Renal: 2. Leonard : Glomerulonephritis I & Ii

Front 1

which has more protein excreted in the urine: nephrotic or nephritic?

Back 1

nephrotic

Front 2

intrinsic (or primary) kidney disease is normally caused by what?

Back 2

Typically is immune-mediated +/- inflammatory cell involvement -->hence, glomerulonephritis

Front 3

Hematuria, azotemia, variable proteinuria,
oliguria, edema, and hypertension

what syndrome?

Back 3

Nephritic syndrome

Front 4

3.5 gm/day proteinuria,
hypoalbuminemia, hyperlipidemia,
lipiduria

what is the syndrome

Back 4

nephrotic syndrome

Front 5

Azotemia ➙ uremia progressing for
months to years

syndrome?

Back 5

chronic renal failure

Front 6

Acute nephritis, proteinuria, and acute
renal failure

syndrome?

Back 6

Rapidly progressive glomerulonephritis

Front 7

linear, granular, and clumping on immunofloruecence shows what?

Back 7

deposition of immune complexes

Front 8

What are the 5 types of nephrotic syndromes?

Back 8

Minimal change disease
Membranous glomerulopathy
Focal segmental glomerulosclerosis (FSGS)
Diabetic glomerulosclerosis
Amyloid nephropathy

Front 9

Minimal change disease
Membranous glomerulopathy
Focal segmental glomerulosclerosis (FSGS)
Diabetic glomerulosclerosis
Amyloid nephropathy

are what type of syndromes?

Back 9

nephrotic

Front 10

Most common form of nephrotic syndrome in children ?

Back 10

Minimal Change Disease (MCD)

Front 11

this nephrotic syndrome Tends to be fairly “albumin selective” proteinuria (albumin >>Ig)

Back 11

Minimal Change Disease (MCD)

Front 12

LM: normal glomerulus
IF: negative
EM: effacement of podocyte foot processes (therefore disrupted filtration slits); no immune deposits

findings of?

Back 12

Minimal Change Disease (MCD)

Front 13

Tx for minimal change disease?

Back 13

Most experience remission with corticosteroid Tx

Very good response in children
Upon withdrawal of corticosteroids, relapses for up to ~ 10 yrs

Front 14

Most common cause of nephrotic syndrome in adults (Caucasians, Asians)

Back 14

Membranous Glomerulopathy

Front 15

LM: thickened capillary walls; no hypercellularity; silver stain can show spikes along thickened loops
IF: confluent granular staining for IgG, C3 along capillary loops
EM: electron dense sub-epithelial immune deposits
4 stages of severity of deposits

what are these findings of?

Back 15

Membranous Glomerulopathy

Front 16

EM: electron dense sub-epithelial immune deposits

found in?

Back 16

Membranous Glomerulopathy

Front 17

IF: confluent granular staining for IgG, C3 along capillary loops

found in?

Back 17

Membranous Glomerulopathy

Front 18

LM: thickened capillary walls; no hypercellularity; silver stain can show spikes along thickened loops

found in?

Back 18

Membranous Glomerulopathy

Front 19

LM: normal glomerulus

found in?

Back 19

Minimal Change Disease (MCD)

Front 20

IF: negative

found in?

Back 20

minimal change disease

Front 21

EM: effacement of podocyte foot processes (therefore disrupted filtration slits); no immune deposits

found in?

Back 21

minimal change disease

Front 22

Scarring of portion of some glomeruli =

Back 22

Focal Segmental Glomerulosclerosis (FSGS)

Front 23

Heterogeneous group of diseases with different etiologies, pathogenesis, treatments, and outcomes =

Back 23

Focal Segmental Glomerulosclerosis (FSGS)

Front 24

how many variants based on morphology are there in primary FSGS?

Back 24

4

Front 25

this variant of FSGS is seen in HIV-assoicated nephropathy... it is very aggressive (ESRD in <1)

Back 25

collapsing variant

Front 26

most common cause of nephrotic disease in African-Americans?

Back 26

FSGS

Front 27

Most common presentation: insidious onset of asymptomatic proteinuria with frequent progression to nephrotic syndrome =

Back 27

FSGS

Front 28

LM: increased ECM, hyalinosis
May be confused with MCD on biopsy as pathologic features are focal and segmental
IF: trapping of IG and C3 in sclerotic regions
EM: diffuse effacement of foot processes, loss of podocytes and collapse of capillaries with increased ECM

finding in

Back 28

FSGS

Front 29

May be confused with MCD on biopsy ....

Back 29

FSGS

Front 30

LM: increased ECM, hyalinosis
May be confused with MCD on biopsy as pathologic features are focal and segmental

Back 30

FSGS

Front 31

EM: diffuse effacement of foot processes, loss of podocytes and collapse of capillaries with increased ECM

findings of

Back 31

FSGS

Front 32

what are beneficial treatments for FSGS (2)

Back 32

Corticosteroids and ACE inhibitors typically beneficial

Front 33

Periodic acid-Schiff (PAS) staining shows perihilar areas of segmental sclerosis and adjacent adhesions to Bowman's capsule

Back 33

FSGS

Front 34

Associated with small vessel disease (sclerosis) throughout body

Back 34

Diabetic Glomerulosclerosis (GS)

Front 35

Presents as microalbuminuria, progresses to overt proteinuria and perhaps nephrotic syndrome in 10-15 yrs post dx of type 1 or 2 diabetes

Back 35

Diabetic Glomerulosclerosis (GS)

Front 36

etiology of Generalized increase in BM material synthesis within microvasculature

Back 36

Diabetic Glomerulosclerosis (GS)

Front 37

LM finding of Diffuse thickening of BM region and proliferation and expansion of mesangium =

Back 37

Diabetic GS

Front 38

Kimmelstiel-Wilson nodules (PAS- and silver- positive acellular, nodular mesangial sclerosis) =

Back 38

Diabetic GS

Front 39

Hyaline arteriololar sclerosis =

Back 39

Diabetic GS

Front 40

~ 30% develop ESRD (leading cause in USA)

Back 40

Diabetic GS

Front 41

basement membrane (BM) is thickened, and there is an increase in mesangial matrix material.

