assessment 7

Question 1

Dx that is a result of genetic deficiency of FoxP3 in Tregs  autoimmunity :

Answer 1

IPEX

Question 2

Type of virus – influenza?

Answer 2

Negative sense RNA virus

Question 3

Antigenic shift causes huge change in this influenza protein:

Answer 3

hemagluttination

Question 4

Insect born protozoa that replicate in EC tissue spaces:

Answer 4

trypanosomes

Question 5

Trypanosomes coated with single type of glycoprotein:

Answer 5

VSG

Question 6

Trypanosome evasion of host immune system?

Answer 6

Programmed re-arrangement of DNA: cassette system to express only 1 of 1000 different VSG (variant specific glycoprotein)

Question 7

State in which no viral proteins produced (no disease caused, no CTL’s active) –

Answer 7

Latency

Question 8

Herpes simplex virus occupies what cells?

Answer 8

Nerve

Question 9

Why are neurons susceptible to viral infections?

Answer 9

Low # MHC I to decrease unnecessary killing by CTL’s

Question 10

Mononucleosis is caused by?

Answer 10

Epstein-Barr Virus

Question 11

Epstein-Barr infects what cells?

Answer 11

Causes them to proliferate until they are killed by CTL’s: B Cells

Question 12

Pathogens most likely to subvert host defenses?

Answer 12

Viruses

Question 13

Mycobacterium TB resistance to immune system?

Answer 13

Prevents fusions of lysosome and phagosome

Question 14

Listeria evasion of immune system?

Answer 14

Escapes phagosome until macrophage killed by CTLs

Question 15

Toxoplasma gondii evasion of the immune system?

Answer 15

Makes its own vesicles inside phagosome

Question 16

What produces toxic shock syndrome toxin 1?

Answer 16

Staphylococcus

Question 17

What does a super antigen bind?

Answer 17

Vb region of TCR and MHC II

Question 18

Superantigen activates… % T cells?

Answer 18

2%-20%

Question 19

Superantigen causes…

Answer 19

Massive release of cytokines

Question 20

Phase of Ag specific Antibody present and detectable in the serum

Answer 20

Seroconversion

Question 21

Lentivirus? Example?

Answer 21

slow, HIV

Question 22

HIV: profound decrease in?

Answer 22

CD4 cells

Question 23

HIV binds with high affinity to_____ and must interact with_____ to gain entry into the cell.

Answer 23

CD4 and chemokine receptor

Question 24

Antigenic variation in HIV is due to _____?

Answer 24

Error prone reverse transcriptase

Question 25

Asplenia increases susceptibility to?

Answer 25

Septic infections (encapsulated bacteria)

Question 26

Neutropenia is a deficiency in _____? must be less than ____ cell/ul?

Answer 26

Granulocytes, <500 cells/ul

Question 27

Severe congenital neutropenia called____? defect?

Answer 27

Kostman syndrome, G-CSF or its receptor

Question 28

Autosomal dominant type of neutropenia that occurs every 2-4 weeks, defect in ELA-2?

Answer 28

Cyclic neutropenia

Question 29

Absence/non functional NK and NKT cells?

Answer 29

Absolute NKD

Question 30

Absence of NK cells/ function?

Answer 30

Classical NKD

Question 31

Loss of NK cell fuction?

Answer 31

Functional NKD

Question 32

Most toll signals through what Trx factor?

Answer 32

NF-KB

Question 33

X linked hypohydrotic ectodermal dysplasia immunodeficiency= defect in what protein?

Answer 33

NEMO

Question 34

Usual results of deficiencies in complements?

Answer 34

EC bacteria

Question 35

Deficiencies in complement regulatory proteins can cause what 2 problems?

Answer 35

1. Encapsulated bacterial susceptibility due to depletion of Cs 2. Destruction of RBCs

Question 36

Mannose Binding Lectin Deficiency: increases … meningitis?

Answer 36

Neisseria

Question 37

Paroxymal Nocturnal Hemoglobinuria caused by? Complement induced intravascular hemolytic anemia due to def … and …

Answer 37

DAF and CD59 (Protects host cells)

Question 38

X-linked agammaglobulinemia caused by defect in? Vulnerable to? ……; leads to lack of signal transduction in B cells; 2 pathogens?

