Renal Pathology III

Question 1

What is the clinical presentation of Minimal Change Disease?

Answer 1

Generalized edema

Question 2

What is the typical patient of Minimal Change Disease?

Answer 2

Children 2-6 years old

Question 3

What is the pathogenesis of Minimal Change Disease?

Answer 3

Reversible podocyte injury that can be due to a number of factors:

• Cytokines
• Depression of immunity
• NSAIDs

Question 4

What are the lab findings of Minimal Change Disease?

Answer 4

Effacement (fusion) of the epithelial foot processes

-> No inflammation/cellular proliferation and no immune complex deposits

Question 5

How does MCD typically resolve?

Answer 5

Resolves at pubert

Question 6

How is MCD treated?

Answer 6

Treatment with steroids

Question 7

A 4-year-old girl presents with swelling of the legs and ankles. Physical examination reveals pitting edema of the lower extremities. Urinalysis show 2 + proteinuria. The urinary sediment contains no inflammatory cells or red blood cells. Serum levels of BUN and creatinine are normal. The patient recovers completely after a course of corticosteroids. Which of the following pathologic findings might be expected in the urine prior to treatment with corticosteroids?

• (A) leukocyte casts
• (B) Eosinophils
• (C) Lipid droplets
• (D) Red blood cells casts
• (E) White blood cells casts

Answer 7

• (C) Lipid droplets

Question 8

For the patient described in the previous slide, electron microscopy of a renal biopsy specimen prior to treatment would most likely demonstrate which of the following abnormalities?

• (A) layering and splitting of the lamina densa
• (B) subepithelial electron-dense deposits
• (C) Electron-dense deposits in the mesangium
• (D) effacement (fusion) of podocyte foot processes
• (E) Loss of microvilli by the tubular lining cells

Answer 8

• (D) effacement (fusion) of podocyte foot processes

Question 9

What is the clinical presentation of Focal and Segmental Glomerular Sclerosis?

Answer 9

• nephrotic syndrome
• higher incidence of hematuria, reduced GFR, HTN,
• non-selective proteinuria more often

Question 10

What is the typical patient of Focal and Segmental Glomerular Sclerosis?

Answer 10

• Mostly adults with African American andHispanic patients more frequent
• Children can also be affected though

Question 11

What is the pathogenesis of Focal and Segmental Glomerular Sclerosis?

Answer 11

Irreversible injury to podocytes due to segmental obliteration (sclerosis) of capillaries with collapse of
capillary loops and increase in mesangial matrix

Question 12

What are the lab findings of Focal and Segmental Glomerular Sclerosis?

Answer 12

• Immunofluorescence - negative

- Electron microscopy: effacement of the epithelial foot processes in ALL glomeruli

Question 13

What is the most common pathology of human biopsy?

Answer 13

Focal and Segmental Glomerular Sclerosis

Question 14

What does FSGS preferentially affect?

Answer 14

Preferentially affects the juxtamedullary glomeruli with a greater percentage becoming affected as the disease progresses

Question 15

What is HIV-FSGS?

Answer 15

Collapse of the tuft + proliferation of visceral epithelial cells and has a VERY poor prognosis

Question 16

What is the clinical presentation of Membranoproliferative glomerulonephritis?

Answer 16

Nephrotic syndrome and hematuria

Question 17

What is the typical patient of Membranoproliferative glomerulonephritis?

Answer 17

Older children and adults

Question 18

What is the pathogenesis of Membranoproliferative glomerulonephritis?

Answer 18

Primary immune complex formation with classical

complement activation

Question 19

What are the clinical findings of Membranoproliferative glomerulonephritis?

Answer 19

• Low levels of complement

- “Double contour” or “tram track” on silver stain

Question 20

What is the typical clinical presentation of Dense Deposit Disease?

Answer 20

Nephrotic syndrome with hematuria

Question 21

What is the pathogenesis of Dense Deposit Disease?

Answer 21

Sustained activation of complement via alternative pathway so it is non-antibody mediated and there are no antibody-Ag complexes

Question 22

What is the typical patient of Dense Deposit Disease?

Answer 22

Older children - rare though

Question 23

What are the lab findings of Dense Deposit Disease?

Answer 23

EM - dense deposits in the lamina densa

Complement levels will be low

Question 24

What is Dense Deposit Disease also known as?

Answer 24

Membranoproliferative GN Type II

Question 25

What diseases show mostly nephrotic features?

Answer 25

- MCD | - Membranous Nephropathy

Question 26

What diseases show mostly nephritic features?

Answer 26

- Postinfectious Glomerulonephritis | - RPGN

Question 27

What diseases show mixed nephritic/nephrotic features?

