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J's Deck > Immunodeficiencies > Flashcards

Immunodeficiencies Flashcards

(48 cards)

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1
Q

low B cell and low Ig (all types)

A

Bruton

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2
Q

defective LYST gene (lysosomal transport)

A

Chediak-Higashi

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3
Q

high AFP

A

ataxia telangiectasia

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4
Q

infections with DiGeorge

A

viral
fungal
protozoa

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5
Q

defective DNA nonhomologous end joining repair

A

ataxia telangiectasia

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6
Q

mutation in STAT3 signalling protein

A

Hyper-IgE (Job)

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7
Q

ATAXIA

A 
Ataxia
Telangiectasia/Tracking eye difficulties
Acute leukemia + lymphoma
X-ray sensitivity
IgA def
AFP
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8
Q

high: IgE, eosinophils

A

Hyper-IgE (Job)

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9
Q

IL-12 receptor deficiency

A

no T cell proliferation

  • mycobacterial
  • fungal infections
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10
Q

no NADPH oxidase

A

chronic granulomatous disease

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11
Q

no class switching

A

hyper-IgM

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12
Q

chronic mucocutaneous candidiasis

A

T cell dysfunction against Candida

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13
Q

partial albinism + recurrent respiratory/lung infections (Staph, Strep) + neuro

A

Chediak-Higashi

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14
Q

ADA deficiency

A

SCID

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15
Q

defect in early stem cell differentiation

A

SCID

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16
Q

X-linked (4)

A

Bruton
hyper IgM
Wiskott-Aldrich
chronic granulomatous disease

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17
Q

deficient CD40L-Cd40 interaction

A

hyper-IgM

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18
Q

low: IgA, T cells

sinus + lung infections

A

ataxia telangiectasia

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19
Q

WAITER

A

Wiskott
Aldrich
Immunodeficiency

Thrombocytopenia + purpura
Eczema
Recurrent pyogenic infections

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20
Q

increased risk of lymphoma + acute leukemias

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A

ataxia telangiectasia

21
Q

anaphylaxis to blood transfusions + products

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A

IgA deficiency

22
Q

CXR newborn; NO thymus

23
Q

Eczema

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A

Wiskott-Aldrich

24
Q

radiation sensitivity

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A

ataxia telangiectasia

25
retain baby teeth --> 2 rows of teeth
Hyper-IgE (Job)
26
Bruton infections
after 3-6 months (once immunity from mom goes away) - polio - coxsackie - Garidia *avoid live vaccines
27
defective phagocyte lysosomes
Chediak-Higashi
28
atophy, asthma
IgA deficiency
29
high: IgM
hyper-IgM syndrome
30
abnormal integrins (phagocytes can't exit circulation)
leukocyte adhesion deficiency syndrome
31
delayed separation of umbilical cord
leukocyte adhesion deficiency syndrome
32
susceptible to S. aureeus + Aspergillus + Nocardia
chronic granulomatous disease
33
defense relies on NK cells
SCID | no B or T cells
34
eczema + recurrent Staph abscesses/boils + corase facial features
Hyper-IgE (Job)
35
mutated WASP gene (cytoskeletal actin polymerization)
Wiskott-Aldrich
36
Thrombocytopenia + purpura
Wiskott-Aldrich
37
impaired differentation of Th-17 cells (recruit PMN)
Hyper-IgE (Job)
38
Hyper-IgE (Job)
- eczema - recurrent cold Staph abscesses (boils) - corase facial features
39
defective Tyr kinase gene
Bruton agammaglobulinemia
40
giant cytoplasmic granules in PMN
Chediak-Higashi
41
1) recurrent infections ( mucocutaneous candidiasis, RSV/VZV/HSV/measles/flu/parainfluenza, PCP) 2) chronic diarrhea 3) failure to thrive
SCID
42
healthy | viral sinus + lung infections
IgA deficiency
43
Chediak-Higashi
partial albinism recurrent respiratory/lung infections neuro
44
low: IgM, IgG --> B, T cells high: IgA, IgE
Wiskott-Aldrich
45
TX for chronic granulomatous disease
TMP-SMA itraconazole IFN-g
46
lack CD 18
leukocyte adhesion deficiency
47
persistent leukocytosis (WBC)
leukocyte adhesion deficiency
48
abnormal gamma chain shared by several interleukin receptors
SCID

J's Deck (18 decks)

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