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Pediatrics > Immunodeficiencies > Flashcards

Immunodeficiencies Flashcards

1
Q

Which cells are part of the adaptive immune system?

A

T cells, B cells, and antibodies

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2
Q

Which cells are part of the innate immune system?

A

NK cells, Macrophages, and Complement

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3
Q

Age of onset 6-9 months? Think ___ disorder

A

T cell

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4
Q

6-12 month age of onset? Think combined

A

B cell and T cell disorder

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5
Q

Age over 12 months at onset of immunodeficiency?

A

B cell disorder

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6
Q

A good general first test in suspected immunodeficiency is

A

quantitative immunoglobulin test and CBC with diff

Don’t forget that maternal antibodies can mask antibody defects for 6-9 months!

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7
Q

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

A

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

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8
Q

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

A

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

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9
Q

Confirm Bruton’s XLA with ___ and treat with ___

A

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

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10
Q

Low IgA, IgG, but normal to high IgM =

A

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

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11
Q

Anaphylactic rxn after blood transfusion =

A

Selective IgA deficiency

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12
Q

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

A

deficiencies in at least 2/3 immunoglobulins

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13
Q

DiGeorge syndrome =

A

22q11.2 deletion

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14
Q

Where do the thymus and facial structures come from embryologically?

A

3rd and 4th pharyngeal pouch.

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15
Q

DiGeorge syndrome features =

A

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

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16
Q

DiGeorge kids should be treated with

A

PCP prophylaxis (TMP/SMX) and scheduled IVIG

17
Q

Cure the immune defect in DiGeorge by doing

A

thymic transplant!

18
Q

Why do DiGeorge kids have hypocalcemia?

A

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

19
Q

Immunostudies in Wiskott-Aldrich show

A

Increased IgE and IgA!

20
Q

How do Wiskott Aldrich kids do?

A

Not great, rarely survive into adulthood.

21
Q

Treat Wiskott-Aldrich with

A

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

22
Q

Telangiectasias + ataxia due to poor DNA repair =

A

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

23
Q

SCID kids functionally have

A

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

24
Q

Chronic granulomatous disease is a defect in

A

phagocytosis

25
Q

What organisms are kids with CGD more susceptible to?

A

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

26
Q

Confirm CGD with what test?

A

Negative nitro blue test revealing an absent respiratory burst.

27
Q

Treat CGD with

A

bone marrow transplant

28
Q

Leukocyte Adhesion Deficiency is a problem with

A

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

29
Q

Early sign of leukocyte adhesion defect is

A

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

30
Q

How is Chediak-Higashi passed down?

A

Autosomal recessive

31
Q

Features of Chediak Higashi?

A

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

32
Q

What will you see on peripheral smear in Chediak Higashi?

A

Giant granules in neutrophils.

Flow cytometry testing of neutrophil oxidative burst is gold standard of diagnosis

33
Q

How to treat Chediak Higashi?

A

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

34
Q

Hyper-IgE syndrome features peripheral ___

A

eosinophilia

35
Q

Features of Hyper IgE include

A

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

36
Q

If Neisseria is in the vignette, it’s

A

C5-C9 Terminal Complement Deficiency

37
Q

Treat hereditary angioedema with

A

FFP, as it is not IgE mediated

Pediatrics (8 decks)

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