Immunodeficiencies Flashcards Preview

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Flashcards in Immunodeficiencies Deck (37):
1

Which cells are part of the adaptive immune system?

T cells, B cells, and antibodies

2

Which cells are part of the innate immune system?

NK cells, Macrophages, and Complement

3

Age of onset 6-9 months? Think ___ disorder

T cell

4

6-12 month age of onset? Think combined

B cell and T cell disorder

5

Age over 12 months at onset of immunodeficiency?

B cell disorder

6

A good general first test in suspected immunodeficiency is

quantitative immunoglobulin test and CBC with diff
Don't forget that maternal antibodies can mask antibody defects for 6-9 months!

7

Generally, for humoral immunity defects, think ___ and treat with __

mucosal infections (sinopulmonary, GI) and treat w/ scheduled IVIG

8

Recurrent "normal" infections (sinusitis, otitis, pneumonia) that are frequent =

X-linked (Bruton's) Agammaglobulinemia (boys only!)

9

Confirm Bruton's XLA with ___ and treat with ___

flow cytometry (no B cells), treat w/ scheduled IVIG

10

Low IgA, IgG, but normal to high IgM =

Hyper-IgM syndrome; again, treat w/ scheduled IVIG

11

Anaphylactic rxn after blood transfusion =

Selective IgA deficiency

12

CVID can present in kids and adults and requires __ for diagnosis

deficiencies in at least 2/3 immunoglobulins

13

DiGeorge syndrome =

22q11.2 deletion

14

Where do the thymus and facial structures come from embryologically?

3rd and 4th pharyngeal pouch.

15

DiGeorge syndrome features =

Micrognathia, wide-spaced eyes, low set ears, absent thymic shadow, hypocalcemia; fungal or PCP infections are a huge red flag cuz this kiddo got no T-cells

16

DiGeorge kids should be treated with

PCP prophylaxis (TMP/SMX) and scheduled IVIG

17

Cure the immune defect in DiGeorge by doing

thymic transplant!

18

Why do DiGeorge kids have hypocalcemia?

Absent parathyroid glands (also can get seizures because of this)

19

Immunostudies in Wiskott-Aldrich show

Increased IgE and IgA!

20

How do Wiskott Aldrich kids do?

Not great, rarely survive into adulthood.

21

Treat Wiskott-Aldrich with

IVIG, splenectomy or bone marrow transplant, and manage eczema and bleeding

22

Telangiectasias + ataxia due to poor DNA repair =

Ataxia-Telangiectasia. Dr. Dustin says it's hella rare.

23

SCID kids functionally have

AIDS, so they need PCP prophylaxis. Also IVIG and bone marrow transplant.

24

Chronic granulomatous disease is a defect in

phagocytosis

25

What organisms are kids with CGD more susceptible to?

Catalase positive ones like staph, staph, and staph. Also aspergillis and serratia!

26

Confirm CGD with what test?

Negative nitro blue test revealing an absent respiratory burst.

27

Treat CGD with

bone marrow transplant

28

Leukocyte Adhesion Deficiency is a problem with

Neutrophil migration, THUS, there's no PUS despite a massive fever and leukocytosis

29

Early sign of leukocyte adhesion defect is

delayed separation of the umbilical cord! Treat with BMT like everything else

30

How is Chediak-Higashi passed down?

Autosomal recessive

31

Features of Chediak Higashi?

albinism, neuropathy, and neutropenia (Danny Chedia was not albino)

32

What will you see on peripheral smear in Chediak Higashi?

Giant granules in neutrophils.
Flow cytometry testing of neutrophil oxidative burst is gold standard of diagnosis

33

How to treat Chediak Higashi?

Treat infections (usually staph of skin) aggressively as they come

34

Hyper-IgE syndrome features peripheral ___

eosinophilia

35

Features of Hyper IgE include

recurrent "cold" abscesses, eczema, retained primary teeth, fractures, and post-infectious pneumatoceles

36

If Neisseria is in the vignette, it's

C5-C9 Terminal Complement Deficiency

37

Treat hereditary angioedema with

FFP, as it is not IgE mediated