Step 1: Renal Diseases

Front 1

3 types of Glomerular dz. If they have subtypes, give those as well.
- what 4 things should you assess for when looking at glomerula morphology? (and what types of dz would they indicate)

Back 1

Nephrotic (Primary, Secondary), Nephritis, RPGN (anti-GBM, Immune-complex, Pauci-immune)
- Cellularity = proliferative, Capillary wall thickness = membranous, Crescents = crescentic, (^mesangium, necrosis, thombosis, amyloid, sclerosis) = sclerosis

Front 2

Child presents with peripheral and periorbital edema. proteinuria (1.5g/day). Immunofluorescent staining (IF) shows a granular pattern. Hypercellular (neutrophils). Kid had a rash last week, and felt a little sick.
- dx?
- seen on EM?
- LM?
- tx?

Back 2

- actue poststreptococcal glomerulonephritis
- subepithelial immune complex humps
- glomeruli enlarged and HYPERCELLULAR.
- resolves spontaneously.

Front 3

What is a crescent?
- implies what type of progression?
- seen in which classes of dz(s)?

Back 3

fibrin and plasma proteins w/glomerular parietal cells, monocytes, and macrophages.
--> thing in Bowman's space that has nuclei!!

- rapidly progressive
- RPGN (antiGBM, immune complex, and pauci-immune)

Front 4

Linear GBM IF staining (usually w/circulating anti-GBM Ab)...
- what's the next thing to look for? Dz if you find it? If you don't?

Back 4

- lung hemorrhage
+ = Goodpasture's
- = Anti-GBM glomerulonephritis

Front 5

Paucity of glomerular IF staining for Ig (and circulating ANCA is seen):
- no systemic vasculitis?
- vascultitis alone?
- w/granulomas?
- w/granulomas, asthma, and eosinophilia?

Back 5

- Pauci-immune GN
- Microscopic polyangitis
- Wegener's Granulomatosis
- Churg-Strauss Syndrome

Front 6

Granular IF usually means what?
- IgA dominance w/o vasculitis?
- IgA and systemic vasculitis?
- IgG/IgM/IgA w/ SLE sx?
- clinical hx of infection, w/ subepithelial humps on EM?
- mesangiocapillary changes by EM?
- numerous supepithelial deposits by EM?
- GBM dense deposits by EM?
- 20nm fibrils seen on EM?

Back 6

immune complex deposition.
- IgA nephropathy
- H-S pupura
- Lupus nephritis
- Acute Post-infectious GN
- Type I MPGN (mesangial cells proliferate)
- Membranous GN
- Type II MPGN (mesangial cells proliferate)
- Fibrillary GN.

Front 7

what is the pathogenesis of crescents? what do they denote?

Back 7

PMNs cause necrosis --> GBM gets killed off, and it can't filter anymore... so macrophages, fibrin, and inflammatory mediators come out.

Denote SEVERITY/rapidity.

Front 8

Crescents present.
- vasculitis alone?
- vasculitis w/ granulomas?
- lung involvment with anti-GBM?
+ staining pattern expected?
- vasculitis w/granulomas and asthma, eosinophils?

Back 8

- microscopic polyarteritis/polyangitis (pANCA)
- Wegener's (cANCA)
- Goodpasture's syndrome (type II hyperS)
+ linear IF staining.
- Churg Strauss syndrome.

Front 9

How can you differentiate MPGN I from II?
- what do both show?
- are they nephritic or nephrotic?

Back 9

Type I has "tram-track" appearance due to GBM splitting caused by mesangial ingrowth.

Type II = "Dense deposits"

Subendothelial immune complexes (IC's) w/ GRANULAR IF.

Can be either!

Front 10

Given the name, list nephrotic or nephritic:
- membranoproliferative GN
- Proliferative GN
- minimal change
- Crescentic GN
- membranous GN

Back 10

Nephrotic
Minimal change glomerulopathy
Membranous glomerulopathy
--Membranoproliferative GN -- either/or
Proliferative GN
Crescentic GN
Nephritic

Front 11

What is the most common cause of death in SLE?
- what type of immune complexes are involved? Where do they deposit?
- staining IF pattern?
- what else can SLE present as?

Back 11

)Diffuse) proliferative GN
- subendothelial DNA-anti-DNA IC's --> "wire-looping" of capillaries.
- granular
- Membranous Glomerulopathy, a Nephrotic syndrome

Front 12

Pt presents with Azotemia (elevated blood nitrogen products BUN>20), decreased urine production (oliguria), blood in urine, and RBC casts in urine. proteinuria is @ 1.5g/day. Pt complains of deafness, and ocular/nervous issues.
- dz?
- etiology?
- expect to see what on EM?

