

A

Epitope

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93
Q

The ____ is the part of the antibody where the antibody binds to the antigen

A

Paratope

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94
Q

____ are antigens that are too small to be recognized

A

Haptens

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95
Q

____ is the innate ability of an antigen to bind with an antibody

A

Antigenicity

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96
Q

____ is the ability of an antigen to react with T and B cells

A

Immunogenicity

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97
Q

Successful binding of the antigen receptor to the epitope results in…

A

-Neutralization
-Precipitation
-Proliferation and differentiation
-Opsonization
-Activation of the inflammatory response

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98
Q

The Ag/Ab binding and cell-mediated response includes what 4 steps?

A

-Antigen capture
-Antigen digestion
-Antigen presentation
-Response

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99
Q

Successful binding of the antigen receptor to the epitope results in ___ and ___, as well as proliferation and differentiation

A

Neutralization and precipitation

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100
Q

Successful binding of the antigen receptor to the epitope also results in stimulation of the cell to leave ___ and enter the cell cycle

A

G0

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101
Q

Repeated mitosis leads to the development of a clone of cells bearing the same ___ ___ with identical specificity

A

Antigen receptor

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102
Q

____ refers to successful binding of the antigen

A

Opsonization

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103
Q

____ are glycoproteins produced by plasma cells

A

Immunoglobulins

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104
Q

Immunoglobulins are the same as ____

A

Antibodies

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105
Q

What are the two units of an immunoglobulin molecule?

A

-Variable region
-Constant region

106
Q

An immunoglobin also has a ___ chain and a ___ chain

A

Light, heavy

107
Q

The ___ region of the immunoglobulin is the antibody binding fragment; it is variable so it rearranges until it matches the antigen and can recognize it

A

Fab

108
Q

The ___ region of the immunoglobulin releases inflammatory mediators

A

Fc

109
Q

___ describes the number of binding sites of an immunoglobulin

A

Valance

110
Q

The ____ class of immunoglobulins is the most common and has 4 subclasses (80% of circulating immunoglobulin)

A

IgG

111
Q

IgG has a valance of ___

A

2

112
Q

IgG is involved in ____ activation

A

Complement

113
Q

IgG is good ___ in the infection to control how much of the agent gets in

A

Early

114
Q

What are the two subclasses of IgA?

A

-IgA1 in blood
-IgA2 in saliva and tears

115
Q

IgA1 has a valance of ___

A

2

116
Q

IgA has a ____ role

A

Preventative

117
Q

IgA2 is a dimer with a secretory piece and has a valance of ___

A

4

118
Q

IgA has an important role in keeping the invader from getting into the body by preventing it from attaching to ___ ___

A

Mucus membranes

119
Q

____ is the first immunoglobulin produced in an infection

A

IgM

120
Q

IgM are very large (pentamer) and have a valance of ___ (but only 5 are used)

A

10

121
Q

There are small amounts of ___ in the blood

A

IgD

122
Q

IgD has a valance of ___

A

2

123
Q

IgD has a possible role in ___ synthesis

A

Ab

124
Q

IgD are also found on developing ___ ___ where the are Ag receptors

A

B lymphocytes

125
Q

____ immunoglobulins are the least concentrated in the blood

A

IgE

126
Q

IgE are involved in ___ and ___ infections

A

Allergic and parasitic

127
Q

IgE has a valance of ___

A

2

128
Q

B (Bursal) cells are present in what two places?

A

-Bone marrow
-Spleen

129
Q

____ drive specific antibody production

A

Hormones

130
Q

Mature B cells exposed to the antigen of interest become ____ cells

A

Plasma

131
Q

The humoral response from B cells is involved with…

A

-Immunoglobin secretion
-Immune memory

132
Q

Once B cells circulate through the bone marrow/spleen, they are programmed to produce ____/____ against the antigen

A

Antibodies/immunoglobins

133
Q

Once B cells are converted to plasma cells, they produce ___ ___ that are stored in the peripheral lymphoid organs

A

Memory cells

134
Q

B cell receptors have a ___ region and an ___ region

A

Fab and Fc

135
Q

B cell action allows for adaptive ___ ___ and ___

A

Immune response and memory

136
Q

What are three types of B cells:

A

-B cells
-Memory B cells
-Plasma B cells

137
Q

What are the 4 phases of infection and immune response?

