Immune Deficiency Disorders Flashcards
Most common primary immunodeficiency
IgA deficiency
Sx of IgA deficiency
recurrent respiratory and GI nfections. IgA levels always low. Levels of IgG subclass 2 may be low.
Tx of IgA deficiency
Do NOT give immunoglobulins-may cause anaphylaxis.
How do you recognize Bruton’s agammaglobulinemia?
X-linked recessive disorder. low or absent B cells. affect males Infxns start after 6 mo when maternal Abs go away. recurrent lung or sinus infections Strep & H. flu
Classic cause of Severe Combined ImmunoDeficiency (SCID)
Adenosine deaminase deficiency (Autosomal recessive) may also be X-linked.
SCID symptoms
B and T cell defects causing infxns in first few months of life. also cutaneous anergy and absent or dysplastic thymus ore lymph nodes.
Pathophysiology of Chronic Granulomatous Disease (CGD)
usually X-linked recessive disorder that affects males. b/c of defect in NADPH oxidase.
Symptomology of CGD
recurrent catalase positive infxns. Staph aureus, Pseudomonas spp.
Confirmatory test CGD
NBT (nitroblue tetrazolium) dye reduction by granulocytes. shows lack of respiratory burst.
Complement deficiencies of C5 through C9
recurrent infxns with: Neisseria spp.
Define Chronic mucocutaneous candidiasis
cellular immunodeficiency specific for Candida. thrush; candidal infxns of scalp, skin, and nails; anergy to Candida spp/ on skin testing No other immunodeficiencies.
Association with Chronic mucocutaneous candidiasis
hypothyroidism
Hyper-IgE eponym
Job Buckley syndrom
Hyper-IgE description
recurrent staph infxns (esp skin) extremely high IgE levels fair skin, fair hair, and eczema.
MOA of steroids
inhibit Interleuki-1 production
