Infections in IC host

Question 1

who gets more infections kids or adults

Answer 1

kids

Question 2

Immunocompromised pts are characterized by having

Answer 2

more infections, increased severity of those infections, diminished response to usually effective therapy, & patterns of infections are different

Question 3

what do you call an infection by an organism that normally does not cause dz but does so when IC

Answer 3

opportunistic infections

Question 4

what are 3 agents of opportunistic infections

Answer 4

Pneumocystis jirovecii, Cryptococcus neoformans, Fungal infections (other than thrush and tinea)

Question 5

what is the basis of primary immunodeficiency

Answer 5

genetic defects

Question 6

what are 3 ways to get a secondary immunodeficiency

Answer 6

1. acquired (HIV/AIDS)
2. anatomic and physiologic abnormalities (congenital asplenia/splenectomy, pumonary cyst, splenectomy)
3. Iatrogenic (urinary catheter, chemo)

Question 7

infectious symptoms begin in first days-weeks of life

Answer 7

primary immunodeficiency

Question 8

infection is often suppressed rather than eradicated by appropriate therapy

Answer 8

primary immunodeficiency

Question 9

growth and development are commonly delayed

Answer 9

primary immunodeficiency

Question 10

on a cellular level what is going on in primary immunodeficiency

Answer 10

• innate immunity: prob with phagocytes and compliment
• b lymphocytes: dysfxn in Ig prod & fxn
• t lymphocytes: can impact b cell fxn

Question 11

absence of neutrophils

Answer 11

kostmann’s congenital neutropenia (primary immunodeficiency)

Question 12

neutrophil level fluctuates up and down

Answer 12

cyclic neutropenia

Question 13

what bug can infect w/ neutrophil abnormalities

Answer 13

Aspergillus, candida, bacteria

Question 14

C1-C4 deficiency

Answer 14

AI disorders

Question 15

C5-C9 deficiency

Answer 15

neisseria spp. infection

Question 16

properidin deficiency

Answer 16

neisseria spp. infection

Question 17

how do you treat complement deficiency

Answer 17

immunizations and antibiotics if needed

Question 18

defective BTK (bruton tyrosine kinase)

Answer 18

x-linked agammaglobulinemia

Question 19

2-20 y/o w/ recurrent infections

Answer 19

CVID

Question 20

what is the MC immunodeficiency

Answer 20

IgA deficiency

Question 21

what do you not want to do in IgA deficiency

Answer 21

IgG replacement!!!!!!!!!!

Question 22

No Tcells w/ effects on Bcell fxn

Answer 22

SCID

Question 23

how do you tx SCID

Answer 23

stem cell transplant

Question 24

congenital thymic hypoplasia, heart dz, hypoparathyroidism, neonatal hypocalcemia

Answer 24

Digeorge syndrome

Question 25

neonatal hypocalcemia

Answer 25

Digeorge syndrome

Question 26

what is a major late complication of digeorge syndrome

Answer 26

AI disorder late in life

Question 27

thrombocytopenia, severe eczema

Answer 27

wiscott-aldridge syndrome

Question 28

high risk for lymphoma

Answer 28

wiscott aldridge

Question 29

how do you treat wiscott aldridge

Answer 29

stem cell transplant

Question 30

what infections do you see with lymphocytic disorders

Answer 30

pneumocystis pneumonia; fungal infections; protozoal infections; disseminated viral infections; bacterial infection, malignancy

Question 31

oral live attenuated vaccine in kid w/ SCID

Answer 31

polio

Question 32

gets infection w/ catalase positive bacteria and fungi

Answer 32

phagocyte deficiency

Question 33

gets neisseria infections

Answer 33

complement deficiency (C5-9)

Question 34

gets infected w/ organisms w/ polysaccharide capsules

Answer 34

Ab deficiency (b cell)

Question 35

gets opportunistic infections fungi and viruses

Answer 35

cellular and combined immunodeficiencies

Question 36

kid comes in with recurrent URI

Answer 36

normal

Question 37

what are you at risk for with either splenectomy or sickle cell dz

Answer 37

sepsis w/ encapsulated bacteria (ESP. S pneumonia!!!!!!!! ; and H flu)

Question 38

Digeorge syndrome

Answer 38

Neonatal hypocalcemia !!!!!!!!

Question 39

3 complications associated w/ pregnancy

Answer 39

UTI, HepE, H1N1 flu

Question 40

what would you see clinically w/ chemo induced immunodeficiency

Answer 40

bacterial/candidal infection; aspergillosis, pneumocystic pneumonia, disseminated varicella

Question 41

what are you worried about with transplant

Answer 41

risk of neutropenia plus profound lymphocytic dysfxn

Question 42

partial albanism

Answer 42

chidiak hagashi

Question 43

pseudomonas infection

Answer 43

leukemia