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CRRAB II Week 4 - WLB > Nephrotic Syndrome - Pathoma > Flashcards

Nephrotic Syndrome - Pathoma Flashcards

1
Q

What is the major hallmark of Nephrotic disorders?

A
  • Proteinuria (>3.5 g/day)
    • Hypoalbuminemia → edema
    • Hypogammuglobulinemia → more infection
    • Hypercoagulable state → lose AT III
    • Hyperlipidemia & hypercholesterolemia
      • blood thin → liver releases fat to thick up blood
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2
Q

What is the most common cause of nephrotic syndrome in children?

A

Minimal Change Disease (75%)

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3
Q

What condition can be associated with Minimal Change Disease?

A

Hodgkin Lymphoma

  • MCD → loss of foot processes due to cytokine-induced damage
  • HL → increased cytokine production by Reed-Sternberg cells
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4
Q

What are less prominent associations with Minimal Change Disease?

A
  • Atopy
  • Recent infection
  • Recent immunization
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5
Q

What is the most common cause of Minimal Change Disease?

A
  • Idiopathic
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6
Q

What is the histological hallmark of Minimal Change Disease? Other histological findings?

A
  • Normal glomeruli on H&E stain
    • lipid may be seen in proximal tubule cells
  • Effacement of foot processes on EM
  • No immune complex deposits
    • negative IF
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7
Q

The “minimal” in Minimal Change Disease refers to the type of proteinuria present, which is?

A

Selective proteinuria

  • loss of albumin
  • no loss of immunoglobulin
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8
Q

What is the effective treatment for Minimal Change Disease?

A
  • Corticosteroids
    • excellent repsonse because damage is due to cytokine release from T-cells
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9
Q

What is the most common cause of nephrotic syndrome in Hispanics and African Americans?

A

Focal Segmented Glomerulosclerosis

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10
Q

What is Focal Segmented Glomerulosclerosis (FSGS) associated with?

A
  • Usually idiopathic
  • ASSOCIATED WITH:
    • HIV
    • Heroin use
    • Sickle Cell Disease
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11
Q

What are the histological findings in FSGS?

(Hint: think about the name)

A
  • Focal & segmental sclerosis on H&E
    • only segment of glomeruli involved
    • only some glomeruli effected (focal)
  • Effacement of foot processes on EM
  • No immune complex deposits
    • negative IF
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12
Q

What would happen if you treated Minimal Change Disease with Corticosteroids, and it did not improve?

A

Disease would progress to Focal Segmental Glomerulosclerosis (FSFS)

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13
Q

What is the treatment for FSGS?

A
  • No effective treatment
  • Poor response to steroids
  • Usually progresses to Chronic Renal Failure
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14
Q

What is the most common cause of nephrotic syndrome in Caucasian adults?

A

Membranous Nephropathy (30%)

(FYI: Dr. Warren → says FSGS is more common with 35% of adults)

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15
Q

What are the causes/associations of Membranous Nephropathy?

A
  • 85% Idopathic
  • May be associated with:
    • Hepatitis B
    • Hepatitis C
    • Solid tumors
    • SLE
    • Drugs: NSAIDs, penicillamine
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16
Q

What is the most common cause of death in patients with Lupus (SLE)?

A

Renal failure

  • Most common kidney disorder = Nephritic syndrome → Diffuse Proliferative Glomerulophritis
    • IF nephrotic syndrome → Membranous Nephropathy
17
Q

What is the key histological finding in Membranous Nephropathy?

(Hint: the name gives it away)

A
  • Thick glomerular basement membrane on H&E
    • due to immune complex deposition
    • granular IF
  • Supepithelial deposits with “spike and dome” appearance on EM
    • foot processes don’t like to sit on immune complexes → cover deposits with more epithelium (dome) → gap in between foot processes = spike
18
Q

What are the key histological findings in Membranoproliferative Glomerulonephritis?

A
  • Thick capillary membranes on H&E
    • with “tram-track” appearance
      • Due to immune complex deposition
        • Granular IF
19
Q

What does the “membranoproliferative” in Membranoproliferative Glomerulonephritis refer to?

A
  • Cytoplasm of Mesangial cell that holds together capillary loops proliferates
    • cytoplasm proliferation separates deposit → creates “tram-track” appearance
20
Q

What are the two types of Membranoproliferative Glomerulonephritis and how are the divided?

A
  • Type I:
    • subendothelial immune complex deposition
    • associated with HBV and HCV
    • more often associated with formation of “tram-tracks”
  • Type II:
    • immune complex deposition within basement membrane
    • associated with autoantibody C3-nephritic factor
      • stabilizes C3 convertase → (can’t be broken down) → overactivation of complement → inflammation
21
Q

What disease has the potential to cause nephritic syndrome, nephrotic syndrome, or both?

A

Membranoproliferative Glomerulonephritis

  • Type II → overactivation of compliment → inflammation → nephritic syndrome
22
Q

What is the difference between Membranous Glomerulonephropathy and Membranoproliferative Glomerulonephritis?

A
  • MGN
    • immune complex deposition in subepithelial
      • under podocytes
  • MPGN
    • Type I: immune complex deposition in subendothelial
      • outside of capillary/under GBM
    • Type II: immune complex deposition in basement membrane
23
Q

How does diabetes mellitus cause Nephrotic syndrome?

A
  • High serum glucose → leads to Non-Enzymatic Glycosylation of vascular basement membrane
    • results in: hyaline arteriolosclerosis
      • makes vessel “leaky” → protein escapes
    • preferentially effects efferent arteriole
      • leads to high glom filtration pressure → results in hyperfiltration → microalbuminuria
      • progresses to nephrotic syndrome
24
Q

How can you improve the high glomerular filtration pressure when hyaline arteriolosclerosis is present in the efferent arteriole?

A
  • Block Ang II with → ACE inhibitors
    • Ang II constricts efferent arteriole
25
Q

What is the histological hallmark of Diabetes mellitus-induced nephrotic syndrome?

A
  • Sclerosis of mesangium
  • Formation of Kimmelstiel-Wilson nodules
    • pathomnemonic for diabetes!
26
Q

What is the most commonly involved organ in Systemic Amyloidosis?

A

Kidney

(obviously)

27
Q

Where does amyloid deposit in the kidney?

A
  • Amyloid deposits → in the mesangium
    • resulting in nephrotic syndrome
28
Q

What would biopsy of the kidney in Systemic Amyloidosis show?

A
  • Congo red staining → apple-green birefringence under polarized light
29
Q

What are the 6 nephrotic syndromes? In pairs?

A
  • 1. Minimal Change Disease & 2. FSGS
  • 3. Membranous Glomerulonephropathy & 4. Membranoproliferative Glomerulonephritis
  • 5. Diabetes mellitus & 6. Amyloidosis

CRRAB II Week 4 - WLB (9 decks)

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