Back 41

Diabetic GS

Front 42

Renal involvement typical in either AA or AL forms of systemic amyloidosis

Nonselective proteinuria; 60% develop nephrotic syndrome

Back 42

Amyloid Nephropathy

Front 43

which amyloid is associated with chronic inflammatory process

Back 43

AA Amyloid

seen in Amyloid Nephropathy

Front 44

person with nephrotic syndrome that has lots of plasma cells or multiple myeloma, what should you be thinking

Back 44

Amyloid Nephropathy

Front 45

which amyloid is associated with plasma cell/B cell neoplasm, derived from light chains of Ig molecules (multiple myeloma)

Back 45

AL amyloid

seen in Amyloid Nephropathy

Front 46

LM: Congo red stain: apple-green birefringence with polarized light = ?

Back 46

Amyloid Nephropathy

Front 47

Tx for AA amyloidosis?

Back 47

remember this is an inflammatory process so

potent anti-inflammatory agents (e.g., colchicine

Front 48

Tx for AL amyloidosis?

Back 48

chemotherapy used to treat multiple myeloma

Front 49

T/F

amyloid deposits are not periodic acid-Schiff–positive

Back 49

True

Front 50

Associated with hematuria, azotemia (elevated BUN), and variable subnephrotic proteinuria & edema

Back 50

Nephritic syndrome

Front 51

One of most common pediatric kidney diseases

Back 51

Acute Post-Infectious GN

Front 52

the following are what type of syndromes?

Acute post-infectious glomerulonephritis
Membranoproliferative glomerulonephritis
---Type 1 and Type 2
Lupus nephritis
IgA nephropathy
Anti-GBM glomerulonephritis

Back 52

Nephritic Syndromes

Front 53

pt has pharygitis, then 2-4 weeks after resolution they have a Type II HS rxn occur affecting the kidney, what do they have

Back 53

Acute Post-Infectious GN

Front 54

Most often related to nephritogenic strains of group A strep is the etiology of what?

Back 54

Acute Post-Infectious GN

Front 55

LM: proliferative GN
Early: increase mesangial matrix with increase PMNs
Late: mildly hypercellular glomeruli

Back 55

Acute Post-Infectious GN

Front 56

IF: “lumpy-bumpy” IgG, C3 around capillaries, mesangium

Back 56

Acute Post-Infectious GN

Front 57

EM: sub-epithelial humps

Back 57

Acute Post-Infectious GN

Front 58

prognosis for Acute Post-Infectious GN

Back 58

Majority improve to baseline within months

Front 59

Proliferation of glomerular cells, alteration in GBM, infiltration by WBCs

Back 59

Membranoproliferative GN (MPGN)

Front 60

Can occur at any age; most frequent in older children and younger adults
More prevalent in underdeveloped countries with high prevalence of chronic infections
Often have low levels of complement (C3)

Back 60

Type I Membranoproliferative GN (MPGN)

Front 61

in Type I Membranoproliferative GN (MPGN) where do immune complexes get deposited? 2

Back 61

mesangium

subendothelial capillary walls

Front 62

LM of glomerular crescents

Back 62

Type I Membranoproliferative GN (MPGN)

Anti-GBM GN

Rapidly Progressive GN

Front 63

Silverstain: doubling ("replication") of GBM...akak tram-tracking

seen in?

Back 63

Type I Membranoproliferative GN (MPGN)

Front 64

EM: subendothelial and mesangial dense deposits (immune complexes)

Back 64

Type I Membranoproliferative GN (MPGN)

Front 65

prognosis of: Treatment of underlying disease process (if secondary)
~ 50% patients progress to ESRD

Back 65

Type I Membranoproliferative GN (MPGN)

Front 66

More pronounced hypocomplementemia

Prognosis typically worse

no immune complex deposition... what is this?

Back 66

Type II MPGN: Dense Deposit Disease

Front 67

Extensive deposition of complement in GBM and mesangial matrix; virtually no Ig (NOT immune complex)

Back 67

Type II MPGN: Dense Deposit Disease

Front 68

Most patients have IgG autoantibody, the C3 nephritic factor (stabilizes activated C3 convertase (C3bBb)

Back 68

Type II MPGN: Dense Deposit Disease

Front 69

IF and EM: “ribbon-like” zone of increased density within the GBM and mesangial matrix

Back 69

Type II MPGN: Dense Deposit Disease

Front 70

Prognosis
Lack of effective treatment with variable progression
Some patients develop numerous glomerular crescents and picture of rapidly progressive GN (RPGN)
~ 50% develop CRF within 10 yrs
Relatively high recurrence within transplanted kidneys

Back 70

Type II MPGN: Dense Deposit Disease