A
  1. Establishment of infection
  2. Induction of adaptive response
  3. Adaptive immune response
  4. Immunological memory
138
Q

Within the cell-mediated response, there is ___-___ complex binding which is responsible for clonal expansion

A

MHC-Ag

139
Q

The MHC-Ag complex binding signals to ___, ___, and ___ cells

A

Tc, Th, B

140
Q

Cytotoxic T cells (Tc) release…

A

-Perforin
-Granulysine
-Granzymes

141
Q

With the MHC-Tc interaction, ___ receptor binds to Class I HC molecules to release perforin, granulysine, and other granulocytes

A

CD8

142
Q

In the MHC-Tc interaction, the B cell presents the antigen, and then the B cell puts it in class 2 molecules so ____ binds to the peptide to cause release of cytokines

A

CD4

143
Q

What are three types of Th cells within the cell-mediated response?

A

-Effector Th cells
-Memory Th cells
-Suppressor Th cells

144
Q

Following the initial exposure, there is the ___ ___

A

Primary response

145
Q

The primary response includes both ___ ___ and ____ response

A

Cell-mediated and humoral

146
Q

Cell mediated response requires ___ and ____ cells

A

Th and Tc

147
Q

The humoral response requires ___ ___ to produce plasma cells and immunoglobulins

A

B cells

148
Q

5-7 days after the primary response, we will have ___ response

A

IgM

149
Q

With a ____ response, you are exposed to the same antigen again

A

Secondary

150
Q

In a secondary response, there are ___ cells available

A

Specific

151
Q

The secondary is a more ____ response

A

Vigorous

152
Q

What types of memory cells are available for a secondary response?

A

-Plasma cells
-B cells

153
Q

____ refers to a specific antibody that is able to be recognized without processing

A

Specificity

154
Q

The difference between a primary and secondary response is that secondary has…

A

-Decreased reactivity time
-More severe reaction
-More IgG
-Memory

155
Q

The first vaccine was created by doctor ___ after he realized that milkmaids who go cowpox were immune to smallpox, so he took the virus and injected it into people

A

Jenner

156
Q

When the immune system function goes awry, it is classified into what three broad categories?

A

-Inappropriate function
-Too much function
-Too little function

157
Q

____ reactions occur when a given stimulus initiates an abnormal/undesirable immune response to a non-threat

A

Hypersensitivity

158
Q

There are ___ classes of hypersensitivity reaction

A

4

159
Q

Types I-III hypersensitivity reactions are ____ mediated

A

Humoral (antibody)

160
Q

Type IV hypersensitivity reaction is ___-___

A

Cell-mediated

161
Q

Type I hypersensitivity reaction is ___ ___ ____

A

Immediate type hypersensitivity

162
Q

The “normal” role of the Type I reaction is to initiate an ___ ___ to neutralize and expel intestinal parasites

A

Inflammatory response

163
Q

Type I hypersensitivity reactions use granules that contain…

A

-Histamine
-Leukotrienes and prostaglandin
-Heparin
-Serotonin
-Cytokines, interleukins, inflammatory mediators

164
Q

Manifestations of type I hypersensitivity are related to ___ content actions

A

Granule

165
Q

Some examples of manifestations of type I hypersensitivity include…

A

-Vasodilation
-Smooth muscle and endothelial cell constriction
-Integumentary systems

166
Q

Type II hypersensitivity reactions are ___ ___ ____

A

Antibody-mediated toxicities

167
Q

Type II hypersensitivity reactions are further subdivided into what two categories?

