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Microbiology > Immunodeficiency d/o's > Flashcards

Immunodeficiency d/o's Flashcards

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Biology 101 flashcards
1
Q

Mutation in what enzyme is responsible for B cell depletion in Bruton’s agammaglobulinemia

A

Tyrosine kinase

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2
Q

Tx for Bruton’s agammaglobulinemia

A

pooled gamma globulin

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3
Q

B-cells (antibody-producing) target what organisms?

A

Bacteria

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4
Q

T-cells target what organisms?

A

Viruses
Protozoa
M. tuberculosis

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5
Q

Phagocytes target what organism?

A

Fungi

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6
Q

X-linked agammaglobulinemia is assoc w/ recurrent infx of enteroviruses, w/c are the ff:

A

Polio
Coxsackie
Echovirus

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7
Q

At what age does X-linked agammaglobulinemia usually manifest?

A

6 mos. of life

when maternal IgG wanes off

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8
Q

MOA of selective IgA deficiency

A

Failure of Isotyoe switching

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9
Q

S/Sx of selective IgA immunodeficiency

A

Recurrent infx of the resp system, GUT, and GIT

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10
Q

Tx contraindicated for IgA immunodeficiency

A

Gamma globulin

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11
Q

Most severe of B-cell d/o’s

A

Common Variable Immunodeficiency

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12
Q

B-cell d/o’s

A

X-linked agammaglobulinemia
Selective IgA deficiency
Common variable immunodeficiency

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13
Q

T-cell d/o’s

A

DiGeorge Syndrome

Chronic Mucocutaneous Candidiasis

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14
Q

MOA of DiGeorge syndrome

A

Profound deficit of T cells d/t failure of thymus and parathyroid devt

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15
Q

Pharyngeal pouches affected in DiGeorge syndrome

A

3rd and 4th pharyngeal pouch

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16
Q

S/Sx of DiGeorge syndrome

A

Tetany d/t hypoCa

Severe viral, fungal or protozoal infx

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17
Q

DiGeorge syndrome’s CATCH 22

A 
Cardiac defect (TOF)
Abnormal facies
Thymic aplasia
Cleft palate
HypoCa
22q11.2 chromosomal deletion
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18
Q

Combined B and T-cell d/o’s

A

Severe Combined Immunodeficiency (SCID)
Wiskott-Aldrich Syndrome
Ataxia-Telangiectasia

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19
Q

MOA of X-linked SCID

A

defect in IL-2 receptors in T cells

20
Q

MOA of Autosomal SCID

A

ADA deficiency

21
Q

Tx for SCID

A

Plastic bubble

BM transplant

22
Q

Tx of DiGeorge Syndrome

A

Transplant of fetal thymus (

23
Q

MOA of Wiskott-Aldrich Syndrome

A

Inability to mount IgM response d/t mutation of WASP gene for actin filament assembly

24
Q

S/Sx of Wiskott-Aldrich syndrome

A 
Thrombocytopenia
Infections (pyogenic)
Eczema
WASP mutation (ety.)
(How do you TIE a WASP?)
25
Q

Autosomal recessive w/ mutationa in DNA repair enzymes leading to IgA deficiency

A

Ataxia-telangiectasia

26
Q

Tx for ataxia-telangiectasia

A

Supportive (antibx)

27
Q

Tx for Wiskott-Aldrich Syndrome

A

BM transplant

28
Q

Age when ataxia-telangiectasia manifests

A

2 y/o

29
Q

Phagocyte d/o’s

A

Chronic granulomatous dse
Chediak-Higashi Syndrome
Leukocyte Adhesion Deficiency

30
Q

Complement d/o’s

A

Early complement deficiency (C2 and C3 def)

Terminal complement deficiency

31
Q

MOA of phagocyte d/o’s

A

lack of NADPH oxidase activity (failure of oxidative burst)

32
Q

Manifests w/ chronic infx w/ catalase (+) bacteria and fungi

A

Chronic granulomatous dse

33
Q

Tx for chronic granulomatous dse

A

None (only antibx prophylaxis)

34
Q

MOA of Chediak-Higashi Syndrome

A

Failure of phagolysosomal fixation

35
Q

Autosomal recessive dse assoc w/ faulty microtubules that impair neutrophil chemotaxis

A

Chediak-Higashi syndrome

36
Q

Organisms that commonly affect pts w/ Chediak-Higashi syndrome

A

Staphylococcus

Streptococcus

37
Q

Autosomal recessive dse w/c has defective adhesion (LFA-1) proteins on the phagocyte surface

A

Leukocyte Adhesion Deficiency

38
Q

Mutation in LAD

A

Integrins

39
Q

Most common early complement deficiency

A

C2 deficiency

40
Q

MOA of terminal complement deficiency

A

inability to form MAC (deficiency in C5-9)

41
Q

Common infx among pts w/ terminal complement def

A

Neisseria

42
Q

Strains in quadrivalent meningococcal vaccine

A

A
C
Y
W135

43
Q

Secondary immunodeficiencies

A

Malnutrition

AIDS

44
Q

Deficiencies in malnutrition

A

Dec AA

Dec synthesis of IgG and complement

45
Q

Tx for AIDS

A

HAART: Highly-active antiretroviral therapy

46
Q

B cell d/o w/ very low levels of all immunoglobulins

A

X-linked agammaglobulinemia

Microbiology (9 decks)

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