II: Immunodeficiency

Question 1

Immunodeficiency is increased frequency or severity of ______ (3)

Answer 1

infection, autoimmunity, malignancy

Question 2

Name 5 infections associated with CGD.

Answer 2

Catalase Positive Organisms: Staph, Nocardia, Aspergillus, Serratia, Salmonella

Question 3

Name a test used to evaluate the function of phagocytes.

Answer 3

Nitro Blue Tetrazolium (NBT) - negative in CGD

Question 4

Name the most common cause of immunodeficiency

Answer 4

Malnutrition

Question 5

Name the most common cause of primary immunodeficiency.

Answer 5

IgA Deficiency

Question 6

Name three virulence factors of Pseudomonas?

Answer 6

Exotoxin A, Phospholipases, Proteases

Question 7

Name two components required to form a biofilm.

Answer 7

Alginate, calcium

Question 8

What is an appropriate therapy for CGD?

Answer 8

Interferon Gamma

Question 9

What is the most common infection associated with CGD?

Answer 9

Staph aureus

Question 10

What test is used for identification of VZV reactivation?

Answer 10

Tzanck Prep (or DFA or PCR)

Question 11

Which factor deficiencies are associated with Neisseria infection?

Answer 11

C5-C9

Question 12

Which factor deficiencies are associated with pyogenic infections?

Answer 12

C1-C4

Question 13

Which factor is most commonly implicated in a complement deficiency?

Answer 13

C2

Question 14

Which immune deficiency? Lysosomal trafficking defect due to microtubule dysfunction in phagosome-lysosome fusion

Answer 14

Chediak-Higashi Syndrome

Question 15

Which immune deficiency? Decreased Th1 response due to autosomal recessively inherited defect

Answer 15

IL-12 receptor deficiency

Question 16

Which immune deficiency? Defect in B cell maturation with many causes leading to low immunoglobulins

Answer 16

Combined Variable Immunodeficiency (CVID)

Question 17

Which immune deficiency? Defect in tyrosine kinase gene that leads to lack of B cell maturation

Answer 17

Bruton Agammaglobulinemia

Question 18

Which immune deficiency? Deficiency of Th17 cells due to STAT3 mutation, impaired recruitment of neutrophils to site of infection

Answer 18

Hyper-IgE Syndrome

Question 19

Which immune deficiency? Failure to develop 3rd and 4th pharyngeal pouches leading to low T cells

Answer 19

DiGeorge Syndrome

Question 20

Which immune deficiency? Integrin defect in phagocytes that impairs migration and chemotaxis

Answer 20

Leukocyte Adhesion Deficiency

Question 21

Which immune deficiency? May be caused by defective IL-2 Receptor gamma chain or adenosine deaminase deficiency

Answer 21

Severe Combined Immunodeficiency (SCID)

Question 22

Which immune deficiency? X-linked rececssive class switching defect due to mutation in CD40L on Th cells

Answer 22

Hyper-IgM Syndrome

Question 23

Which immune deficiency? X-linked recessive mutation that impairs T cell ability to reorganize actin cytoskeleton

Answer 23

Wiskott-Aldrich Syndrome

Question 24

Which pathogen is the top cause of mortality among nosomial and opportunistic infections?

Answer 24

Pseudomonas

Question 25

Which pathogen? Highly resistant bacteria common in soil and water, outbreaks in ICUs, causes a variety of infections

Answer 25

Acinetobacter baumannii

Question 26

Which pathology involves normal B and T cell numbers with low immuoglobulin?

Answer 26

Combined Variable Immunodeficiency (CVID)

Question 27

Which pathology is notable for increasing risk of pneumococcal infection?

Answer 27

IgA Deficiency

Question 28

Which pathology is notable for recurrent pyogenic sinopulmonary infections, often caused by Strep pneumoniae?

Answer 28

Combined Variable Immunodeficiency (CVID)

Question 29

Which pathology may present with chronic diarrhea in teens or early 20s?

Answer 29

Combined Variable Immunodeficiency (CVID)

Question 30

Which pathology presents mostly with respiratory infections, but also encapsulated bacteria, viruses, and Giardia?

Answer 30

Antibody deficiency

Question 31

Which pathology presents with a defect in NADPH oxidase?

Answer 31

Chronic Granulomatous Disease

Question 32

Which type of immunodeficiency? Susceptibility to a specific infection that may wane with age; common in the population

Answer 32

Private

Question 33

Which type of immunodeficiency? Susceptibility to a variety of pathogens; rare in the population

Answer 33

Public

Question 34

Which virulence factor? ADP-ribosyltransferase that inhibits protein synthesis

Answer 34

Exotoxin A

Question 35

Which virulence factor? Aid in tissue invasion

Answer 35

Proteases

Question 36

Which virulence factor? Enables pseudomonas to live on lung surfactant

Answer 36

Phospholipases