A

-Cytolytic reactions
-Antibody mediated

168
Q

The normal function of a cytolytic/cytotoxic reaction is…

A

Lysis of bacteria and other pathogens

169
Q

Antibody-mediated reactions would normally function to…

A

-Neutralize toxins
-Viral receptor blockade

170
Q

With an antibody-mediated reaction, an antibody against a functional molecule or receptor results in their activation or inactivation because the self-cell is identified as ____

A

Foreign

171
Q

Examples of antibody-mediated reactions (type II hypersensitivity):

A

-Acquired hemophilia
-Grave’s disease
-Myasthenia Gravis

172
Q

Type III hypersensitivity reactions are “___ ___” disorders

A

Immune complex

173
Q

Normal function of type III hypersensitivity reactions is ___ ___ at the site of pathogen invasion (vessel walls, joints, kidneys)

A

Acute inflammation

174
Q

With type III hypersensitivity, complement is activated and ___ and ___ are released; neutrophils are attracted by C5a and they release enzymes that destroy the endothelium and red blood cells escape from within the blood vessels

A

C3a and C5a

175
Q

An example of type III hypersensitivity is ___ ___

A

Serum sickness

176
Q

Serum sickness occurs __-__ days after non-human sera injection

A

10-14

177
Q

Serum sickness results in…

A

-Vasculitis
-Glomerulonephritis
-Arthralgia
-Fever

178
Q

Type IV hypersensitivity reactions are ___ ___ ___

A

Delayed type hypersensitivity

179
Q

Normal function of type IV hypersensitivity is to control ____ by mycobacteria, treponema, other bacteria, fungi, viruses, and parasites

A

Infections

180
Q

Type IV hypersensitivity reactions are classified into what two subtypes?

A

-T cell mediated delayed hypersensitivity
-T cell mediated cytotoxicity

181
Q

The normal function of type IV delayed hypersensitivity is to destroy ___ and ___ ___ cells

A

Invaders and viral infected

182
Q

In type IV delayed hypersensitivity, sensitized Th cells bind to the ____-___ ____, releasing cytokines, causing inflammation and tissue damage

A

MHCII-antigen complex

183
Q

Examples of type IV delayed hypersensitivity:

A

-Response to intradermal injection of mycobacterium tuberculosis-derived purified protein derivative (TB testing/PPD testing)
-Multiple sclerosis
-Direct activation of type IV hypersensitivity reactions also occur due to “superantigen” exposure
-Transplant rejection

184
Q

The normal function of type IV cytotoxic reactions is to…

A

Destroy invaders and viral infected cells

185
Q

With type IV cytotoxic reactions, ___ cells recognize MHCI-presenting cells and immediately destroy the cell

A

Tc

186
Q

Examples of type IV hypersensitivity cytotoxic reactions:

A

-Contact dermatitis
-Granulomatous inflammation
-Transplant rejections

187
Q

Transplant rejection reactions occur when transplanted tissue is not recognized as ___ ___ due to the immune response and MHC genotyping

A

Host tissue

188
Q

Transplant rejection occurs in what 3 categories?

A

-Hyperacute rejection
-Acute rejection
-Chronic rejection

189
Q

Hyperacute rejection happens in the first ___ to ___ after transplantation

A

Minutes to hours

190
Q

Hyperacute rejection occurs due to pre-existing ____ that cause a massive inflammatory and immune response

A

Antibodies

191
Q

Hyperacute rejection requires ___ ___

A

Immediate treatment

192
Q

Acute transplant rejection reactions occur in the first ___ to ___ after transplant

A

Weeks to months

193
Q

With acute rejection, the ___ itself is being attacked

A

Graft

194
Q

Acute rejection may be reversed with ____

A

Immunosuppression

195
Q

A chronic rejection reaction occurs ___ to ___ after the transplant

A

Months to years

196
Q

Repeat episodes of __ rejection can result in chronic rejection

A

Acute

197
Q

Chronic rejection reactions do not respond well to ____

A

Therapy

198
Q

Graft versus host disease is a different type of reaction only seen in ___ ___ transplant

