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Pathoma - Kidney and Urinary Tract Pathology > Nephrotic Syndrome > Flashcards

Nephrotic Syndrome Flashcards

1
Q

What is the cause of most nephrotic syndromes?

A

Most are idiopathic!

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2
Q

What characterizes most glomerular disorders (nephrotic syndrome)?

A

Proteinuria >3.5 g/day

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3
Q

What four things does the proteinuria in nephrotic syndrome result in?

A
  1. Hypoalbuminemia
  2. Hypogammaglobulinemia
  3. Hypercoagulable state
  4. Hyperlipidemia and Hypercholesterolemia
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4
Q

What is the major protein in the blood?

A

Albumin

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5
Q

What does Hypoalbuminemia cause?

A

Dec. albumin –> Dec. osmotic pressure (in blood vessel) –> Inc. fluid in tissue –> pitting edema

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6
Q

What does Hypogammaglobulinemia cause?

A

Loss of gammaglobulin –> Increased risk of infection

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7
Q

What causes the Hypercoagulable state in nephrotic syndromes?

A

Loss of Antithrombin III

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8
Q

What does Hyperlipidemia and Hypercholesterolemia cause in nephrotic syndromes?

A

It may result in fatty casts in the urine

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9
Q

What creates the Hyperlipidemia and Hypercholesterolemia in nephrotic syndromes?

A

Loss of protein (hypoalbuminemia) –> Blood becomes thin –> liver throws out fat and cholesterol in blood to thicken it up

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10
Q

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

A

Minimal Change Disease (MCD)

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11
Q

What can cause/be related to Minimal Change Disease (MCD)?

A

Usually idiopathic –> may be associated with Hodgkin lymphoma

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12
Q

What makes up the three layers of the glomerular filtration barrier?

A
  1. Endothelial cells
  2. Basement Membrane
  3. Epithelial cells/Foot processes of podocytes
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13
Q

What do you see on H&E stain of MCD?

A
  • Normal glomeruli

- Lipid may be seen in the proximal tubule cells

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14
Q

What is seen on electron microscopy (EM) of MCD?

A

Effacement of foot processes

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15
Q

What is seen on Immunofluorescence of MCD?

A

No immune complex deposits –> Negative IF

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16
Q

What type of proteinuria is seen in MCD?

A

Selective proteinuria = loss of albumin but no immunoglobulin

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17
Q

How is MCD treated? What is the response like?

A

Excellent response to steroids

-Only nephrotic syndrome that responds this well

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18
Q

Why is MCD treated will with steroids? What mediates the damage caused by the disease?

A

Damage is mediated by cytokines and T cells

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19
Q

What causes MCD in Hodgkin Lymphoma?

A

Massive production of cytokines by Reed-Sternberg Cells!

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20
Q

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

A

Focal Segmental Glomerulosclerosis (FSGS)

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21
Q

What is the cause of Focal Segmental Glomerulosclerosis (FSGS)?

A

Usually idiopathic

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22
Q

What might FSGS be associated with?

A

HIV, heroin use, and sickle cell disease

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23
Q

If you have an HIV patient that develops nephrotic syndrome, what does this tell you?

A

He/she has FSGS

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24
Q

What does glomerulosclerosis look like?

A

Dense, pink collagen deposition

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25
Q

What does FSGS show on H&E stain?

A

Focal (only some glomeruli) and segmental (involving only part of the glomerulus) sclerosis.

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26
Q

If MCD doesn’t respond to steroids, what might it become?

A

FSGS

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27
Q

What is seen on EM in FSGS?

A

Effacement of foot processes

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28
Q

What is seen on IF of FSGS?

A
  • No immune complex deposits

- Negative IF

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29
Q

How do you treat FSGS? What is the response like?

A

Poor response to steroids

30
Q

What does FSGS progress to?

A

CRF (chronic renal failure)

31
Q

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

A

Membranous Nephropathy

32
Q

What causes Membranous Nephropathy?

A

Usually idiopathic

33
Q

What conditions may be associated with Membranous Nephropathy?

A

Hepatitis B or C, Solid tumors, SLE or drugs (e.g. NSAIDs and penicillamine)

34
Q

What is the most common cause of death in SLE?

A

Renal failure!

35
Q

What is seen in Membranous Nephropathy on H&E?

A

Thick glomerular basement membrane

36
Q

What causes the thick glomerular basement membrane in Membranous Nephropathy?

A

Immune complex deposition

37
Q

What is seen on IF of Membranous Nephropathy?

