Renal Pathology III Flashcards Preview

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Flashcards in Renal Pathology III Deck (36):
1

What is the clinical presentation of Minimal Change Disease?

Generalized edema

2

What is the typical patient of Minimal Change Disease?

Children 2-6 years old

3

What is the pathogenesis of Minimal Change Disease?

Reversible podocyte injury that can be due to a number of factors:
- Cytokines
- Depression of immunity
- NSAIDs

4

What are the lab findings of Minimal Change Disease?

Effacement (fusion) of the epithelial foot processes

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

5

How does MCD typically resolve?

Resolves at pubert

6

How is MCD treated?

Treatment with steroids

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

• (C) Lipid droplets

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

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

9

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

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

10

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

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

11

What is the pathogenesis of Focal and Segmental Glomerular Sclerosis?

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

12

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

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

13

What is the most common pathology of human biopsy?

Focal and Segmental Glomerular Sclerosis

14

What does FSGS preferentially affect?

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

15

What is HIV-FSGS?

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

16

What is the clinical presentation of Membranoproliferative glomerulonephritis?

Nephrotic syndrome and hematuria

17

What is the typical patient of Membranoproliferative glomerulonephritis?

Older children and adults

18

What is the pathogenesis of Membranoproliferative glomerulonephritis?

Primary immune complex formation with classical
complement activation

19

What are the clinical findings of Membranoproliferative glomerulonephritis?

- Low levels of complement
- “Double contour” or “tram track” on silver stain

20

What is the typical clinical presentation of Dense Deposit Disease?

Nephrotic syndrome with hematuria

21

What is the pathogenesis of Dense Deposit Disease?

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

22

What is the typical patient of Dense Deposit Disease?

Older children - rare though

23

What are the lab findings of Dense Deposit Disease?

EM - dense deposits in the lamina densa
Complement levels will be low

24

What is Dense Deposit Disease also known as?

Membranoproliferative GN Type II

25

What diseases show mostly nephrotic features?

-MCD
-Membranous Nephropathy

26

What diseases show mostly nephritic features?

-Postinfectious Glomerulonephritis
-RPGN

27

What diseases show mixed nephritic/nephrotic features?

-FSGS
-MPGN (including DDD)

28

Amyloidosis

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.

29

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

Fat tissue

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

• (E) focal and segmental glomerulosclerosis

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

• (C) Lipid droplets

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

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

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

D. He will be treated without a biopsy

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

• (D) Fusion of podocyte foot processes

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

• (D) Recovery without serious consequences

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

B. He will be scheduled for a biopsy

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