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Immun. def.disorders
PATHO TEST 2
Question | Answer |
---|---|
Severe combined immunodeficency disorder (SCID) | B and T cells malfunction No live vaccines Treatrrow - bone marrow transplant - death within 2 yrs w/out treatment |
Selective IgA Deficiency (sIgAD) | Most common primary immune deficiency asymptomatic w/normal IgG and Ig Increased atopic and autoimmune diseases some meds/infections cause IgA deficiency monthly Ig injections can help |
Chronic Mucocutaneous Candidiasis | T cells can't respond to Candida infection autoimmune regulator (AIRE) deficiency 80% children <3 oral candidias and candida dermatitis |
Hypogammaglobulinemia | Al Igs decrease defect in B-cell developement (lack) Primary cause - genetic Secondary cause - medications/environment Diagnosis after age 2 due to Ig developement poor Ig response to vaccination -can't produce antibodies |
Wiskott-Aldrich syndrome | RARE!!! X-linked recessive disease boys more prone Eczema, thromboctyopenia, infectious disease risk elevates impairs development of T cells, platelets, and Igs bleeding first presentation test for antibody-mediated response to vaccines |
DiGeorge Syndrome | T cell deficiency because of lack of thymus development genetic deletion at 22q11.2 other tissues affected - heart - parathyroid - facial structure - cognitive development disorder multidisciplinary care needed |