CHP 14 - Immunodificiency

Question 1

What is the difference between primary immunodeficiency vs secondary immunodeficiency?

Answer 1

Primary immunodeficiency is an inheritance immune disorder whereas secondary is an immune disorder that occurs due to non-inherited factors

Question 2

What are factors that could lead to immunodefiency disorders?

Answer 2

Malnutrition, age, infection, disease, drugs, or exposure to toxins

Question 3

Omenn syndrome patients lack the ________ protein for ___________.

Answer 3

RAG2; Somatic recombination

Question 4

What is the function of RAG2 in B cells and T cells?

Answer 4

Somatic recombination of the VDJ segments of the immunoglobulins or T cell receptor to amplify receptor diversity

Question 5

AIDS is a primary or secondary immunodeficiency disorder?

Answer 5

Secondary because it was acquired through a viral infection

Question 6

What determines the severity of disease?

Answer 6

Severe immunodeficiencies result from issues in immune cell development

Tolerable immunodeficiencies result from limited effects in selected cells

Frequency

Question 7

Immunodeficiency in ___ cells are the most common inherited immunodeficiency

Answer 7

B cells

Question 8

T cell and innate cell immunodeficiencies usually cause _________ morbidity

Answer 8

Severe

Question 9

B cell immunodeficiencies can be ___________.

Answer 9

Tolerable

Question 10

Mutations in C3 prevent the ___________ __________. These patients are susceptible to gram (+/-) bacteria

Answer 10

Complement system; gram - bacteria

Question 11

What are the three major functions of the complement system?

Answer 11

1) enhance opsonization which results in more phagocytosis
2) Pathogen lysis through the MAC (membrane attack complex)
3) recruitment of other innate cells

Question 12

Patients who hace Omenn syndrome which lack both T cells and B cells can develop __________ and are susceptible to infection by many types of microbes

Answer 12

SCID – severe combined immunodeficiency

Question 13

HIV targets/infects which immune cells?

Hint: Adaptive Immune system, be specific

Answer 13

Cd4 T cells

Question 14

Mutation in C3 result in susceptibility to _________ infection

Answer 14

Microbial

Question 15

Mutation in C5-C9 genes result in susceptibility to?

Answer 15

Some bacterial infection specifically gram negative bacteria

Question 16

Loss of phagocytosis would leads to increase susceptibility to infection by (extra/intra)cellular pathogens

Answer 16

Extracellular pathogens

Question 17

What is Leukocyte adhesion deficiency?

Answer 17

luekocytes inability to properly bind to pathogen to initiate phagocytosis

Question 18

Leukocytes adhesion deficiency is the lack of _______ adhesion molecules

Answer 18

cell surface

Question 19

What is Chediak-Higashi syndrome?

Answer 19

The inability for the phagosome to fuse with lysosomes therefore unable to digest or breakdown pathogens

Question 20

What is chronic granulomatous?

Answer 20

phagocytes (neutrophils) inability to destroy pathogens because granulocytes are not working properly

Question 21

LAD can be due to mutations in ______ intergrin that is shared by ______,______, and _______ adhesion proteins

Answer 21

B2;LFA-1,CR3; and CR4

Question 22

Loss of LFA-1 impairs?

Answer 22

innate cell migration to sites of infection

Question 23

Loss of CR3-CR4 results in an ability to

Answer 23

Recognize C3b on pathogens

Question 24

Which gene is responsible for the formation of the phagolysosome

Answer 24

LYST gene

Question 25

Patients with Chediak-Higashi syndrome are more susceptible to infections from which species of bacteria?

Answer 25

Staphylococcus and streptolococcus

Question 26

Chronic granulomatous disease is a result of mutation in genes responsible for the function of?

Answer 26

NADPH oxidase

Question 27

What is the function of NADPH oxidase?

Answer 27

create high oxygenated areas resulting in respiratory burst, this allows for digestion of pathogens

Question 28

Which disease is characterized with inefficient killing of microbes result in chronic inflammation and formation of granulomas

Answer 28

Chronic granulomatous disease

Question 29

A mutation on which transcription factor would lead to classical NK cell deficiency?

Answer 29

GAT3

Question 30

Functional NK cell deficiency is a mutation on which gene? why is this gene important

Answer 30

Gene that codes for the recptor FcgammaR; this receptor is a costimulatory receptor that helper t cells must bind to for cytoxicity to occur

Question 31

Scid is

Answer 31

Severe combined immunodeficiencies