Pathology of Nephrotic Syndrome

Question 1

What is the most common nephrotic disorder in adults?

Answer 1

Focal segmental glomerulosclerosis

Question 2

Segmental sclerosis and hyaline deposition are characteristic of what nephrotic syndrome?

Answer 2

Focal segmental glomerulosclerosis

Question 3

What is the most common nephrotic disorder in children?

Answer 3

Minimal change disease

Question 4

True/False. Membranoproliferative glomerulonephritis can present as either nephritic or nephrotic disorders?

Answer 4

True - it can also present as both

Question 5

What complement protein is excessively activated in Type II MPGN?

Answer 5

C3 (alternative pathway)

Question 6

Some cases of membranous nephropathy is caused by autoantibodies against what antigen?

Answer 6

M-type Phospholipase A2 Receptor (PLA2R)

Question 7

Hyperlipidemia is associated with nephrotic disorders. Why is this?

Answer 7

Protein is being lost in the urine. The body increases synthesis of lipoproteins, which includes LDL, leading to hyperlipidemia

Question 8

Describe the pathogenesis of focal segmental glomerulosclerosis.

Answer 8

Degeneration and disruption of epithelial cells with effacement of foot processes. Eventually leads to detachment of foot processes (similar process to minimal change disease)

Question 9

Diffuse thickening of the glomerular capillary wall due to immune deposits is characteristic of this nephrotic disorder.

Answer 9

Membranous nephropathy

Question 10

Mesangial sclerosis in diabetic neuropathy is positive for what stain?

Answer 10

PAS+

Question 12

What are Kimmelstiel-Wilson lesions?

Answer 12

Nodules of mesangial sclerosis found in diabetic nephropathy

Question 13

What risks are most associated with Type I MPGN?

Answer 13

SLE, CLL, hepatitis B, hepatitis C

Question 14

What stain is utilized to identify renal amyloidosis?

Answer 14

Congo Red Stain

Question 15

There are two types of membranoproliferative glomerulonephritis. How do they differ?

Answer 15

Type I - Deposition of IgG and complement immune complexes

Type II - Activation of complement

Question 16

True/False. Minimal change disease responds well to treatment with corticosteroids.

Answer 16

True

Question 17

What is the leading cause of end-stage renal disease in the US?

Answer 17

Diabetic nephropathy

Question 18

This nephrotic disorder has a “pin and dome” appearance on EM.

Answer 18

Membranous nephropathy

Question 19

Both types of membranoproliferative glomerulonephritis present with a “tram track” appearance of the basement membrane on light microscopy. How do they differ on EM?

Answer 19

Type I - subendothelial deposits

Type II - intramembranous deposits

Question 20

What histologic findings are present on analysis of a nephron in a patient with minimal change disease?

Answer 20

Diffuse effacement of epithelial foot processes, no deposits, no immune complexes

Question 21

True/False. Focal segmental glomerulosclerosis responds well to corticosteroid treatment.

Answer 21

False - this is one of the distinguishing factors between FSGS and minimal change disease

Question 22

Diabetic neprhopathy begins with thickening of the basement membrane. What pathologic changes occur as the disease progresses?

Answer 22

Expansion of the mesangial matrix forming nodular sclerosis and hyaline arteriolosclerosis

Question 23

What symptoms are most associated with nephrotic disorders?

Answer 23

Proteinuria, hypoalbuminemia, hyperlipidemia, edema

Question 24

This variant of focal segmental glomerulosclerosis is due to podocyte expression of HIV genes.

Answer 24

Collapsing variant of FSGS