CSIM 1.31 - Immunodeficiency

Question 1

What is the role of complement proteins?

Answer 1

Opsinisation
Immune-complex clearance
Direct lysis (MAC)

Question 2

What is the role of neutrophils?

Answer 2

Phagacytosis and activation of bactericidal mechanisms

Question 3

Which antibodies are transferred through the placenta and which are transferred during breastfeeding?

Answer 3

IgG - placental transfer

IGA - breast feeding

Question 4

What is agammagloulinaemia?

Answer 4

X-lined//autosomal recessive condition where there is a failure of B-cell maturation - NO plasma cells are made for antibody secretion

Question 5

Defects in phagocyte production may lead to which clinical condition?

Answer 5

Neutropenia

Question 6

Defects in adhesion molecules (affecting chemotaxis) may lead to which clinical conditions?

Answer 6

Leukocyte adhesion (I-III)

Question 7

Defects of superoxide O2- ions (produced in phagosome) may lead to which clinical conditions?

Answer 7

Chronic granulomatous disease (deficient NADPH oxidase)

Question 8

Defects in complement protein production of C2 and C4 may lead to which clinical conditions?

Answer 8

Systemic lupus erythematosus

Question 9

Defects in the C1-esterase inhibitor within the complement system may lead to which clinical conditions?

Answer 9

Hereditary angioedema

Question 10

What is severe combined immune deficiency?

Answer 10

Combined absence of B-cell and T-cell

Presentation within 3 months of persistent and severe infections

Question 11

Define immunodeficiency?

Answer 11

Failure to achieve immune function to provide efficient self-limited host defence against environment whilst preserving tolerance to self