Glomerular Diseases
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- 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,