05.14 - Nephrotic Syndrome 2 (Nichols, Showkat) - PP + Handout Flashcards Preview

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Flashcards in 05.14 - Nephrotic Syndrome 2 (Nichols, Showkat) - PP + Handout Deck (58):
1

suPAR

Binds Beta3-integrin --> FSGS

1

Reduce __ is associated with secondary FSGS

Reduced nephron mass

2

Chronic, slowly progressive disease wih GBM thickening, Subepithelial immune complex deposits, and effacement of foot processes

Membranous Nephropathy

2

IF of Post-Strep GN

Diffuse granular deposts of IgG and C3

2

Prognosis of Post-Strep GN

Generally good in children; 40% of adults develop chronic azotemia

2

ApoL1 mutation makes you susceptible to

FSGS

3

AI w/ immune complexes formed in situ from binding of filtered auto-ab to podocyte m-type PLA2R

Probably pathogenic mechanism causing most cases of Primary Membranous Nephropathy

4

IF in MCD and FSGS are usually both

negative

5

Role of suPAR, beta3-integrin, and podocytes in Focal Segmental Glomerulosclerosis

suPAR binds to and activates beta3-integrin, a major podocyte anchoring protein

5

EM of Focal Segmental Glomerulosclerosis

Effacement of foot processes

5

If poor response to steroids in MCD, think of

Unsampled focal segmental glomerulosclerosis

5

Why is effacement of foot process likely mediated by MAC

Intermediary chemotactic fragments, C3a and C5a, are washed away into urinary space

6

Demographic of Post-Strep GN

School age children, male preponderance

7

LM of Membranous Nephropathy

Thickening of GBM

9

Most common demographic of Focal Segmental Glomerulosclerosis

Adult black males

10

Why no inflammation in Membranous Nephropathy

Complement not activated b/c at a site that is not in contact with circulating inflammatory cells

11

Most common cause of Nephortic Syndrome in Caucasian Adults

Membranous Nephropathy

11

Thickened GBM w/out increased cellularity

Membranous Nephropathy

12

Most common cause of Nephrotic Syndrome in Black Adults

64% Focal Segmental Glomerulosclerosis

13

IF of FSGS

Normal

14

Membranous Nephropathy vs Post-Infectious GN: Which is nephritic and which nephrotic

Membranous Nephropathy is nephrotic

15

Diffuse endocapillary proliferation and infiltration by numerous neutrophils

Post-Strep GN

16

IF of Membranous Nephropathy

Granular subepithelial deposits of IgG and Complement

17

Complement changes in Post-Strep GN

Low C3, but normal C4 - Demonstrates alternative pathway activation

18

Suggested pathogenesis of Focal Segmental Glomerulosclerosis

suPAR binds to and activates beta3-integrin, a major podocyte anchoring protein

19

What causes Spike and Dome appearance

Subepithelial deposits with thin tracts of new GBM separating the deposits

20

Which subtype of Focal Segmental Glomerulosclerosis has a rapid onset of nephrotic syndrome with rapid progression to renal failure

Collapsing Glomerulopathy

20

What is hyalinosis in FSGS

Accumulation of leaked plasma protiens and lipids

22

What causes 75% of nephrotic syndrome cases in children

Minimal Change Disease

23

Soluble Urokinase-type plasminogen activator receptor (suPAR) is elevated in serum of 2/3 of patients with

Focal Segmental Glomerulosclerosis

23

Tx of FSGS

Corticosteroids (poor response) and Calcineurin inhibitors

26

LM histology of Focal Segmental Glomerulosclerosis

Scarring, Adhesions to bowman's capsule, Occluded cap lumens, Hyalinosis

27

The Collapsing Glomerulopathy subtype of FSGS is often associated with

HIV infection or drug toxicity

28

suPAR binds to and activates

Beta3-integrin, a major podocyte-anchoring protein

29

Prognosis of Minimal Change Disease

Resolves with steroid tx in >90% of kids; Response slow in adults; Recurrence is common

31

Minimal change disease causes about__ of nephrotic cases in children

75%

32

What mutation in AA's confers 10.5-fold higher incidence of Focal Segmental Glomerulosclerosis

ApoL1

34

5 features accompanying Nephrotic Syndrome that indicate FSGS over MCD

Older Age, Hematuria, HTN, Non-selective Proteinuria, Poor response to steroids

35

Probably pathogenic mechanism causing most cases of Primary Membranous Nephropathy

AI w/ immune complexes formed in situ from binding of filtered auto-ab to podocyte m-type PLA2R

37

Ages of minimal change disease

Very young or very old

38

Presentation of Membranous Nephropathy

Most with nephrotic syndrome, rest with asymptomatic proteinuria

40

Most common subtype of Focal Segmental Glomerulosclerosis

Not Otherwise Specified

41

Demographic of Membranous Nephropathy

Caucasian Male Adults

42

EM of Membranous Nephropathy

Effacement of FP's and Spike and Dome: Subepithelial deposits with tracks of BM separating them

43

Top two findings in FSGS

Nephrotic Range Proteinuria, Hematuria

44

LM of Post-Strep GN

Diffuse proliferative GN, prominent endocapillary proliferation and numerous neutrophils

44

NEP is the target antigen in

Congenital Membranous nephropathy

46

m-type PLA2

Immune complexes --> Most primary Membranous Nephropathy

48

LM, IF, and EM of Minimal Change Disease

LM and IF are negative, but EM shows foot process effacement

49

Post-Strep GN typically presents with

Nephritic Syndrome

50

Tea or Cola Colored Urine

Post-Infectious GN

52

Why is it important to differentiate FSGS from MCD

Prognosis is much worse in FSGS

53

PLA2R is the target antigen in

Primary (idiopathic) Membranous Nephopathy

54

EM of Post-Strep GN

Subepithelial electron-dense deposits = Humps

55

Insidious onset of Nephrotic Syndrome in child with good response to steroid tx

Minimal Change Disease

56

Cornerstone of tx in Minimal Change Disease

Oral Glucocorticoids

57

What causes podocyte injury in Membranous Nephropathy

Formation of MAC due to auto-Ab binding (to m-type PLA2R)

58

Time fram of Post-Strep GN after infection

1-6 weeks