A

Bone marrow

199
Q

With GVHD, the transplanted cells produce immune cells that attack the ____

A

Host

200
Q

___ ___ occur when the immune system fails to recognize ‘self’ markers and attacks the ‘self’ tissue

A

Autoimmune disorders

201
Q

Autoimmune disorders are ____-____ and ___-___ disorders

A

Collagen-vascular and connective-tissue

202
Q

Autoimmune disorders are primarily ___ ___ ____ reactions

A

Type III hypersensitivity reactions (some type II characteristics and mechanisms)

203
Q

Are autoimmune disorders more common in males or females?

A

Females

204
Q

Autoimmune disorders can be ___ ___ or ____ diseases

A

Single organ or systemic

205
Q

Autoimmune disorders result from a breakdown of ___ ___

A

Self-tolerance

206
Q

What 3 major mechanisms provide for self tolerance?

A

-Clonal deletion (self-reactive cells)
-Clonal anergy (failure to respond to antigen)
-Peripheral suppression of/by T cells

207
Q

What are some examples of single organ autoimmune disorders?

A

-Hashimoto’s thyroiditis
-Goodpasture’s syndrome
-Autoimmune thrombocytopenia
-Insulin-dependent diabetes mellitus
-Myasthenia gravis
-Grave’s disease

208
Q

Hashimoto’s thyroiditis is a _____ (T cells) attack on thyroid cells

A

Lymphocytic

209
Q

Hashimoto’s affects ___-___ of every 1,000 Americans

A

1-1.5

210
Q

Does Hashimoto’s affect more women or men?

A

Women

211
Q

Hashimoto’s usually affects people between ___-___ years old

A

45-65

212
Q

With Hashimoto’s, the functioning thyroid cells will secret more ____, causing hyperthyroid symptoms which may be asymptomatic, or cause increased metabolism/energy (people feel good)

A

Thyroxine

213
Q

Then, fibrosis occurs and inadequate thyroxine is produced, leading to ____ symptoms like decreased metabolism, weight gain, fatigue

A

Hypothyroid

214
Q

What are three examples of systemic autoimmune disorders?

A

-Systemic Lupus Erythematosus
-Rheumatoid arthritis
-Sjogren’s syndrome

215
Q

Systemic lupus erythematosus is a failure to maintain ___ ___

A

Self tolerance

216
Q

Lupus affects ___ of every 100,000 people

A

53

217
Q

Is Lupus more common in men or women?

A

Women

218
Q

Lupus occurs more commonly, and with a more severe presentation, in what population?

A

Black Americans

219
Q

What are three possible mechanisms of Lupus?

A

-Failure to maintain self-tolerance
-Genetic and environmental factors
-Possibly EBV

220
Q

Manifestations of Lupus:

A

-Low-grade fever
-Photosensitivity
-Ulcers in mouth and nose
-Muscle aches
-Arthritis
-Fatigue
-Loss of appetite
-Butterfly rash on the face
-Inflammation of the pleura and pericardium
-Poor circulation of fingers and toes

221
Q

Inadequate immune response falls into one of what two categories?

A

-Primary immunodeficiency is present at birth
-Secondary immunodeficiency is an acquired disorder

222
Q

Whether primary or secondary immunodeficiency, the end result is poor immune response and frequent ___ ___

A

Opportunistic infections

223
Q

Brunton’s Disease is a ____ ____ disease

A

Primary immunodeficiency

224
Q

Bruton’s disease affects ___ in 100,000 male infants at 5-6 months old which is when T-lymphocyes die off

A

1

225
Q

Bruton’s disease is a ____ disease

A

Genetic

226
Q

Bruton’s disease is also called…

A

X-linked agammaglobulinemia

227
Q

Bruton’s disease is caused by a ___ gene on the X chromosome

A

Bkt

228
Q

The normal Bkt gene product promotes ___ ___ maturation before release into the bloodstream