Back 12

Alport's syndrome
- mutation in type IV collagen
On EM, the basement membrane is seen to be "split".

Front 13

Pt present with BUN of 30, decreased urine production, and RBC casts in Urine. IgA levels are high. Pt just had a bout with gastroenteritis.
- dz?
- see histologically/LM?
- expect to see what on IF?
- what would the systemic form of this dz be called? What sx would you see?

Back 13

Berger's dz (IgA GN); often presents post URI or acute gastroenteritis
- increased cellularity, normal wall thickness, no crescents, +/- proliferative mesangium.
- IC deposited in mesangium.
- H-S purpura: would have systemic vasculitis too.

Front 14

Most common glomerular dz in HIV pts? Is it more severe in them?
- etiology of dz?
- racial predominance?
- pathogenesis?
- type of dz presentation? (nephrotic/nephritic)
- what is the material you see on LM?
- IF pattern?

Back 14

Focal Segmental Glomerular Sclerosis; presents more severely in HIV pts.
- kidneys "overworking"
- AA's
- accumulation of acellular material (plasma proteins/lipids) in mesangium.
- nephrotic
- hyaline (pink) material... looks sort of like it's filling up half the glomeruli
- granular Ig and complement trapped in mesangium... this is the "pink" material lighting up!

Front 15

What glomerular dz usually progresses slowly to CRF, and can present as either nephrotic or nephritic syndrome?
- IF appearance?
- associations for various types?
- how can you tell the difference on EM?

Back 15

MPGN
- subendothelial IC w/ granular IF.
- Type I ~ HBV > HCV [GBM splitting w/"tram-track" appearance]
- Type II ~ C3 nephritic factor. [dense deposits]

Front 16

If SLE is going to present as a nephrotic syndrome, which one will it be?
- what class presentation is this?
- what is it's nephritic presentations?
- What is class VI?

Back 16

Membranous glomerulonephritis (it's proliferative GN when it presents with nephritic)
- class V
- class III/IV: Proliferative GN (Focal/Diffuse)
- class II: Mesangioproliferative GN
- Class VI is chronic sclerotic GN

Front 17

What is the most common cause of adult nephrotic syndrome?
- LM?
- EM?
- two pathogenesis?
- IF?

Back 17

Membranous Glomerulonephropathy.
- thick capillary wall /GBM...
- deposits are on outside of GBM just under the podocyte foot processes. "Spike and Dome"
1. Ab b/subepithelial Ag
2. Ag-Ab complex deposits
- granular

Front 18

Effacement of foot processes is common in what dz?

Back 18

all forms of glomerular injury

Front 19

+ Congo red stain think what?
-what type of birefringenence?
- associated with which conditions?
- LM?

Back 19

Amyloid nephropathy
- apple-green
- MM, TB, RA
- thick GBM, thick mesangium, thick artery walls (Thick everything)

Front 20

What can Thrombotic microangiopathy cause?
- cells seen on blood smear?
- pathology?
- associated dz?

Back 20

Acute Renal Failure
- schistocytes
- glomerular cap. thomboses/stenosis/necrosis.
- TTP/HUS.

Front 21

Thick mesangium-nodules, along with thick GBM, and thick arteriolar walls i/ a pt w/ diabetes?
- pathogenesis?
- EM?
- IF? Why?
- names of these nodules of mesangium?

Back 21

Diabetic Glomerulonephropathy/sclerosis.
- Nonezymatic glycosylation of GBM and efferent arterioles ^^ permeability... glycosylated plasma protein insudates into GBM/mesangium
- Uniformly thick GBM
- linear IgG (it's trapped along with everything else)
- K-W nodules
-

Front 22

how do you gauge the thickness of the GBM?

Back 22

it should be ~ height of one podocyte foot process. if it's bigger, pathology.

Front 23

What nephrotic syndrome is common in kids, and associated w/ lymphoma sometimes?
- LM?
- EM?
- shows a selective loss of what? why?
- tx?
- dz is aka?

Back 23

Minimal change glomerulopathy
- normal
- just foot process effacement (kinda stuck together)
- albumin; lost of GBM polyanions (charge barrier?)
- responds to corticosteroids!
- lipoid nephrosis