II: Immunodeficiency Flashcards
Immunodeficiency is increased frequency or severity of ______ (3)
infection, autoimmunity, malignancy
Name 5 infections associated with CGD.
Catalase Positive Organisms: Staph, Nocardia, Aspergillus, Serratia, Salmonella
Name a test used to evaluate the function of phagocytes.
Nitro Blue Tetrazolium (NBT) - negative in CGD
Name the most common cause of immunodeficiency
Malnutrition
Name the most common cause of primary immunodeficiency.
IgA Deficiency
Name three virulence factors of Pseudomonas?
Exotoxin A, Phospholipases, Proteases
Name two components required to form a biofilm.
Alginate, calcium
What is an appropriate therapy for CGD?
Interferon Gamma
What is the most common infection associated with CGD?
Staph aureus
What test is used for identification of VZV reactivation?
Tzanck Prep (or DFA or PCR)
Which factor deficiencies are associated with Neisseria infection?
C5-C9
Which factor deficiencies are associated with pyogenic infections?
C1-C4
Which factor is most commonly implicated in a complement deficiency?
C2
Which immune deficiency? Lysosomal trafficking defect due to microtubule dysfunction in phagosome-lysosome fusion
Chediak-Higashi Syndrome
Which immune deficiency? Decreased Th1 response due to autosomal recessively inherited defect
IL-12 receptor deficiency
Which immune deficiency? Defect in B cell maturation with many causes leading to low immunoglobulins
Combined Variable Immunodeficiency (CVID)
Which immune deficiency? Defect in tyrosine kinase gene that leads to lack of B cell maturation
Bruton Agammaglobulinemia
Which immune deficiency? Deficiency of Th17 cells due to STAT3 mutation, impaired recruitment of neutrophils to site of infection
Hyper-IgE Syndrome
Which immune deficiency? Failure to develop 3rd and 4th pharyngeal pouches leading to low T cells
DiGeorge Syndrome
Which immune deficiency? Integrin defect in phagocytes that impairs migration and chemotaxis
Leukocyte Adhesion Deficiency
Which immune deficiency? May be caused by defective IL-2 Receptor gamma chain or adenosine deaminase deficiency
Severe Combined Immunodeficiency (SCID)
Which immune deficiency? X-linked rececssive class switching defect due to mutation in CD40L on Th cells
Hyper-IgM Syndrome
Which immune deficiency? X-linked recessive mutation that impairs T cell ability to reorganize actin cytoskeleton
Wiskott-Aldrich Syndrome
Which pathogen is the top cause of mortality among nosomial and opportunistic infections?
Pseudomonas
Which pathogen? Highly resistant bacteria common in soil and water, outbreaks in ICUs, causes a variety of infections
Acinetobacter baumannii
Which pathology involves normal B and T cell numbers with low immuoglobulin?
Combined Variable Immunodeficiency (CVID)
Which pathology is notable for increasing risk of pneumococcal infection?
IgA Deficiency
Which pathology is notable for recurrent pyogenic sinopulmonary infections, often caused by Strep pneumoniae?
Combined Variable Immunodeficiency (CVID)
Which pathology may present with chronic diarrhea in teens or early 20s?
Combined Variable Immunodeficiency (CVID)
Which pathology presents mostly with respiratory infections, but also encapsulated bacteria, viruses, and Giardia?
Antibody deficiency
Which pathology presents with a defect in NADPH oxidase?
Chronic Granulomatous Disease
Which type of immunodeficiency? Susceptibility to a specific infection that may wane with age; common in the population
Private
Which type of immunodeficiency? Susceptibility to a variety of pathogens; rare in the population
Public
Which virulence factor? ADP-ribosyltransferase that inhibits protein synthesis
Exotoxin A
Which virulence factor? Aid in tissue invasion
Proteases
Which virulence factor? Enables pseudomonas to live on lung surfactant
Phospholipases