Answer 27

- FSGS | - MPGN (including DDD)

Question 28

Amyloidosis

Answer 28

Amyloidosis is a consequence of deposition of abnormally folded protein which acquires a beta-pleated sheet conformation. Although many (>25) different proteins have been shows to form deposits of amyloid, all deposits of amyloid share the same staining pattern with affinity to Congo red dye and fibrillar ultrastructural appearance.

Question 29

What kind biopsy can be used to screen for amyloidosis in kidney disease?

Answer 29

Fat tissue

Question 30

A 30-year-old man with a history of drug addiction presents with a 6-month history of progressive swelling in his ankles and abdomen. Urinalysis shows heavy proteinuria (>4g /24 hours) but no evidence of inflammatory cells or RBCs . Laboratory studies reveal hyperlipidemia and hypoalbuminemia. Serum creatinine level is normal. The blood test for ANCA is negative. The patient responds well to treatment with corticosteroids, but edema and proteinuria recur the following year. The steroid treatment is repeated with the same results. Upon the third recurrence of edema and proteinuria, the patient becomes steroid resistant. A renal biopsy is shown. Which of the following is the most likely diagnosis for this patient’s glomerulopathy? * (A) Acute glomerulonephritis * (B) Amyloidosis * (C) Crescentic glomerulonephritis * (D) Diffuse proliferative glomerulonephritis * (E) focal and segmental glomerulosclerosis

Answer 30

• (E) focal and segmental glomerulosclerosis

Question 31

A 4-year-old girl presents with swelling of the legs and ankles. Physical examination reveals pitting edema of the lower extremities. Urinalysis show 2 + proteinuria. The urinary sediment contains no inflammatory cells or red blood cells. Serum levels of BUN and creatinine are normal. The patient recovers completely after a course of corticosteroids. Which of the following pathologic findings might be expected in the urine prior to treatment with corticosteroids? * (A) Amyloid casts * (B) Eosinophils * (C) Lipid droplets * (D) Red blood cells casts * (E) White blood cells casts

Answer 31

• (C) Lipid droplets

Question 32

An 18-year-old African-American man has had increasing lethargy, proteinuria, and edema following a bout of the flu 3 weeks ago. At that time, the patient visited his primary care physician, who prescribed corticosteroids for 3 weeks without improvement of his condition. His physician then referred him to a nephrologist, who performed a renal biopsy and diagnosed him with focal segmental glomerulosclerosis. Which of the following was the most likely histologic description in this patient’s biopsy report? * (A) Entire glomerular tufts show obliterated capillaries; >50% of glomeruli affected * (B) Entire glomerular tufts show obliterated capillaries with cellular proliferation * (C) Parts of the glomerular tufts show obliterated capillaries; less than 50% of glomeruli affected * (E) Parts of the glomerular tufts show obliterated capillaries with cellular proliferation

Answer 32

• (C) Parts of the glomerular tufts show obliterated capillaries; less than 50% of glomeruli affected

Question 33

3 yo boy presented with periorbital and generalized edema UA: proteinuria, 2.5 gm/24 hr Which would be the most appropriate choice: A. Kidney biopsy is needed B. He will need hearing testing C. Treatment with steroids will not be effective D. He will be treated without a biopsy E. He does not have nephrotic syndrome yet

Answer 33

D. He will be treated without a biopsy

Question 34

3 yo boy presented with periorbital and generalized edema UA: proteinuria, 2.5 gm/24 hr For the patient described in the previous question, electron microscopy of a renal biopsy specimen prior to treatment would most likely demonstrate which of the following abnormalities? • (A) Duplication of capillary basement membranes • (B) Electron-dense immune deposits in the capillary basement membranes. • (C) Electron-dense immune deposits in the mesangium • (D) Fusion of podocyte foot processes • (E) Splitting of the lamina densa

Answer 34

• (D) Fusion of podocyte foot processes

Question 35

3 yo boy presented with periorbital and generalized edema UA: proteinuria, 2.5 gm/24 hr Which of the following is the most likely outcome of diseases in the patient described in the previous question? * (A) chronic renal failure * (B) Development of nephritic syndrome * (C) Membranoproliferative glomerulonephritis * (D) Recovery without serious consequences * (E) Transition into crescentic glomerulonephritis

Answer 35

• (D) Recovery without serious consequences

Question 36

A 10 yo boy presented with NS. He has a 5 year history of relapsing nephrotic syndrome, initially responding to steroids, subsequently became steroid-dependent and steroid-resistant. His serum creatinine is raising. Which would be the most appropriate choice: A. He will be treated without a biopsy B. He will be scheduled for a biopsy C. He will require hearing testing D. His biopsy will show membranous nephropathy E. His biopsy will show minimal change disease

Answer 36

B. He will be scheduled for a biopsy