Answer 38

Bruton's tyrosine kinase leads to lack of signal transduction in B cells; EC bacteria and cannot neutralize viruses

Question 39

Surrogate light chain that pairs with mu heavy chain? (deficiency causes lack of pre-B receptors)

Answer 39

Gamma 5

Question 40

2 mutations that can cause X-linked hyper IgM syndrome:

Answer 40

1- defect in CD40L (no 2nd activation) 2-deficiency in activation induced cytidine deaminase (cannot isotype switch or somatic hypermutate)

Question 41

IgA deficiency leads to increased susceptibility to?

Answer 41

Parasites

Question 42

Selective IgG deficiency – most common in kids? In adults? Kids: … and adults ….

Answer 42

Kids: G2 and adults G3

Question 43

Group of 150 primary immunodeficiencies: reduced levels of antibodies; most common primary immunodeficiency disorder: (dx in 20s or 30s):

Answer 43

common variable immunodeficiency

Question 44

Classical bare lymphocyte syndrome is a deficiency in …?

Answer 44

MHC II; no positive selection of CD4 cells in thymus

Question 45

2 defects to cause defect in CD8 function?

Answer 45

1-TAP deficiency 2-CD8 alpha chain defect

Question 46

Defect in cytoskeletal reorganization that is needed for T cells to deliver cytokines and other signals to B cells and macrophages – …… (SCID – T cells cannot talk to B cells)

Answer 46

Wiskott-Aldrich syndrome

Question 47

Adenosine deaminase deficiency (ADA) or purine nucleotide phosphorylase def results in?

Answer 47

Accumulation of toxic nucleotide catabolites that kill developing B and T cells

Question 48

CD4 defects can result in ______?

Answer 48

CD4 defects can result in… SCID (severe combined immunodeficiency – CD4 are important for cell-mediated and humoral response)

Question 49

Common gamma chain def, Jak3, and CD3 def result in?

Answer 49

defects in T cell function causing SCID

Question 50

Mis-sense mutation that partially inactivates RAG causing absence of B cells and oligoclonal autoreactive T-cells – (shares a phenotype with common gamma chain def)

Answer 50

Omenn Syndrome

Question 51

DiGeorge results in small deletion in what chromosome resulting in what?

Answer 51

Chromosome 22, no thymus

Question 52

APECED, results in a myriad of autoimmune diseases due to a deficiency in what?

Answer 52

AIRE

Question 53

IPEX , onset 1st year of life: 3 symptoms?

Answer 53

Watery diarrhea, eczematous dermatitis, endocrinopathy

Question 54

Role of Factor P (properdin) – enhances…

Answer 54

enhances the alternative complement pathway (prevents destruction of C3b)

Question 55

Genetic dx characterized by lymphadenopathy and splenomegaly: immune cells fail to … following immune response --> overpopulation of secondary lymphoid tissue:

Answer 55

ALPS (autoimmune lymphoproliferative syndrome)

Question 56

Delivery of initial smallpox vaccine –

Answer 56

variolation

Question 57

Virus used to vaccinate against smallpox (cow pox)

Answer 57

vaccinia (vacus = cow)

Question 58

Only part of pathogen is used as immunogen =

Answer 58

subunit vaccine

Question 59

Inactivated toxin

Answer 59

toxoid

Question 60

Most widely used live bacterial vaccine that is not used in the US is used against? And called?

Answer 60

BCG Bacille-Calmette –Guerin; Mycobacterium tuberculosis

Question 61

What does DTP vaccine consist of and what does it target?

Answer 61

Diphtheria & tetanus toxoids combined with killed Bordetalla pertussis (whopping cough)

Question 62

2 killed virus vaccines?

Answer 62

Salk polio, influenza

Question 63

Most anti-virals are what?

Answer 63

Live attenuated vaccines

Question 64

Name 4 live-attenuted viral vaccines:

Answer 64

measles, mumps, Sabin polio, yellow fever

Question 65

Substance that enhances the immunogenicity of the antigen –

Answer 65

Adjuvant

Question 66

Adjuvants can turn a … protein antigen into a … antigen which is more readily taken up by APCs

Answer 66

soluble > particulate

Question 67

What is added as a source of PAMPs and to increase inflammation around vaccine?

Answer 67

Mycobacterial cell wall components