Primary Immunodeficiency

Question 1

what can the abnormality be in?

Answer 1

components of innate
stages of lymphocyte development
responses of mature lymphocytes to antigenic stimulation

Question 2

repeated infection with encapsulated bacteria is a sign of defective?

Answer 2

antibody production

Question 3

B cell deficiency leads to?

Answer 3

pyogenic bacteria, enteric bacteria and viruses

Question 4

IgG and IgA deficiency leads to?

Answer 4

recurrent respiratory infection by pneumococcus and haemophillus

Question 5

what infections when reduced number/function of phagocytes?

Answer 5

staphylococci, gram-negative bacteria and fungi

Question 6

what does defects in T cells lead to?

Answer 6

intracellular infections: protozoa, viruses, mycobacteria

Question 7

what is recurrent candida infections suggestive of?

Answer 7

defects in TH17 pathway

Question 8

causes of primary immunodeficiency?

Answer 8

mutations
polymorphisms
polygenic disorders

Question 9

clinical presentation of SCID?

Answer 9

infections in the first few weeks of life
diarrhoea
unusual rash

Question 10

best treatment?

Answer 10

Stem Cell Transplantation