Path Test 4: Renal

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Path Test 4: Renal
2013-02-16 17:11:20
Path Test Renal

Path Test 4 Renal
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  1. Nephrotic Syndrome Symptoms
    • Hyperproteinuria (> 3.5g/ day)
    • Hyperlipidemia
    • Hypercoaguability (due to loss of ATIII in urine)
    • Edema
    • Hypoalbuminemia
  2. Nephritic Syndrome (Acute Nephritis) Symptoms
    • Hematuria, RBC casts in urine
    • Azotemia
    • Oliguria (decreased urine output)
    • Mild-to-moderate HTN
    • Proteinuria and edema (less severe than in nephrotic syndrome)
  3. IF deposits:
    MPGN Type I?
    MPGN Type II?
    Post-infectious Glomerulonephropathy?
    Lupus Nephritis?
    IgA Nephropathy?
    • FSGS:  IgM, C3
    • MG:  IgG (granular), IgA (in 25%)
    • MCD:  Negative
    • MPGN Type I:  IgG, C3 (+/- IgM)
    • MPGN Type II:  C3, irregular/granular capillary wall and mesangium
    • PIG:  IgG, IgM, C3 (granular)
    • Lupus Nephritis:  Full House!  IgG, IgM, IgA, C3, C1q, C4
    • IgA Nephropathy:  IgA in mesangium, C3 (NOT C1q, C4)
    • Alport:  None
    • Anti-GBM:  IgG (linear)
  4. Crescents
    • MPGN (some)
    • Anti-GBM:  Goodpastures
  5. Serologic findings of p- or c-ANCA
    Pauci-immune glomerulonephritis
  6. RPGN associated w/
    • Lupus
    • Membranoproliferative glomerulonephritis
    • IgA nephritis
  7. Sclerosis of some, but not all glomeruli (focal)
    Only a portion of the capillary tuft is involved (segmental)
    Focal Segmental Glomerular Sclerosis
  8. Most common cause of nephrotic syndrome in the US
    Focal Segmental Glomerular Sclerosis
  9. Hallmark of FSGS
    Visceral epithelial damage
  10. Response to corticosteroid therapy:
    • FSGS- poor response
    • MCD- great response
    • MG- variable response
  11. FSGS:  LM
    Sclerosis and collapse of glomerular capillary segments; hypercellularity of affected glomeruli; Hyalinosis
  12. FSGS: IF
    Granular IgM and C3 deposits
  13. FSGS:  EM
    • Effacement of foot processes
    • Vacuolization of visceral epithelial cells
    • Widening of lamina rara externa
  14. Focal cystic dilation of tubule segments and tubuloreticular inclusions (in endothelial cells via EM)
    FSGS HIV-associated nephropathy
  15. Nephrotic syndrome w/ thromboembolic episodes
    Membranous Glomerulonephropathy
  16. MG:  LM
    • Thickened capillary walls
    • Spikes (silver stain)
  17. MG:  IF
    Granular IgG (IgA in 25%)
  18. MG:  EM
    Subepithelial dense deposits
  19. Common in children, often follows respiratory infxn or immunization
    Minimal Change Disease
  20. Minimal Change Disease is associated w/
    Hodgkin Lymphoma
  21. MCD:  LM
  22. MCD:  IF
  23. MCD: EM
    • Effacement of podocytes
    • Lipid droplets in the interstitial and tubular cells
  24. 2 Nephrotic Syndromes w/ effacement of foot processes
    • Focal Segmental Glomerular Sclerosis
    • Minimal Change Disease
  25. Occurs in myeloma patients
  26. Two types of Amyloidosis
    • AL (light chain)
    • AA types (SAA protein)
  27. Amyloidosis:  LM
    Thickened vessels, expanded glomerular matrix
  28. Amyloidosis:  IF
    Light chains (AL, kappa or lambda monoclonality)
  29. Amyloidosis:  EM
    Straight, non-branching fibrils
  30. Characteristic stain of Amyloidosis
    Apple green birefringence w/ Congo Red stain
  31. 3 Types of Membranoproliferative Glomerulonephritis (MPGN)
    • Type I:  Immune complex mediated; classical and alternative
    • Type II:  Circulating C3 nephritic factor; alternative
    • Type III:  Features Type I and membranous glomerulopathy
  32. Nephrotic Syndrome, often w/ a nephritic component (hematuria), that affects adolescents and young adults
  33. Immune complex mediated, activates classical and alternative complement pathways
    MPGN (Type I)
  34. MPGN Type I:  LM
    • Hypercellular glomeruli
    • Tram track on PAS/silver stain
  35. Tram Track (silver stain) on LM
    MPGN Type I
  36. MPGN Type I:  IF
    C3, IgG (+/- IgM) in capillary loops and mesangium
  37. MPGN Type I:  EM
    Subendothelial dense deposits
  38. MPGN Type 2: LM
    • Thickening of GBM
    • Dense deposits in segmental fashion "string of sausages"
  39. MPGN Type II:  EM
    Intramembranous dense deposits in lamina densa

