Pathoma 12.3 Nephrotic Syndrome

Question 1

What are the 6 nephrotic syndromes

Answer 1

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

Question 2

What is the hallmark of nephrotic syndromes?

Answer 2

PROTEINURIA >3.5 g /day

Question 3

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

Answer 3

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

Question 4

What causes the Hypercoagulable state seen in nephrotic syndromes?

Answer 4

Loss of ATIII in the urine

Question 5

What is the most common nephrotic syndrome in kids

Answer 5

MCD

Question 6

what is MCD usually associated with

Answer 6

Hodgkin lymphoma

Question 7

What is the hallmark of MCD

Answer 7

normal glomeruli on H&E stain

Question 8

What causes pathology of MCD

Answer 8

effacement of podocyte foot processes

Question 9

What is the mechanism of pathology in MCD

Answer 9

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

Question 10

What is the IF result in MCD

Answer 10

negative becasue MCD is not IC (immune complex) mediated

Question 11

What is treatment for MCD and why is this unique

Answer 11

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

Question 12

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

Answer 12

Hispanics and AAs

Question 13

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

Answer 13

HIV (also SSD, heroin use)

Question 14

What does FSGN look like on LM?

Answer 14

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

Question 15

What does FSGN have in common with MCD (2)

Answer 15

both show effacement of podocyte foot processes

and both have no

Question 16

What does IF show in FSGN

Answer 16

nothing- no IC deposits

Question 17

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

Answer 17

IC deposition

Question 18

What aer the 3 “membranous” nephropathies?

Answer 18

Membranous Nephropathy
membranoproliferative glomerulonephritis type I
membranoproliferative glomerulonephritis type II

Question 19

Which nephrotic syndrome shows “tram track” appearance

Answer 19

membranoproliferative glomerulonephritis (type I usually)

Question 20

Which nephrotic syndrome is MC in caucasian adults?

Answer 20

Membranous Nephropathy

Question 21

Where is the IC deposition in each of :

1. Membranous Nephropathy
2. membranoproliferative glomerulonephritis type I
3. membranoproliferative glomerulonephritis type I

Answer 21

1. subepithelial (sub-podocyte)
2. subendothelial
3. intramembranous

Question 22

If you have nephropathy in SLE what is the type?

Answer 22

Membranous Nephropathy!!!!

Question 23

“Membranous” nephropathies show what on H&E

Answer 23

thick GBM

Question 24

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

Answer 24

due to IC deposition beneath podocytes

Question 25

What does IF show in Membranous Nephropathy and membranoproliferative glomerulonephritis

Answer 25

granular IC deposition

Question 26

Which nephropathy is most strongly assc with HCV

Answer 26

membranoproliferative glomerulonephritis

Question 27

Which type of cell proliferates in membranoproliferative glomerulonephritis

Answer 27

mesangial cells

Question 28

Which nephropathy is caused by overactivation of complement?

Answer 28

membranoproliferative glomerulonephritis type II

Question 29

What is, and what does C3 nephritic factor do?

Answer 29

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

Question 30

What are the two systemic diseases of nephropathy?

Answer 30

Diabetes Mellitus | Systemic Amyloidosis

Question 31

What is the first pathologic insult in DM?

Answer 31

nonenzymatic glycosylation of vascular BM

Question 32

What does nonenzymatic glycosylation of vascular BM result in

Answer 32

hyaline arteriolosclerosis

Question 33

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

Answer 33

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

Question 34

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

Answer 34

hyperfiltration injury--> microalbuminemia

Question 35

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

Answer 35

sclerosis of the mesangium in DM induced nephrotic syndrome

Question 36

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

Answer 36

ACEIs- they prevent ATII constriction of efferent arteriole relieving hyperfiltration

Question 37

Where does amyloid deposit in the kidney?

Answer 37

the mesangium

Question 38

What stain diagnoses Systemic amyloidosis and what dopes it show

Answer 38

Congo Red stain shows apple green birefringence