R10 Glomerular Diseases II

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Author:
jknell
ID:
205764
Filename:
R10 Glomerular Diseases II
Updated:
2013-03-09 12:41:59
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Renal II
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Renal II
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  1. Secondary GNs
    • Goodpasture's Disease
    • -crescentic GN (anti-GBM+)

    • Systemic Vasculitis
    • -MP, WG, CS syndrome
    • -Proliferative GN (ANCA+)
    • -┬▒crescents, ┬▒vasculitis

    • SLE
    • -ANA+

    • Henoch-Schonlein disease
    • -proliferative GN (IgA)
  2. Systemic Vasculitis GN
    • -destruction of elastin
    • -heavy lymphocytic infiltrate
  3. SLE GN
    • WHO class
    • 1. Minimal (no tx)
    • 2. Mesangial (no tx)
    • 3. Focal Proliferative (steroids or none)
    • 4. Diffuse Proliferative (steroids/ mycophenolate/ cyclophosphamide/ plasmapheresis)
    • -crescentic
    • -membranoproliferative
    • 5. Membranous (steroids or none)

  4. Henoch-Schonlein GN
    • Pathophysiology:
    • 1. Mucosal antigen presentation
    • 2. Abnormal IgA response
    • 3. Circulating Ag:Ab complexes (IgA)
    • 4. Mesangial IgA deposition/involvement of skin, joints, GI tract etc

  5. HIV vs Heroin Nephropathy


    • HIV Nephropathy
    • -HIV infects renal cells (?)
    • -infection leads to disregulated cytokine production (?)
    • -common pathophysiology

    • Heroin Nephropathy
    • -mesangial deposition of foreign material from blood stream (?)
    • -Mesangial reaction (?)
    • -common pathophysiology

    • Common Pathophysiology:
    • -abnormal accumulation of hyaline material focally and segmentally within glomeruli
    • -histology similar to idiopathic focal glomerulosclerosis
    • -nephrotic syndrome and progressive renal failure
  6. Familial GN
    • 1. Alport Syndrome
    • 2. Thin Basement Membrane Disease (benign familial hematuria syndrome)
    • 3. Genetic Podocythopathies
    • -Congenic nephrotic syndrome
    • -steroid-resistant nephrotic syndrome
    • -FSG1
    • -FSG2
  7. Alport Syndrome
    -genetic defects of alpha chains of type IV collagen (2 alpha chains on X chromosome, 4 on autosomes)

    -mixed picture of autosomal dominant with greater penetrance in males

    -defective structure of GBM

    -sclerosing glomerular lesions with foam cells, interstitial lesions

    • Sx:
    • -hematuria
    • -proteinuria
    • -progressive renal failure

    • Associated congenital sx:
    • -nerve deafness
    • -ocular abnormalities
  8. Thin Basement Membrane Disease
    "Benign Familial Hematuria Syndrome"

    • -autosomal dominant inheritance
    • -seen in siblings

    • Presentation:
    • -congenitally thin GBM
    • -minor histological abnormalities
    • -recurrent episodes of hematuria

    *doesn't usually cause a decline in renal function

  9. Genetic Podocytophaties
    • -cause FSGS-like lesions

    • Congenic nephrotic syndrome:
    • -nephrin

    • Steroid-Resistant:
    • -Podocin

    • FSG 1:
    • -╬▒ actinin 4

    • FSG 2:
    • -TRP C6
  10. Metabolic Disorders that cause Glomerular Diseases
    -Diabetic Nephropathy
  11. Diabetic Glomerular Disease
    • Epidemiology:
    • -fastest growing cause of ESRD
    • -> 1/3 of ESRD cases
    • -10% DM1, 90% DM2
    • -40-50% DM1, 10-20% DM2 develop diabetic nephropathy

    • Diabetic Nephropathy
    • -progressive azotemia
    • -proteinuria
    • -edema
    • -HTN

    • Pathology:
    • -diffuse (mesangial thickening)

    • -Nodular (Kimmelstein-Wilson lesions)

    • Natural History:
    • -starts out early with an increased GFR (hyperfiltration) and episodic albuminuria
    • -later on GFR decreases and patient has hyperalbuminuria

    • Pathophysiology:
    • -advanced Glycation End-Products (AGE)
    • -non-enzymatically glycated products --> ROS etc --> TGFb, VEGF etc.
  12. Remnant Kidney Hypothesis
  13. Renal Amyloidosis
    • Pathophysiology:
    • -amyloid fibrils are indigestible protein in a crystalline "beta-pleated sheet" configuration
    • -arises from various serum proteins (Ig's, beta-2 microglobulin, amyloid A protein etc)
    • -amyloid accumulates in glomerular mesangial regions

    • Histology:
    • -congo red stain
    • -apple green birefringence under polarized light
    • -fibrillar periodicity on EM
    • Causes nephrotic syndrome and chronic renal failure

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