12.3 Nephrotic Syndrome

Question 1

What is the hallmark of nephrotic syndrome?

Answer 1

Proteinuria

Question 2

What is the main protein loss in nephrotic syndrome?

Answer 2

Albumin

Question 3

Why is there a hypercoaguable state in nephrotic syndrome?

Answer 3

Preferential loss of AT III as well as the slowed production of coagulation factors.

Question 4

Why does hyperlipidemia occur with nephrotic syndrome?

Answer 4

Increased liver production of lipids and cholesterol to counteract the thinning of blood proteins.

Question 5

Who is generally affected by Minimal Change Disease?

Answer 5

Children

Question 6

What condition is associated with MCD?

Answer 6

Hodgkin’s Lymphoma

Question 7

What happens to the foot processes in MCD?

Answer 7

The are effaced (fused together)

Question 8

What is the H&E finding in MCD?

Answer 8

Normal findings

Question 9

What proteins are lost in MCD?

Answer 9

Albumin

Question 10

What is the main treatment for MCD that has good response?

Answer 10

Steroids - they know out the effect of the cytokines

Question 11

Who is generally affected with Focal Segmental Glomerular Sclerosis (FSGS)?

Answer 11

African Americans

Hispanics

Question 12

What are some of the associations with FSGS?

Answer 12

HIV
Heroin Use
Sickle Cell Disease

Question 13

What will be seen on FSGS microscopy?

Answer 13

• Focal and Segmental Glomerular Sclerosis (only some are affected)
• Effacement of the foot processes

Question 14

Does FSGS respond to steroids?

Answer 14

NO

Question 15

Who is primarily affected by membranous nephropathy?

Answer 15

Caucasian adults

Question 16

What can membranous neuropathy be associated with?

Answer 16

SLE
Solid tumors
Hepatitis B/C
Drugs - NSAIDs and pencillamine

Question 17

What is found on microscopy of membranous nephropathy?

Answer 17

• Thick glomerular basement membrane
• “Spike and dome” appearance
• Immune complex deposition with granular IF

Question 18

What does “membranous” indicate?

Answer 18

Immune complex deposition - shows with granular IF

Question 19

What are the microscopy findings with membranoproliferazive glomerulonephritis (MPGN)?

Answer 19

• Thick capillary membranes with “tram track” appearance

- Immune complex deposition with granular IF

Question 20

What is Type I MPGN?

Answer 20

Sub endothelial immune complex deposits - associated with hepatitis B/C

• More often with the formation of “tram tracks”

Question 21

What is Type II MPGN?

Answer 21

Deposition of immune complex in the basement membrane - associated with C3 nephritic factor mutation that stabilizes C3 convertase

Question 22

What is a unique feature of MPGN?

Answer 22

It can create nephrotic, nephritic or both syndromes

Question 23

Where is the deposition in membranous nephropthy?

Answer 23

Sub epithelial deposition

Question 24

What is the pathogenesis of diabetic nephropathy?

Answer 24

Non-enzymatic glycosylation of vascular basement membrane due to high glucose levels which leads to hyaline arteriolosclerosis - preferentially in the efferent arteriole that results in increased glomerular pressure

Question 25

How does diabetes lead to nephrotic syndrome?

Answer 25

Hyperfiltration will result from NEG which leads to microalbuminuria, eventually causing nephrotic syndrome

Question 26

How does systemic amyloidosis cause nephrotic syndrome?

Answer 26

Amyloid most commonly deposits in the kidney in the mesangium which leads to nephrotic syndrome

Question 27

What are the lab findings for amyloid?

Answer 27

Apple-green birefringence under polarized light.