Primary Immunodeficiencies Flashcards Preview

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Flashcards in Primary Immunodeficiencies Deck (18)
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1
Q

Most common primary immunodef

A

IgA def

2
Q

X linked recessive, males, low B cells, infections after 6 months when no more mom Ab, poor response to vaccines

A

Bruton aggamglobulinemia/X-linked

3
Q

Hypoplasia of third and fourth pharyngeal pouches

A

DiGeorge

4
Q

Autosomal recessive or X-linked, caused by adenosine deamine def

A

SCID

5
Q

X link rec, male, triad: eczema, thrombocytopenia, recurrent infections(usu resp)

A

Wiskot Aldrich

6
Q

X linked rec (male), defect in NADPH oxidase therefore recurrent infections with catalase + bugs like STaph aureus and Pseudomonas

A

Chronic granulomatous

7
Q

Autosomal recessive, giant granules in neutrophils with oculocutaneous albinism, due to defect in microtubule polymerization

A

Chediak Higashi

8
Q

Cellular immunodef for Candida, assoc with hypothyroidism

A

Chronic mucocutaneous candidiasis

9
Q

Recurrent staph infections, fair skin, red hair, eczema

A

Hyper IgE/Job-Buckley

10
Q

Poor vaccine response, susceptible to P jiroveci, sinopulm, GI infection

A

Hyper IgM with elevated IgM and low IgG and IgA

11
Q

Delayed separation umbilical cord, necrotic skin lesions, early loss of teeth

A

Leukocyte adhesion defect

12
Q

Presents at 6 months, prone to bacterial infections

A

Abnormal B lymphocyte maturation

13
Q

What is low in IgA def

What should you not give

A

IgA and IgG

Do not give immunoglobulins: cause development of anti-IgA Ab - if anaphylaxis after give Ab think IgA def

14
Q

Common infections: Strep and Haemophilus

A

X linked aggamaglobulinemia/Bruton’s

15
Q

Hypocalemia and tetany in first 24-48 hrs of life, absent or hypoplastic thymus, congenital heart defect, fungal and viral infections common

A

DiGeorge

16
Q

B and T cell defects, severe infections, cutaneous anergy, absent or dysplastic thymus and lymph nodes

A

SCID

17
Q

Deficient nitroblue tetrazolium (NBT) dye reduction by granulocytes
Positive superoxide production testing

A

Chronic granulomatous disease

-No respiratory burst

18
Q

C5 through C9 def causing Neisseria infections

A

Complement def