A

Granular IF - due to Immune Complex Deposition

38
Q

What do you see on EM of Membranous Nephropathy?

A

Subepithelial deposits with ‘spike and dome’ appearance.

39
Q

Where do immune complexes deposit in Membranous Nephropathy?

A

Subepithelial

40
Q

How is Membranous Nephropathy teated? How does it respond?

A

Poor response to steroids

41
Q

What does Membranous Nephropathy progress to?

A

CRF (chronic renal failure)

42
Q

What nephritis can cause Nephrotic, Nephritic and/or both syndromes?

A

Membranoproliferative Glomerulonephritis

43
Q

What is seen on H&E of Membranoproliferative Glomerulonephritis?

A

Thick glomerular basement membrane, often with a ‘tram-track’ appearance

44
Q

What causes the thick glomerular basement membrane in Membranoproliferative Glomerulonephritis?

A

Immune complex deposition

45
Q

What is seen in IF of Membranoproliferative Glomerulonephritis?

A

Immune complex deposition (granular IF)

46
Q

What are the two types of Membranoproliferative Glomerulonephritis?

A
  1. Type I

2. Type II

47
Q

What are the two types of Membranoproliferative Glomerulonephritis based on?

A

Location of deposits

48
Q

Where are the deposits in Type I Membranoproliferative Glomerulonephritis?

A

Subendothelial

49
Q

What is Type I Membranoproliferative Glomerulonephritis associated with?

A

HBV and HCV (these are more often associated with tram-track appearance)

50
Q

Where are the deposits in Type II Membranoproliferative Glomerulonephritis?

A

Intramembranous

51
Q

What is Type II Membranoproliferative Glomerulonephritis also known as?

A

Dense deposit disease

52
Q

What is Type II Membranoproliferative Glomerulonephritis associated with?

A

C3 Nephritic factor

53
Q

What is C3 Nephritic factor and what does it do?

A

It’s an autoantibody that stabilizes C3 convertase —> leads to overactivation of complement –> Inflammation –> Low levels of circulating C3

54
Q

What does C3 convertase do?

A

Converts C3 to C3a and C3b

55
Q

What does Membranoproliferative Glomerulonephritis have a poor response to?

A

Steroids

56
Q

What does Membranoproliferative Glomerulonephritis progress to?

A

CRF (chronic renal failure)

57
Q

What causes the tram-track appearance of the basement membrane in Membranoproliferative Glomerulonephritis?

A

Mesangial cells grow out in-between Immune complexes/basement membrane, dividing them into strips and making them look like two tracks.

58
Q

What happens to the basement membrane in Diabetes Mellitus?

A

High serum glucose –> non enzymatic glycosylation of the vascular basement membrane (sugar sticking to basement membrane) –> Hyaline Arteriolosclerosis

59
Q

What type of arteriolosclerosis is associated with Diabetes Mellitus?

A

Hyaline Arteriolosclerosis

60
Q

What glomerular arteriole is more affected in Diabetes Mellitus?

A

Efferent arteriole is more affected.

61
Q

What does arteriolosclerosis of the efferent arteriole cause?

A

High glomerular filtration pressure

62
Q

What does hyperfiltration injury in Diabetes Mellitus lead to?

A

Microalbuminuria

63
Q

What does the increased pressure in the efferent arteriole in Diabetes Mellitus eventually progress to?

A

Nephrotic syndrome

64
Q

What characterizes Nephrotic syndrome in Diabetes Mellitus?

A
  • Sclerosis of the mesangium

- Formation of Kimmelstiel-Wilson nodules

65
Q

What drugs slow the progression of hyper filtration induced damage in Diabetes Mellitus?

A

ACE Inhibitors

66
Q

How does arteriolosclerosis lead to nephrotic syndrome in Diabetes Mellitus?

A

Inc. GFP –> Sclerosis –> Nephrotic syndrome

67
Q

What does Angiotensin II exacerbate that ACE inhibitors stop?

A

Narrowing of the efferent arteriole

68
Q

What is the most commonly involved organ in Systemic Amyloidosis?

A

Kidney!!

69
Q

How does Systemic Amyloidosis cause Nephrotic syndrome?

A

Amyloid deposits in the mesangium –> results in nephrotic syndrome

70
Q

What stain is used to characterize Systemic Amyloidosis?

A

Congo red

71
Q

What appearance does Systemic Amyloidosis have with Congo red stain?

A

Apple-green birefringence under polarized light

Pathoma - Kidney and Urinary Tract Pathology (9 decks)

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