PHYS 799 Lecture Notes - Lecture 44: Chronic Granulomatous Disease, Membranoproliferative Glomerulonephritis, Nadph Oxidase
7 Jun 2019
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Immunodeficiency recurrent infections, immune dysregulation autoimmunity. Primary (congenital) = genetic, secondary (acquired) = not genetic. Early classical (c1-c4) invasive infections w/encapsulated bacteria (s. pneumo, h. flu, Late classical (c5-c9) invasive infections exclusively w/neisseria (meningitidis, gonorrehoeae) Test classical pathway w/ch50 test mix serum and ab-coated rbcs, observe serum levels needed to cause hemolysis. C1 inhibitor (important for blocking bradykinin production) hereditary angioedema (rapid swelling, triggered by stress) C3 nephritic factor ab against c3bbb (alternative c3 convertase), prolongs c3 convertase half-life membranoproliferative glomerulonephritis type ii (mpgn ii) Factor h/i/mcp atypical hemolytic uremic syndrome all result in increase in c3b levels, damage to endothelial cells, inflammation, clotting. Acquired neutropenia most common from chemo, drugs, autoimmunity. General symptoms infections, fever, mouth sores, peri-rectal pain/abscesses, skin infections, gum inflammation. Leukocyte adhesion deficiencies: lad i w/integrins, lad ii w/selectins. High circulating neutrophil levels, recurrent skin and soft tissue infections (boils and abscesses w/no pus)
