immunodeficiency

Question 1

what are inborn (primary) and acquired immunodefiency (secondary)

Answer 1

inborn= present from birth. can be inherited or mutation (recessive or dominance).
lead to minor increased risk of infection or inability to control any infection

acquired= can occur any time after birth.
due to malnutrition, infection, age, use of certain drugs

Question 2

inborn
-diagnosis
-disease in cells

Answer 2

-recurrent infection in children. due to muation in one or multiple genes

-B cells/ antibody,
T and B cell combined (x-linked servere combined immunodefiency),
T cell (hyper igM syndrome)
phagocytes (chronic granulomatous disease)
-complement

Question 3

examples of inate immunodeficiency
1) T and B cell combined = x linked severe combined immunodefiency
-who does it occur in
-what gene does it defect
-what happened to david vetter
-how to prevent EBV
-what is ADA and treatment

Answer 3

-occurs more in males as x linked
-defect in gene IL2 receptor gamma
-received bone marrow transplant from his sister, but led to him getting EBV. he died.
-to prevent EBV screen bone marrow
-ADA is a mutation that harms lymphocytes and immune systum, the treatment is bone marrow transplant

Question 4

2) T cell (hyper-igM syndrome)
-what mutation in T cell molecule gene does the defect occur in
-the response of the mutation

Answer 4

-CD40L gene
-leads to poor antibody response

Question 5

3) phagocytes (chronic granulomatous disease)
-what enzyme is defected
-impacts of this mutation
-impacts on phagocytes

Answer 5

-NADPH defects
-means cells have no superoxide burst
-therefore phagocytes cannot kill pathogens they have phagocytosed.

Question 6

why live attenutaed vaccines can be dangerous for people with immunodefiencies
e.g measules, polio and rotavirus

Answer 6

in people with primary immunodefeincies vaccination can lead to severe diseases

Question 7

examples of acquired immunodefiency
1) HIV infection
-what is it?
-treatment

Answer 7

depletion of CD4 helper T cells
-treatment with antiretrovial therapty to prevent development of aids

Question 8

2) malnutrition
-what is it
-why they get it
-complex relationship that leads to mortaility

Answer 8

-inhibits lymphocyte maturation and function
-they make poor responses to vaccines
-loads of factors can lead to malnutrition

Question 9

3) removal of immune organs thymus and spleem

Answer 9

thymus- reduced T cell
spleen- decreased phagocyte of microbes

Question 10

4) autoimmune immunodefiencies
-what virus does it relate to severe outcomes in
-what antibodies does some people have against immune molecules e.g. cytokines
-treatment

Answer 10

-covid-19
-type 1 and 2 interferons, IL-17
-people with autoantibodies
-drugs that deplete B cells or plasma cells