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36 Cards in this Set

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