Pathoma 12.3 Nephrotic Syndrome Flashcards

1
Q

What are the 6 nephrotic syndromes

A

Minimal change disease (MCD)
Focal Segmental Glomerulonephrosis (FSGN)
Membranous nephropathy
membranoproliferative glomerulonephritis (MPGN)
Diabetes Mellitus
Systemic Amyloidosis

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2
Q

What is the hallmark of nephrotic syndromes?

A

PROTEINURIA >3.5 g /day

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3
Q

increased loss of protein in the urine leads to what 4 sequelae?

A
  1. Hypoalbuminemia = edema dt low oncotic pressure
  2. Hypogammaglobulinemia = increased risk of infx
  3. Hypercoagulable state - see next card
  4. Hyperlipidemia/hypercholesterolemia= fatty casts in urine
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4
Q

What causes the Hypercoagulable state seen in nephrotic syndromes?

A

Loss of ATIII in the urine

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5
Q

What is the most common nephrotic syndrome in kids

A

MCD

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6
Q

what is MCD usually associated with

A

Hodgkin lymphoma

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7
Q

What is the hallmark of MCD

A

normal glomeruli on H&E stain

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8
Q

What causes pathology of MCD

A

effacement of podocyte foot processes

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9
Q

What is the mechanism of pathology in MCD

A

hodgkin lymphoma or other process causes over-production of cytokines–> effacement of foot processes–> loss of albumin

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10
Q

What is the IF result in MCD

A

negative becasue MCD is not IC (immune complex) mediated

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11
Q

What is treatment for MCD and why is this unique

A

it is the only nephrotic syndrome that responds to steroids (bc the damage is mediated bycytokines from T cells)

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12
Q

What two populations is focal segmental glomerulonephritis (FSGN) the MC nephrotic syndrome in?

A

Hispanics and AAs

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13
Q

What disease is FSGN highly associated with (and 2 other lower associations)

A

HIV (also SSD, heroin use)

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14
Q

What does FSGN look like on LM?

A

dense pink collagen deposition in one “segment” of select glomeruli (focal = not all the glomi are affected)

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15
Q

What does FSGN have in common with MCD (2)

A

both show effacement of podocyte foot processes

and both have no

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16
Q

What does IF show in FSGN

A

nothing- no IC deposits

17
Q

If a nephrotic syndrome has “membranous” in it’s name it means it has what pathology?

A

IC deposition

18
Q

What aer the 3 “membranous” nephropathies?

A

Membranous Nephropathy
membranoproliferative glomerulonephritis type I
membranoproliferative glomerulonephritis type II

19
Q

Which nephrotic syndrome shows “tram track” appearance

A

membranoproliferative glomerulonephritis (type I usually)

20
Q

Which nephrotic syndrome is MC in caucasian adults?

A

Membranous Nephropathy

21
Q

Where is the IC deposition in each of :

  1. Membranous Nephropathy
  2. membranoproliferative glomerulonephritis type I
  3. membranoproliferative glomerulonephritis type I
A
  1. subepithelial (sub-podocyte)
  2. subendothelial
  3. intramembranous
22
Q

If you have nephropathy in SLE what is the type?

A

Membranous Nephropathy!!!!

23
Q

“Membranous” nephropathies show what on H&E

A

thick GBM

24
Q

“spike and dome” on EM is due to what and found in what disease?

A

due to IC deposition beneath podocytes

25
Q

What does IF show in Membranous Nephropathy and membranoproliferative glomerulonephritis

A

granular IC deposition

26
Q

Which nephropathy is most strongly assc with HCV

A

membranoproliferative glomerulonephritis

27
Q

Which type of cell proliferates in membranoproliferative glomerulonephritis

A

mesangial cells

28
Q

Which nephropathy is caused by overactivation of complement?

A

membranoproliferative glomerulonephritis type II

29
Q

What is, and what does C3 nephritic factor do?

A

it is an auto-antibody that stabilizes C3 convertase instead of allowing it to be broken down immediately. This leads to it’s overactivation

30
Q

What are the two systemic diseases of nephropathy?

A

Diabetes Mellitus

Systemic Amyloidosis

31
Q

What is the first pathologic insult in DM?

A

nonenzymatic glycosylation of vascular BM

32
Q

What does nonenzymatic glycosylation of vascular BM result in

A

hyaline arteriolosclerosis

33
Q

In hyaline arteriolosclerosis: which arteriole of kidney is more affected, and what is the result

A

efferent is more affected –> high GFR dt increased filtration pressure

34
Q

increased filtration pressure dt hyaline arteriolosclerosis: leads to what 2 things

A

hyperfiltration injury–> microalbuminemia

35
Q

Kimmelstein Wilson Nodules are sclerosis of what? seen in what dz

A

sclerosis of the mesangium in DM induced nephrotic syndrome

36
Q

What drug class is used to treat DM induced nephrotic syndrome and MOA

A

ACEIs- they prevent ATII constriction of efferent arteriole relieving hyperfiltration

37
Q

Where does amyloid deposit in the kidney?

A

the mesangium

38
Q

What stain diagnoses Systemic amyloidosis and what dopes it show

A

Congo Red stain shows apple green birefringence