Nephrotic Syndrome Pathology Flashcards

1
Q

If your only pathological finding is foot process fusion, what is the dx?

A

minimal change disease

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2
Q

The primary protein in the slit diaphragm has recently been identified and is termed _____.

A

nephrin

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2
Q

Why are pts with nephrotic syndrome at an increased risk of infection?

A

loss of IgG and complement (esp. factor B) into the urine

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2
Q

What is the prognosis for minimal change disease?

A

good

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3
Q

What is the major abnormality that defines nephritic syndrome?

A

active inflammation within the glomerulus that leads to damage and subsequent loss of filtration and a reduced GFR

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3
Q

Why do children with nephrotic syndrome have poor growth and osteomalacia?

A

loss of vitamin D and its binding protein in the urine

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4
Q

What causes FSGS?

A

a circulating factor that affects the podocyte (maybe suPAR)

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5
Q

Name 3 properties of mesangial cells.

A
  1. secretes a basement membrane-like matrix which acts as a structural support to the glomerulus
  2. has smooth muscle-like properties and can contract, thus affecting capillary surface area and filtration
  3. has some macrophage-like properties including the ability to secrete cytokines, growth factors, proteases and oxidants
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5
Q

What does a “Maltese cross” under polarized light indicate?

A

lipiduria, indicative of nephrotic syndrome

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6
Q

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

A

diabetic nephropathy

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7
Q

______ is a type of nephrotic syndrome and the most common one in young adults and African Americans.

A

FSGS

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8
Q

What are the following associations common to?

  • HBV
  • gold and penicillamine drugs
  • lupus (type V)
  • cancer
A

Membranous Nephropathy

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9
Q

What do these path findings indicate?

  • thickening of the GBM by light microscopy with dense, subepi deposits
  • Spikes and domes along the BM
  • IF positive for immunoglobulin and C3 and C5b-9
A

Membranous nephropathy

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9
Q

What is Type I MPGN?

A

IgG and C3 immune complex deposition in the capillary walls and mesangium

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10
Q

What is the tx for FSGS?

A

long term steroids and cyclosporine

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10
Q

How does Membranous nephropathy occur?

A

autoimmune mediated by an antibody directed against an antigen (PLA2) on the podocyte

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11
Q

A mutation in the ______ gene is responsible for the congenital nephrotic syndrome of the Finish type.

A

nephrin

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12
Q

Which nephrotic syndrome often presents like a nephritic syndrome?

A

MPGN

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13
Q

_______ is an immune mediated glomerular disease associated with immune complex deposits in the subepithelial space

A

Membranous nephropathy

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15
Q

The most important barrier to protein is the ______.

A

filtration slit diaphragm

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17
Q

Why are pts with nephrotic syndrome at an increased risk of thrombosis?

A
  • increase in synthesis of coagulation factors (fibrinogen, factors V, VIII, IX, X) by the liver
  • urinary loss of anti-thrombin III
  • increased platelet aggregability
  • impairment of the fibrinolytic system
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18
Q

These underlying conditions are all common to?

  • prior minimal change disease
  • heroin
  • HIV infection
  • sickle cell disease
  • parvo virus infection
  • obesity
A

FSGS

19
Q

How does minimal change disease pt present?

A
  • edema
  • hx of allergic disease
  • severe hypoalbuminemia
  • possible Hodgkin’s disease
20
Q

What are some known associations to MPGN development?

A

HCV

21
Q

What is the prognosis for FSGS?

A

poor

22
Q

How is vitamin D measured in the blood?

A

by measuring ionized calcium and 25-0H vitamin D levels

23
Q

IgG and C3 immune complex deposition in the capillary walls and mesangium would indicate?

A

type I MPGN

24
Q

A mutation in the nephrin gene is responsible for ________.

A

congenital nephrotic syndrome of the Finish type

25
Q

What is the prognosis for MPGN?

A

poor

27
Q

What is the tx for minimal change disease?

A

steroids (ie prednisone- should have rapid response. if not = prednisone and cyclophosphamide)

28
Q

C3 deposition only with the BM replaced by dense deposits would indicate?

A

Type II MPGN

29
Q

What do these path. findings indicate?

  • Light microscopy shows segmental scarring (sclerosis) in some (focal) glomeruli
  • Immunofluourescence shows some nonspecific staining of IgM and C3
  • EM shows diffuse foot process fusion consistent with a generalized capillary wall defect
A

FSGS

30
Q

What is the tx for Membranous Nephropathy?

A
  • Steroids
  • cytotoxic drugs (chlorambucil, cyclophosphamide)
  • ACE-I
31
Q

Nephritic syndrome presents with?

A
  • microhematuria and occasionally red cell casts
  • non-nephrotic proteinuria
  • decreased GFR
  • HTN
  • edema
32
Q

Name 4 systemic diseases associated with nephrotic syndrome.

A
  1. Diabetes
  2. Amyloid
  3. Light Chain Deposition Disease
  4. Lupus (SLE) Membranous Type V
33
Q

Name 3 components of the glomerular basement membrane.

A
  1. type IV collagen
  2. laminin and entactin (glycoproteins important in endothelial and epithelial cell attachment)
  3. heparan sulfate (proteoglycan important in providing a negative charge to the GBM)
35
Q

______ is the most common cause of idiopathic nephrotic syndrome in children.

A

Minimal change disease

37
Q

The Nephrotic syndrome results as a consequence of marked albuminuria (>3.5g/d), which leads to ____ and ____.

A

hypoalbuminemia and edema

37
Q

What can minimal change disease progress into?

A

FSGS

38
Q

What is thought to be the cause of minimal change disease?

A

a circulating T cell product that damages the podocyte and permeability layer

39
Q

Nephrotic range proteinuria almost always means that there is a disruption of the ______.

A

slit diaphragm

40
Q

_______ is the most common cause of idiopathic nephrotic syndrome in older adults, esp males.

A

Membranous nephropathy

40
Q

What is Type II MPGN?

A

C3 deposition only; BM replaced by dense deposits

42
Q

Name 4 associations with Membranous Nephropathy.

A
  1. HBV
  2. gold and penicillamine drugs
  3. lupus (type V)
  4. cancer
43
Q

Why does hyperlipidemia occur in nephrotic syndrome?

A
  • increased lipoprotein synthesis (VLDL, LDL) in the liver
  • decreased peripheral removal of VLDL
44
Q

What is the major abnormality that defines nephrotic syndrome?

A

an excessive leak of protein through the glomerular capillary wall into the urinary space

46
Q

These characteristics define what disorder? 

  • Proteinuria (>3.5 gm/day or >40 mg/hr/M2 in children)
  • Hypoalbuminemia (
  • Edema
  • Hyperlipidemia
  • Lipiduria
A

nephrotic syndrome

47
Q

What are some s/s of acute renal vein thrombosis?

A
  • flank pain
  • an enlarged kidney by ultrasound
  • hematuria
48
Q

Minimal change disease is the most common cause of idiopathic nephrotic syndrome in _____.

A

children

49
Q

Nephritic or nephrotic: Minimal change disease

A

nephrotic

50
Q

What is the prognosis for membranous nephropathy?

A

poor

51
Q

What are some underlying associations to FSGS development?

A
  • prior minimal change disease
  • heroin
  • HIV infection
  • sickle cell disease
  • parvo virus infection obesity
53
Q

What is the tx for MPGN?

A
  • alternate day steroids
  • anti-platelets
  • treatment of HCV if present
54
Q

Membranous nephropathy is the most common cause of idiopathic nephrotic syndrome in ______.

A

older adults, esp males