Renal: 12.3: Nephrotic Syndromes Flashcards

1
Q

Why does Hodgkin lymphoma cause minimal change disease?

A
  • Hodgkin lymphoma = huge amounts of cytokines
  • m.c.d. = effacement (flattening) of the foot processes (podocytes) due to cytokines
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2
Q

What is C3 nephritic factor?

A

an autoantibody that stabilizes C3 convertase –> overactivation of complement –> inflammation, damage to the glomerulus

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3
Q

What kind of nephrotic syndrome do pts with hepatitis B or C get?

A

membranous nephropathy

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4
Q

What usually causes membranous nephropathy?

A

idiopathic

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5
Q

What is the most commonly involved organ in systemic amyloidosis?

A

the kidney

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6
Q

What is minimal change disease?

A

effacement (flattening) of the foot processes (podocytes) due to cytokines

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7
Q

What is the key finding on immunofluorescence in membranous nephropathy?

A

granular immune complex deposition

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8
Q

What can focal segmental glomerulonephritis (FSGS) progress to?

A

chronic renal failure

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9
Q

What is penicillamine? What is it used to treat?

A
  • a chelating agent
  • tx for heavy metal intox or deposition
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10
Q

What kind of nephrotic syndrome do pts with solid tumors get?

A

membranous nephropathy

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11
Q

What is focal segmental glomerulonephritis (FSGS) sometimes associated with?

A
  • HIV
  • heroin
  • sickle cell disease
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12
Q

Why do pts with nephrotic syndrome have a hypercoaguable state?

A

preferential loss of antithrombin III (AT3) –> can’t break up thrombin

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13
Q

What is Type I membranoproliferative glomerulonephritis (MPGN)?

A

Type I = subendothelial deposits, assoc. with HBV and HCV, tram-tracking more often seen

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14
Q

Why do pts with nephrotic syndrome have hyperlipidemia and hypercholesterolemia?

A

the liver tries to compensate for the thinned blood

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15
Q

Dx?

  • thickened glomerular basement membranes
  • granular immune complex depositions
  • subendothelial deposits with “spike and dome” appearance
A

membranous nephropathy

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16
Q

What can membranous nephropathy be associated with?

A
  • hepatitis B or C
  • solid tumors
  • SLE
  • drugs (NSAIDs, penicillamine)
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17
Q

What findings are seen on EM in focal segmental glomerulonephritis (FSGS)?

A

effacement of the foot processes

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18
Q

What does membranoproliferative glomerulonephritis (MPGN) look like on H&E?

A

thick capillary membranes with “tram-track” appearance

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19
Q

Dx?

  • effacement (flattening) of the foot processes (podocytes) due to cytokines
  • normal glomeruli
  • lipid in proximal tubules
A

minimal change disease

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20
Q

What is hyaline arteriolosclerosis assoc. with?

A
  • diabetes
  • HTN
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21
Q

What kind of nephrotic syndrome to HIV pts get?

A

focal segmental glomerulonephritis (FSGS)

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22
Q

What is the usual cause of focal segmental glomerulonephritis (FSGS)?

A

idiopathic

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23
Q

What is the key finding on EM in membranous nephropathy?

A

subendothelial deposits with “spike and dome” appearance

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24
Q

What can minimal change disease progress to if the steroids don’t work?

A

focal segmental glomerulonephritis (FSGS)

