Renal Disorders III

Question 1

Causes of nephrotic syndrome

Answer 1

• primary: minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy
• secondary: diabetic nephropathy

Question 2

Minimal change disease: who does it affect

Answer 2

• most common in children (peak 2-6yrs)

Question 3

Minimal change disease appearance on light and immunofluorescence microscopy

Answer 3

NORMAL

Question 4

Treatment for minimal change disease

Answer 4

• response well to steroids

Question 5

Key feature of minimal change disease: electron microscopy

Answer 5

• diffuse epithelial foot process effacement

Question 6

Focal segmental glomerulosclerosis (FSGS): who does it affect

Answer 6

• common in african americans

Question 7

FSGS signs

Answer 7

• hypertension

- microscopic hematuria

Question 8

FSGS biopsy signs

Answer 8

LM- segmental sclerosis
IF- mild IgM and C3 or negative
EM- diffuse epithelial cell injury

Question 9

FSGS treatment

Answer 9

• response to steroids is variable (UNLIKE minimal change disease)

Question 10

FSGS key features

Answer 10

• segmental glomerular sclerosis (LM)

- epithelial foot process effacement (EM)

Question 11

Membranous nephropathy biopsy

Answer 11

LM: thickened capillary loops
IF: granular capillary loop pattern
EM: sub epithelial deposits (SPIKES)

Question 12

Special stain done with membranous nephropathy and purpose

Answer 12

• silver stain: can see SPIKES better

Question 13

Spikes in membranous nephropathy: pink vs. black

Answer 13

• pink = deposits

- black = basement membrane

Question 14

Membranous nephropathy key feature

Answer 14

• sub epithelial immune deposits causing “spikes” by silver stain

Question 15

Membranous nephropathy causes and clinical course

Answer 15

• idiopathic: 85%
• secondary: 15%
• clinical course: chronic, waxing and waning of proteinuria

Question 16

Conditions associated with membranous nephropathy

Answer 16

1. idiopathic
2. autoimmune diseases: lupus erythematosus
3. infectious diseases: hepatitis B
4. drugs: penicillamine
5. miscellaneous: malignancies

Question 17

Diabetic nephropathy presentation

Answer 17

• proteinuria, nephrotic syndrome or chronic kidney disease

Question 18

Histologic finding with diabetic nephropathy

Answer 18

• nodular glomerulosclerosis

Question 19

Thrombotic microangiopathy: leads to what

Answer 19

1. microangiopathic hemolytic anemia: schistocytes
2. thrombocytopenia
3. kidney injury

Question 20

Features of thrombotic microangiopathy

Answer 20

• fibrinoid necrosis of arteriole
• fragmented red cells in arteriolar wall
• onion skinning of arteriole

Question 21

Causes of thrombotic microangiopathy

Answer 21

1. hemolytic uremic syndrome/thrombotic thrombocytopenic pupura
2. malignant hypertension
3. anti phospholipid syndrome

Question 22

Adult polycystic kidney disease (APKD): cause

Answer 22

• autosomal dominant inheritance

- defect in PKD1 gene (Cr 16) which encodes the polycystin-1 protein

Question 23

APKD: result

Answer 23

• multiple expanding cysts in the kidney that eventually destroys kidney
• asymptomatic until 4th decade

Question 24

Symptoms of APKD

Answer 24

• flank pain and hematuria

- saccular aneurysms in circle of willis can lead to subarachnoid hemorrhage and death

Question 25

Nephrotic syndrome signs

Answer 25

• edema
• proteinuria, >3.5 g/24hours
• hypoalbuminemia, plasma levels