Nephrotic Syndrome

Question 1

What is nephrotic syndrome?

Answer 1

Glomerular disorders characterized by proteinuria (>3.5g/day).

Question 2

What are some of the findings in Nephrotic Syndrome?

Answer 2

Hypoalbuminemia (pitting edema)
Hypogammagloninemia (increased risk of infection)
Hypercoagulable state (loss of antithrombin 3)
Hyperlipidemia
Hypercholesterolemia

Question 3

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

Answer 3

Minimal Change Disease

Question 4

What are the characteristic findings in minimal change disease?

Answer 4

Normal glomeruli on H&E stain.
Effacement of foot processes on EM.
No immune complex deposition, - IF.
Great response to steroids (unique for nephrotic syndromes).

Question 5

What is the most common cause of nephrotic syndrome in US adults?

Answer 5

Focal Segmental Glomerulosclerosis.

Question 6

What are the characteristic findings in focal segmental glomerulosclerosis?

Answer 6

LM-> sclerosis of some glomeruli
EM->Effacement of podocytes
IF-> IgM and C3

Question 7

What is the most common nephrotic syndrome in Caucasian adults?

Answer 7

Membranous nephropathy

Question 8

What are the characteristic findings in membranous nephropathy?

Answer 8

LM->Thick glomerular basement membrane on H&E
EM->subepithelial deposits, “spike and dome”
IF-> Granular IgG and C3.

Question 9

What is the name of the nephrotic syndrome that is characterized by a thickened GBM and with “tram-track” appearance?

Answer 9

Membranoproliferative glomerulonephritis

Question 10

What are the two types of membranoproliferatrive glomerulonephritis?

Answer 10

Type 1-> subendothelial associated with HBV and HCV

Type 2-> Intramembranous deposition, C3 nephritic factor

Question 11

What leads to the nonenzymatic glycosylation of the vascular basement membrane resulting in hyaline arteriolosclerosis?

Answer 11

Diabetes mellitus which leads to increase glucose concentrations.

Question 12

What specific structures are altered in DM?

Answer 12

Glomerular efferent arteriole is more effected than afferent leading to high glomerular filtration pressure. Can cause microalbuminuria.

Question 13

What are the histological hallmarks of DM?

Answer 13

Nodular glomerular sclerosis, Diffuse capillary BM thickening, Diffuse mesangial sclerosis

Question 14

What is the most commonly involved organ in systemic amyloidosis?

Answer 14

THE KIDNEY

Question 15

What is characteristic findings in systemic amyloidosis?

Answer 15

Apple-green birefringence under polarized light with Congo red

Question 16

What are the two etiologies of membranous glomerulonephrolpathy?

Answer 16

1) idiopathic-> autoimmune possibly?

2) secondary-> SLE, drugs, tumors, infections (Hep C, B)

Question 17

What is the pathogenesis of MPGN type 1 (primary form) and who is most often affected?

Answer 17

• Immune complex deposition.

- Most likely to affect young adults and occasionally children

Question 18

What is the pathogenesis of MPGN type 1 (secondary form) and who does it affect?

Answer 18

• Almost exclusively affects adults.

- Frequently associated with chronic antigenemia, HCV, SLE, metastasis of cancers.

Question 19

What two nephrotic glomerular diseases have a higher incidence in adults than kids?

Answer 19

• Membranous glomerulopathy

- Focal Segmental glomerulosclerosis

Question 20

What will chronic DM lead to in the kidney?

Answer 20

Advanced renal hyaline arteriosclerosis

Question 21

What are the characteristic findings on EM for SLE/ diffuse proliferative lupus nephritis?

Answer 21

Subendothelial Immune deposits that correspond to “wire loops” by LM.

Question 22

What are the changes that occur in the glomerulus in response to diffuse proliferative lupus nephritis?

Answer 22

-marked increase incellularity, increased glomerular size, the glomerulus appears stuffed into bowmans capsule

Question 23

What are the findings on IF in SLE and diffuse proliferative lupus nephritis?

Answer 23

Mesangial and capillary wall (subendothelial) IgG localization