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Pathoma LAH > Renal: 12.3: Nephrotic Syndromes > Flashcards

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
Q

Dx?

  • amyloid deposits in the mesangium
A

systemic amyloidosis

26
Q

Dx?

  • pink sclerosis within some portions of the glomerulus
  • effacement of the foot processes
A

focal segmental glomerulonephritis (FSGS)

27
Q

What is the key finding on H&E in membranous nephropathy?

A

thickened glomerular basement membranes

28
Q

What causes thickening of the membrane in “membranous” diseases?

A

immune complex depositions

29
Q

What does “proliferative” indicate in nephrotic syndromes?

A
  • the cytoplasm in the mesangial cell proliferates to separate the immune deposits
    • creates a tram-track appearance
30
Q

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

A

minimal change disease

31
Q

Which part of the kidney is more affected by hyaline arteriolosclerosis? What does this cause?

A
  • the efferent arteriole
  • high glomerular filtration pressure
32
Q

What imaging findings are classic for systemic amyloidosis?

A

apple green birefringence under polarized light

33
Q

What are the 2 types of membranoproliferative glomerulonephritis (MPGN)? What makes them different?

A
  1. Type I = subendothelial deposits, assoc. with HBV and HCV
  2. Type II = intramembranous (basement membrane) deposits, assoc. with C3 nephritic factor
34
Q

What usually causes minimal change disease? What can cause it on rare occasions?

A
  • usu. idiopathic
  • rare = Hodgkin lymphoma
35
Q

What are the hallmarks of nephrotic syndrome?

A
  • proteinuria >3.5g/day
  • hypoalbuminemia –> edema
  • hypogammaglobulinemia –> increased infections
  • hypercoaguable state
  • hyperlipidemia
  • hypercholesterolemia
36
Q

What will the immunofluorescence show in minimal change disease?

A

no findings (no immune complex deposits)

37
Q

What causes the thick capillary membranes with “tram-track” appearance in membranoproliferative glomerulonephritis (MPGN)?

A

immune complex deposition

38
Q

What is the most common nephrotic syndrome among Blacks and Hispanics?

A

focal segmental glomerulonephritis (FSGS)

39
Q

This drug is a chelating agent used to treat heavy metal intox or deposition.

A

penicillamine

40
Q

High serum glucose leads to non-enzymatic glycosylation of vascular basement membranes, including in the kidney, resulting in _______.

A

hyaline arteriolosclerosis

41
Q

What happens microscopically in minimal change disease?

A
  • effacement (flattening) of the foot processes (podocytes) due to cytokines
  • normal glomeruli
  • lipid in proximal tubules
42
Q

What is a Kimmelsteil-Wilson nodule assoc. with?

A

hyaline arteriolosclerosis in diabetes

43
Q

Which nephrotic syndrome can be treated with steroids?

A

minimal change disease

44
Q

Why do systemic amyloidosis pts get nephrotic syndromes?

A

amyloid deposits in the mesangium

45
Q

What is the tx for minimal change disease? Why?

A
  • steroids
  • damage is mediated by cytokines from T cells; knock out T cells = cure
46
Q

What kind of nephrotic syndrome do SLE pts get?

A

membranous nephropathy

47
Q

What kind of nephrotic syndrome do NSAIDs, penicillamine cause?

A

membranous nephropathy

48
Q

What is the most common nephrotic syndrome among white patients?

A

membranous nephropathy

49
Q

What proteins are lost in minimal change disease?

A

albumin only = selective proteinuria

50
Q

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

A

minimal change disease

51
Q

What is the most common renal disorder in SLE pts?

A

diffuse proliferative glomerulonephritis

52
Q

What kind of nephrotic syndrome to heroin abusing pts get?

A

focal segmental glomerulonephritis (FSGS)

53
Q

Dx?

  • kidney appears apple green birefringence under polarized light
A

systemic amyloidosis

54
Q

What kind of nephrotic syndrome to sickle cell pts get?

A

focal segmental glomerulonephritis (FSGS)

55
Q

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

A

none- no immune complex deposits in FSGS

56
Q

What is the most common cause of death in SLE pts?

A

renal failure

57
Q

What findings are seen on H&E in focal segmental glomerulonephritis (FSGS)?

A

pink sclerosis within the glomerulus (only part of it, though- hence focal and segmental)

58
Q

Dx?

  • thick capillary membranes with “tram-track” appearance
A

membranoproliferative glomerulonephritis (MPGN)

59
Q

Which type of membranoproliferative glomerulonephritis (MPGN) is subendothelial, assoc. with HBV and HCV?

A

Type I

60
Q

What is Type II membranoproliferative glomerulonephritis (MPGN)?

A

Type II = intramembranous (basement membrane) deposits, assoc. with C3 nephritic factor

61
Q

Which type of membranoproliferative glomerulonephritis (MPGN) is intramembranous, assoc. with C3 nephritic factor?

A

Type II

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