    *FSGS has widening of lamina rara externa
  40. Circulating C3 nephritic factor
    MPGN Type II

    This causes persistent C3 activation and hypocomplementemia
  41. Diabetic Nephropathy 5 Stages
    • 1:  Increased GFR, microalbuminuria
    • 2:  GFR and albumin excretion normalizes
    • 3:  HTN develops, microalbuminemia returns
    • 4:  Persistent proteinuria and HTN
    • 5:  ESRD
  42. Microangiopathy
    Glycosylation of proteins --> thickened basement membrane
    Diabetic Nephropathy
  43. Diabetic Nephropathy:  LM
    • Thickening of basement membrane
    • Kimmelstiel-Wilson lesion
    • Capsular "drop" lesions- eosinophili mass btw Bowman's capsule and parietal epithelium
    • "Fibrin Cap Lesion"- eosinophilic lipid-rich structure found within glomerular capillary lamina
  44. Kimmelstiel-Wilson lesion
    Diabetic Nephropathy
  45. Capsular drop lesions
    Diabetic Nephropathy
  46. Fibrin cap lesion
    Diabetic Nephropathy
  47. 1-4 weeks after a strep infxn (Grp. A Beta-hemolytic) in children
    Post-infectious Glomerulonephritis
  48. Symptom of Post-infectious Glomerulonephritis
    Periorbital Edema
  49. PIG:  LM
    Glomerular Hypercellularity
  50. PIG:  IF
    Granular IgG, IgM, C3
  51. PIG:  EM
    Subepithelial "humps"- lumpy bumpy
  52. Subepithelial humps
  53. DNA and anti-DNA Abs to histones
    Lupus Nephritis
  54. Lupus Nephritis Classes
    • •Class I: Negative or minimal mesangial
    • deposits

    •Class II:  More extensive mesangial deposits than class I

    • •Class III:  Diffuse mesangial deposits and widely scattered irregular
    • deposits in capillary loops

    •Class IV:  Extensive immune complex deposits in capillaries and mesangium

    •Class V: Uniform subepithelial deposits (especially IgG) in capillaries
  55. IgA deposits in mesangium (often w/ C3)
    IgA Nephropathy (Berger's Disease)
  56. IgA Nephropathy (Berger's Disease) is seen w/ increased frequency in pts. w/
    Gluten enteropathy (Celiac Disease)
  57. IgA Nephropathy:  LM
    Mesangial proliferation
  58. IgA Nephropathy:  IF
    • IgA deposits in mesangium
    • C3 (NOT C1q, C4)
  59. IgA Nephropathy:  EM
    • Large dense depostis in the mesangium
    • Subendothelial, subepithelial and intramembranous deposits
  60. Non-thrombycytopenic palpable purpura of the lower extremities; renal disease w/ hematuria
    Henoch-Schonlein Purpura
  61. HSP occurs in
    Children 3-8 (can occur in adults) often after onset of URTI
  62. HSP is associated w/
    IgA Nephropathy (same microscopic findings)
  63. Hereditary Nephritis (Alport Syndrome):  Defect in what
    Type IV collagen (basement membrane)
  64. Mutation of Hereditary Nephritis (Alport Syndrome)
    Sex-linked mutations of alpha-5 chain
  65. Presents w/ hematuria, gradual renal failure, neurosensory hearing loss, anterior lenticonus
    Hereditary Nephritis (Alport Syndrome)
  66. Alport Syndrome:  LM
    • Gradual global sclerosis
    • Interstitial foam cells
  67. Interstitial foam cells on LM
    Hereditary Nephritis (Alport Syndrome)
  68. Alport:  IF
  69. Alport:  EM
    Laminated, frayed, irregular basement membranes
  70. Attenuation of basement membrane  150-250 nm (normal 300-400 nm)
    Thin Basement Membrane Lesion
  71. Mutations of Thin Basement Membrane Lesion
    COL4A3, COL4A4 genes that encode alpha chains of type IV collagen
  72. Antibodies to NC1 domain of a3(IV) chain of type IV collagen
  73. Anti-GBM  + Pulmonary involvement w/ hemorrhage
    Goodpasture syndrome
  74. Prominent cellular crescents and linear IgG deposits
    Anti-GBM Disease