Nephrotic Syndrome

Question 1

What is nephrotic syndrome?

Answer 1

Glomerular disorders characterized by proteinuria characterized by:

Hypoalbuminemia - pitting edema

Hypogammaglobulinemia - increased ris kof infection

Hypercoagulable state - due to loss of antithrombin III

Hyperlipidemia and hypercholesterolemia - may result in faty casts in urine

Question 2

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

Answer 2

Minimal change disease

Question 3

What is the etiology of minimal change disease?

Answer 3

Idiopathic usually

May be associatedwith Hodgkin Lymphoma

Question 4

What is seen on H&E stain, EM, and IF of MCD?

Answer 4

H&E: Normal glomeruli; Lipid may be seen in proximal tubule cells

EM: effacement of foot processes

IF: no immune complex deposits, negaitive IF

Question 5

What kind of protein is lost in the urine in MCD?

Answer 5

Selective proteinuria for albumin. No loss of immunoglobulin

Question 6

Tx for MCD

Answer 6

Excellent response to steroids (damage is mediated by cytokines from T cells)

Question 7

What is focal segmental glomerulosclerosis (FSGS)?

Answer 7

MCC of nephrotic syndrome in hispanics, and African Americans

Question 8

Etiology of FSGS

Answer 8

Usually idiopathic

May be associated with HIV, heroin use, and sickle cell disease

Question 9

What is seen on the H&E stain, EM and IF of FSGS?

Answer 9

H&E stain - Focal (some glomeruli) and segmental (involving only part of the glomerulus) sclerosis

EM - effacement of foot processes

IF - negative IF (no immune complex deposits)

Question 10

Tx for FSGS?

Answer 10

Poor response to steroids - usually progresses to chronic renal failure

Question 11

MCC of nephrotic syndrome in Caucasian adults?

Answer 11

membranous nephropathy

Question 12

Cause of membranous nephropathy?

Answer 12

Usually idiopathic

May be associated with Hep B/C, solid tumors, SLE or drugs

Question 13

Does SLE normally develop nephrotic or nephritic syndrome?

Answer 13

SLe usually develops nephritic syndrome but when it does develop nephrotic, it will be membranous

Question 14

What is seen on H&E stain, EM and If of membranous nephropathy?

Answer 14

H&E - thick glomerular BM on H&E

IF - immune complex deposition

EM - subepithelial deposits with “spike and dome” appearance

Question 15

Does membranous nephropathy respond to steroids?

Answer 15

Poor response to steroids

Progresses to chronic renal failure

Question 16

What proliferates in membranoproliferative glomerulonephritis?

Answer 16

Mesangial cells proliferate

Question 17

What is seen on H&E stain and IF of membranoproliferative glomerulonephritis?

Answer 17

H&E - thick glomerular BM often with tram-track apperance (more often seen with Type I)

IF - granular due to immune complex deposits

Question 18

What are the two types of membranoproliferative glomerulonephritis?

Answer 18

Type I - subendothelial deposits; associated with HBV and HCV

Type II (dense deposit disease) - intramembranous; associated iwth C3 nephritic factor

Question 19

Does MPGN lead to nephritic or nephrotic syndrome?

Answer 19

Either or both

Question 20

Does MPGN respond to steroids?

Answer 20

Poor response; progresses to chronic renal failure

Question 21

How does DM lead to nephrotic syndrome?

Answer 21

High serum glucose causes nonenzymatic glycosylation of the vascular BM resulting in hyaline arteriolosclerosis

This affects the efferent arteriole more than the afferent arteriole leading to a high GFR

Eventually progresses to nephrotic syndrome due to sclerosis of mesangial cells

Question 22

How is DM nephrotic ysndrome characterized?

Answer 22

Characterized by sclerosis of the mesangium with formation of Kimmelstiel-wilson nodules

Question 23

Tx for DM nephrotic syndrome

Answer 23

ACE inhibitors

Question 24

How does systemic amyloidosis cause nephrotic syndrome?

Answer 24

Kidney is the most commonly involved organ in systemic amyloidosis

Amyloid deposits in the mesangium, resulting in nephrotic syndrome

Question 25

How is systemic amyloidosis nephrotic syndrome characterized?

Answer 25

Apple-green birefringence under polarized light after staining with congo red

Question 26

what conditions cuse both nephritic and nephrotic syndrome?

Answer 26

Mebranoproliferative glomerulonephritis

Diffuse proliferative glomerulonephritis