A

B cell

229
Q

Bruton’s disease causes B cells to not ___, which leads to the absence of all types of immunoglobulins

A

Mature

230
Q

People with Bruton’s disease are prone to recurrent ___ ___

A

Pyogenic infections

231
Q

DiGeorge Syndrome is a severe ___ ___ deficiency

A

T cell

232
Q

DiGeorge syndrome affects ___ in ___ people

A

1 in 4000

233
Q

DiGeorge syndrome causes significant ___ ___

A

Congenial defects

234
Q

We can remember the symptoms of DiGeorge Syndrome with ____-___

A

CATCH-22

235
Q

CATCH-22 stands for…

A

-Cardiac anomaly
-Abnormal facies
-T cell deficit
-Cleft palate
-Hypocalcemia
-Defect on chromosome 22

236
Q

Manifestations of CATCH-22 include…

A

-Cognitive impairments
-Delayed speech
-Psychotic episode
-Death

237
Q

Severe combined immunodeficiency (SCID) is also known as ___ ___ ____

A

Bubble Boy Disease

238
Q

SCID is a severe ____ ____

A

Primary immunodeficiency

239
Q

SCID affects 1 in ___-___

A

50,000-100,000

240
Q

SCID is genetic and ___-linked and there may or may not be other chromosomes involved

A

X

241
Q

Other genes affected by SCID may be:

A

-Common gamma chain of interleukins
-Adenosine deaminase
-Purine nucleoside phosphorylase
-RAG1/RAG2
-TAP

242
Q

SCID is hallmarked by severe __ and __ cell defect

A

T and B

243
Q

With SCID, ___ cells and ___ production are also defective

A

Plasma cells and immunoglobulin

244
Q

Manifestations of SCID include…

A

-Severe, chronic/recurrent infections of all types
-Usually diagnosed at about 6 months of age when the child presents with multiple infections (newborn screening)

245
Q

Treatment for SCID includes…

A

-Bone marrow transplant
-Total isolation
-Stem cell transplantation (rejection might be an issue)
-Gene therapy (leukemia)

246
Q

AIDS is a ____ immune disorder

A

Secondary

247
Q

There is an AIDS pandemic, affecting ___% of the worldwide population

A

0.6

248
Q

HIV attacks ___ cells

A

CD4+

249
Q

HIV gets into cells via ___ (early) or ____ (later) receptors

A

CCR5; CXCR4

250
Q

HIV causes ___ ___ destruction

A

T cell

251
Q

HIV leads to a loss of ___ ___ ___

A

Cell-mediated immunity

252
Q

Three stages of HIV/AIDS:

A

-Early symptoms
-Progression
-Conversion to AIDS

253
Q

Treatment of HIV includes…

A

-Prevention
-Antiretroviral therapy

254
Q

Currently, there is no cure or ____ for HIV/AIDS

A

Vaccine

255
Q

A ___ ___ ____ for HIV/AIDS has had limited success (2/30 cured, most died), until 2007

A

Bone marrow transplant

256
Q

The ___ ___ had acute myeloid leukemia and HIV; he was given chemotherapy and two bone marrow transplants (CCR5 delta 32 mutation; no CXCR4 genotyping)

A

Berlin Patient

257
Q

Since the second transplant, the patient has been off of ____

A

Antiretroviral therapy

258
Q

In a 2010 follow-up with the Berlin Patient, HIV was undetectable since ___ days post 1st bone marrow transplant; he also has normal CD4 T cell counts

A

60

259
Q

Disadvantages with bone marrow transplant for AIDS:

A

-Few CCR5 delta 32 donors
-Costly
-High risk procedure

260
Q

Vaccine development for AIDS is using traditional methods as well as…

A

RNAi (silencing the CCR5)

Pathophysiology (18 decks)

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