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25
Dx? * amyloid deposits in the mesangium
systemic amyloidosis
26
Dx? * pink sclerosis within some portions of the glomerulus * effacement of the foot processes
focal segmental glomerulonephritis (FSGS)
27
What is the key finding on H&E in membranous nephropathy?
thickened glomerular basement membranes
28
What causes thickening of the membrane in "membranous" diseases?
immune complex depositions
29
What does "proliferative" indicate in nephrotic syndromes?
* the cytoplasm in the mesangial cell proliferates to separate the immune deposits * creates a tram-track appearance
30
This is effacement (flattening) of the foot processes (podocytes) due to cytokines.
minimal change disease
31
Which part of the kidney is more affected by hyaline arteriolosclerosis? What does this cause?
* the efferent arteriole * high glomerular filtration pressure
32
What imaging findings are classic for systemic amyloidosis?
apple green birefringence under polarized light
33
What are the 2 types of membranoproliferative glomerulonephritis (MPGN)? What makes them different?
1. Type I = subendothelial deposits, assoc. with HBV and HCV 2. Type II = intramembranous (basement membrane) deposits, assoc. with C3 nephritic factor
34
What usually causes minimal change disease? What can cause it on rare occasions?
* usu. idiopathic * rare = Hodgkin lymphoma
35
What are the hallmarks of nephrotic syndrome?
* proteinuria \>3.5g/day * hypoalbuminemia --\> edema * hypogammaglobulinemia --\> increased infections * hypercoaguable state * hyperlipidemia * hypercholesterolemia
36
What will the immunofluorescence show in minimal change disease?
no findings (no immune complex deposits)
37
What causes the thick capillary membranes with "tram-track" appearance in membranoproliferative glomerulonephritis (MPGN)?
immune complex deposition
38
What is the most common nephrotic syndrome among Blacks and Hispanics?
focal segmental glomerulonephritis (FSGS)
39
This drug is a chelating agent used to treat heavy metal intox or deposition.
penicillamine
40
High serum glucose leads to non-enzymatic glycosylation of vascular basement membranes, including in the kidney, resulting in \_\_\_\_\_\_\_.
hyaline arteriolosclerosis
41
What happens microscopically in minimal change disease?
* effacement (flattening) of the foot processes (podocytes) due to cytokines * normal glomeruli * lipid in proximal tubules
42
What is a Kimmelsteil-Wilson nodule assoc. with?
hyaline arteriolosclerosis in diabetes
43
Which nephrotic syndrome can be treated with steroids?
minimal change disease
44
Why do systemic amyloidosis pts get nephrotic syndromes?
amyloid deposits in the mesangium
45
What is the tx for minimal change disease? Why?
* steroids * damage is mediated by cytokines from T cells; knock out T cells = cure
46
What kind of nephrotic syndrome do SLE pts get?
membranous nephropathy
47
What kind of nephrotic syndrome do NSAIDs, penicillamine cause?
membranous nephropathy
48
What is the most common nephrotic syndrome among white patients?
membranous nephropathy
49
What proteins are lost in minimal change disease?
albumin only = selective proteinuria
50
What is the most common cause of nephrotic syndrome in children?
minimal change disease
51
What is the most common renal disorder in SLE pts?
diffuse proliferative glomerulonephritis
52
What kind of nephrotic syndrome to heroin abusing pts get?
focal segmental glomerulonephritis (FSGS)
53
Dx? * kidney appears apple green birefringence under polarized light
systemic amyloidosis
54
What kind of nephrotic syndrome to sickle cell pts get?
focal segmental glomerulonephritis (FSGS)
55
What findings are seen on immunofluorescence in focal segmental glomerulonephritis (FSGS)?
none- no immune complex deposits in FSGS
56
What is the most common cause of death in SLE pts?
renal failure
57
What findings are seen on H&E in focal segmental glomerulonephritis (FSGS)?
pink sclerosis within the glomerulus (only part of it, though- hence focal and segmental)
58
Dx? * thick capillary membranes with "tram-track" appearance
membranoproliferative glomerulonephritis (MPGN)
59
Which type of membranoproliferative glomerulonephritis (MPGN) is subendothelial, assoc. with HBV and HCV?
Type I
60
What is Type II membranoproliferative glomerulonephritis (MPGN)?
Type II = intramembranous (basement membrane) deposits, assoc. with C3 nephritic factor
61
Which type of membranoproliferative glomerulonephritis (MPGN) is intramembranous, assoc. with C3 nephritic factor?
Type II