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Flashcards in Immune Deficiencies Presentations Deck (21)
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1
Q

X-linked (Bruton) agammaglobulinemia

A

Recurrent bacterial and enteroviral infections after 5 months (decreased maternal IgG)

2
Q

Selective IgA deficiency

A

Majority Asymptomatic. Can see Airway and GI infections, Autoimmune disease, Atopy, Anaphylaxis to IgA-containing products.

3
Q

Common variable immunodeficiency

A

Can be acquired in 20s-30s; increased risk of autoimmune disease, bronchiectasis lymphoma, sinopulmonary infections

4
Q

Thymic aplasia (DiGeorge syndrome)

A

Tetany (hypocalcemia), recurrent viral/fungal infections (T-cell deficiency), conotruncal abnormalities (e.g. tetralogy of Fallot, truncus arteriosus)

5
Q

IL-12 receptor deficiency

A

Disseminated mycobacterial and fungal infections; may present after administration of BCG vaccine

6
Q

Autosomal dominant hyper-IgE syndrome (Job syndrome)

A

FATED: coarse Facies, cold (noninflamed staphylococcal Abscesses, retained primary Teeth, inc. IgE, Dermatologic problems (eczema).

7
Q

Chronic mucocutaneous candidiasis

A

Noninvasive Candida albicans infections of skin and mucous membranes.

8
Q

SCID

A

Failure to thrive, chronic diarrhea, thrush. Recurrent viral, bacterial, fungal, and protozoal infections. Treatment: bone marrow transplant (no concern for rejection)

9
Q

Ataxia-telangiectasia

A

Triad: cerebellar defects (Ataxia), spider Angiomas (telangiectasia), IgA deficiency

10
Q

Hyper-IgM syndrome

A

Severe pyogenic infections early in life; opportunistic infection with PCP, Cryptosporidium, CMV

11
Q

Wiskott-Aldrich syndrome

A

WATER: Wiskott-Aldrich: Thrombocytopenic purpura, Eczema, Recurrent infection. Increased risk of autoimmune disease and malignancy.

12
Q

Leukocyte adhesion deficiency (type 1)

A

Recurrent bacterial skin and mucosal infections, absent pus formation, impaired wound healing, delayed separation of umbilical cord (>30 days)

13
Q

Chediak-Higashi syndrome

A

recurrent pyogenic infections by staph and strep, partial albinism, peripheral neuropathy, progressive neurodegeneration, infiltrative lymphohistiocytosis

14
Q

Chronic granulomatous disease

A

Increased susceptibility to catalase + organisms (PLACESS): Pseudomonas, Listeria, Aspergillus, Candida, E. coli, S. aureus, Serratia

15
Q

Ataxia-Telangiectasia simple

A

Ataxia, Telengiectasia, Sinopulmonary infections

16
Q

Chediak-Higashi simple

A

Oculocutaneous albinism, pyogenic infections, progressive neurologic dysfunction

17
Q

Chronic granulomatous disease simple

A

Severe bacterial and fungal infections, granuloma formation

18
Q

DiGeorge syndrome simple

A

Congenital heart disease, dysmorphic facies, hypocalcemia

19
Q

SCID simple

A

Severe bacterial & viral infections in infancy, chronic diarrhea, mucocutaneous candidiasis

20
Q

C5-C9 deficiency simple

A

Recurrent Neisseria infection

21
Q

Wiskott-Aldrich syndrome simple

A

Recurrent infections that worsen with age, easy bleeding, eczema