Re Glomerular Pathology I: Nephrotic Synd

Front 1

silver stain in normal glomerulus shows...

Back 1

highlights BM, Cap wall, masangial cells

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tubules in cortex are mostly...

Back 2

proximal tubules
(90%)

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visceral epithelial cells are...

Back 3

podocytes

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parietal epithelial cells are...

Back 4

lining of BC

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filter layer (most) responsible for Ultrafiltrate...

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BM
size and charge filtered
(albumin small enough to come out otherwise)

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negative charges in BM from

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Type IV collagen, heparin sulfate, proteoglycans

Front 7

layers of BM ONLY seen on...

Back 7

EM
(or cartoon)

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glomerular dz in adults

Back 8

mostly secondary
-diabetic GN
-amyloidosis

primary dz is 60%
-Membranous GN
-FSGS
-minimal change
-membranoproliferative (mostly kids)

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glomerular dz in children

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95% are primary dz
-minimal change glomerulopathy (65%)
-FSGS
-membranoproliferative (kid only)
-membranous (SLE or inf)
-secondary

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clinical manifestations of glomerular dz

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1-asymptomatic proteinuria
2-nephrotic syndrome
3-asymptomatic hematuria
4-nephritic syndrome
*glomerulonephritis
*acute glomerulonephritis
*crescentic glomerulonephritis
*chronic glomerulonephritis
5-end stage renal dz

Front 11

clinical picture of nephrotic syndrome

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edema, proteinuria, hyperlipidemia

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mechanism of edema in nephrotic syndrome

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renal injury leading to:
-increased g cap perm
(proteinuria, decreased onc pressure)

-inappropriate RAS/aldost
(lowers plasma volume & increases RAS/Ald activation)

-decreased glomerular filtration fraction & impaired Na excretion

Front 13

child
proteinuria
normal LM
IMFL neg for Ig's & C3's

Back 13

Minimal Change dz
-oval fat bodies (from "proteinacious backup")
-EM shows fusion of foot processes

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possible mechanism for primary minimal change disease

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change in charge on BM
-loss of GC polyanions
-Lymphokines

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prognosis & tx for minimal change dz

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excellent prognosis
responds to steroids

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conditions associated with minimal change GN

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neoplasms
-Hodgkin's, NHL, carcinoma
Allergy
Drugs
-Li, NSAIDS, Probenecid
AIDS
Congenital Nephrotic synd
DM
other glomerular dz

Front 17

"maltese cross figure"

Back 17

oval fat bodies
in glomerular nephropathy
esp minimal change disease

Front 18

most common cause of nephrotic syndrome in adults

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membranous glomerulopathy
assoc'd w/RV thrombosis (hyperviscosity)
Immune complex deposition in kidney

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glomerulopathy associated with hyperviscosity

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membranous glomerulopathy
(assoc w/RV thrombosis)

Front 20

LM with prominant/thickened cap loops "wire loops" and spikes

subepithelial deposits

effaced foot processess

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membranous glomerulopathy
-IMFL w/pos IgG

need biopsy (EM) to disting primary vs secondary

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primary membranous glomerulopathy

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affects only BM
"subepithelial deposits"

Front 22

secondary membranous glomerulopathy

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affects BM, mesangial, etc

requires work up for malignancy and ANA

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common cause of nephrotic syndrome in adults & children

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FSGS- focal, segmental glomerular sclerosis

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nephropathy associated with hyperperfusion injury

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FSGS
primary causes unknown
secondary causes:
-HIV
-IV Heroine
-reflux nephropathy
-parenchymal shrink
-GN

Front 25

LM with foam cells, podocyte effusion, enlarged glomeruli

IMFL pos for IgM & C3

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FSGS

IgM & C3 on sclerotic BM & some mesangials

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hypocomplementemia w/hematuria

LM: very cellular w/tram tracking, immune deposits w/trichrome

IMFL: pos for complement & IgG

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membranoproliferative glomerulonephritis

mixed syndrome

Type I assoc w/Hep C, lymphoprolif, in-dwelling caths
EM: mesangial interposition & subendo Immune deposits

Type II high recurr rate w/renal transplants
EM: BM transformation
"Dense Deposit Dz"

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Dense Deposit Disease

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Type II membranoproliferative GN

cigar shaped dense deposits

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Cigar Shaped Dense Deposits

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Type II membranoproliferative GN

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most common cause of end stage renal disease in USA

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diabetic glomerulosclerosis

first presents as asymptomatic proteinuria

b/c glycosylation of IgG

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LM: Nodular mesangial expansion

IMFL: linear staining of BM for IgG

EM: thick BM (densa), expanded mesangials

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Diabetic G-sclerosis
"Kimmelstiel-Wilson Nodules"

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LM:
congo red stain pos for mesang & vessel

cotton candy expansion of mesangium

apple green bifringence

EM: randomly oriented fibrilary tissue

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renal amyloidosis

2 Types
- AA: assoc w/lt chain, pos for kappa & lambda

-AL: devoid of lt chain, kappa or lambda neg, assoc w/inflamm or hered states

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beta sheets found in ...

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amyloid dz

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nephrotic syndrom associated dz's

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minimal change dz
(children)
- oval fat bodies
-effused foot process on EM

membranous glomerulopathy
(adults)
- wire loops, spikes, imm complex

FSGS
(both)
-foam cells
-IgM, C3
-hyperperfusion inj

MPGN- mixed synd, complement activation, tram tracking
-type I: Hep C
-type II: "dense body" recurs in renal transplant

Diabetic glomerulosclerosis
-glycosylated IgG
-nodular mesangial expansion
-THICK BM

amyloidosis
-AL or AA
-polarizes (immunofixation)
-beta sheets (congo red mol's)