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36 Cards in this Set
- Front
- Back
Pathogenesis of Nephrotic Syndrome
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increased basement membrane permeability (loss of negative charge on GBM)
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Clinical Manifestations of Nephrotic Syndrome
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Proteinuria, Hypoalbunemia, Generalized edema, Hiperlipidemia
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Disorders manifest by nephrotic syndrome
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Minimal Change disease (lipoid nephrosis), Focal segmental glomerulosclerosis, Membranous glomerulonephritis, Diabetic nephropathy, renal amyloidosis, lupus nephropathy
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Clinical Manifestations of Nephritic Syndrome
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Oliguria, Azotemia, Hypertension, Hematuria (red cell casts)
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Disorders manifest by nephritic syndrome
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Poststrep GN, Rapidly progressive (crescentic) GN, Goodpasture Disease, Alport Syndrome
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Minimal Change Disease (lipoid nephrosis)
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most common cause of NEPHROTIC syn in children (ages 2-6). normal appearing glomeruli by light microscopy. fusing of epithelial foot processes on EM.
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Treatment in Minimal Change Disease?
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Steriods (thought to be immune related). Usually responsive.
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Focal Segmental Glomerulosclerosis
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nephrotic syndrome with sclerotic capillary tufts.
Focal (involves only some golmeruli), segmental (involves on part of the glomerulus). |
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What is sclerosis?
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involves collapse of basement membranes, increased matrix and deposition of hyaline.
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Membranous Glomerulonephrits
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most common cause of nephrotic syn in adults. immune complex disease of unknown etiology. THICKENED CAPILLARY WALLS, subepithelial immune complexes-- spike and dome, and granular immunoflouresence of IgG or C3.
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Diabetic nephropathy
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nephrotic syn. increase in gbm thickness. increase in mesangial matrix. diffuse glomerulosclerosis or nodular glomerulosclerosis with Kimmelstiel Wilson nodules (accumulations of mesangial matrix material)
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What is the etiology of diabetic nephropathy?
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1) advanced glycosylation end products. 2) Increased GFR induced hypertrophy (fewer vessels = more load for each glomerulus).
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Renal amyloidosis
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nephrotic syndrome. subendothelial and mesangial amyloid deposits. stain with congo red. associated with chronic inflammatory diseases (rheumatoid arthritis or plasma cell disorders-- multiple myeloma).
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Lupus nephropathy
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nephrotic syndrome (sometimes has nephritic features-- hematuria). subendothelial immune complex deposition. Five forms.
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Poststrep GN
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nephritic syn. subepithelial electron dense humps, lumpy bumpy immunofluorescence.
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what is the cause of Acute poststreptococcal glomerulonephritis
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group A beta-hemolytic strep, immune complex disease with antigen of antigen-antibody complexes of streptococcal origin
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morphology of glomeruli in Acute Post Strep GN
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enlarged hypercellular, swollen, bloodless with proliferation of mesangial and endothelial cells. electron-dense HUMPS on epithelial side of BM (subepithelial)
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Rapidly progressive (crescentic) glomerulonephritis
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nephritic syn. crescents, ANCA-negative forms with immune complexes or anti-GBM antibodies. ANCA-postive (pauci-immune) form with wegener granulomatosis
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goodpasture syndrome
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nephritic syn.
linear immunofluorescence, split BM |
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alport syndrome
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nephritic syn.
split BM. |
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proteinuria, hypoalbuminemia, edema, hyperlipidemia
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Nephrotic Syndrome
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fusing epithelial foot processes
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Minimal Change disease-- nephrotic syndrome
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most common cause of nephrotic syn in adults
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Membranous glomerulonephritis
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most common cause of nephrotic syn in kids
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Minimal Change Disease (lipoid nephrosis)
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characterized by thickened capillary walls on LM, thickening of BM on EM, immune complexes in subepithel locations,
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Membranous Glomerulonephrosis
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spike and dome, granular IgG or C3
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Membranous Glomerulonephrosis
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Increase in messangial matrix resulting in diffuse, nodular glomerulosclerosis
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Diabetic Nephropathy-- nephrotic syn
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congo red stained subendothelial and mesangial deposits
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renal amyloidosis
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Oliguria, azotemia, hypertension, hematuria
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nephritic syndrome
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enlarged hypercellular, swollen, bloodless glomeruli
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Poststrep GN-- nephritic syn
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nephritic syndrome progressing rapidly to renal failure, crescents of fibrin in Bowman space
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Rapidly Progressive GN, post strep in 50% of cases
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antiglomerular basement membrane antibodies with linear immunofluorescence of IgG
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Goodpasture Syndrome-- w/ hemoptysis
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hereditary nephritis (with irregular glomerular BM thickening with foci of splitting of lamina densa) associated with nerve deafness and ocular disorders (lens dislocation and cataracts)
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alport syndrome-- mutation in gene for type four collagen
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Deposition of IgA in mesangium-- activate alternative complement pathway, recurrent hematuria
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IgA nephropathy (Berger Disease)
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tram track
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Membranoproliferative GN
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Membranoproliferative GN
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characterized by slow progression to chronic renal disease, alterations in BM, prolif of glomerular cells and leukocyte infiltration, type 1 is an immune complex nephritis assoc with unknown antigen and tram-track appearance, type 2 has tram track appearance as well, but is characterized by irregular electron dense material dposited w/i the glom basement membrane,
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