10. Path of Glomerular Disease

Question 0

which cells of the glom filter particles by both size and charge?

Answer 0

podocytes, via the diaphragm across the filtration slit. Basement membrane also filters by charge and size. Endothelial cells (capillary) only filter by size.

Question 1

which cells in the glomerulus have negative charges?

Answer 1

both epithelial/podocytes and the endothelial capillary wall

Question 2

Type IV collagen is impt to which component of the glomerulus?

Answer 2

major component of the basement membrane. triple helical alpha molecules, in dimers or tetramer formations

Question 4

hereditary mutation in alpha chains of Type IV collagen leads to what disease?

Answer 4

Alport syndrome

Question 5

Filtration governed by what 4 factors?

Answer 5

size, charge, shape/flexibility of cells, hemodynamic forces

Question 6

most cases of glomerulonephritis involve what kind of mechanisms?

Answer 6

immune. deposits of immunoglobulins found in 70% of all GN patients

Question 7

what are the 2 major forms of Ab-associated injury?

Answer 7

-in situ -circulating immune complex nephritis

Question 8

what kind of injury is anti-GBM nephritis?

Answer 8

in situ, ab-associated injury

Question 9

what kind of injury is membranous GN?

Answer 9

in situ, ab-associated injury

Question 10

anti-GBM nephritis: antibodies are directed against what?

Answer 10

antigens intrinsic to the GBM

Question 11

Anti-GBM nephritis: where do immune complexes form and congregate?

Answer 11

in the GBM

Question 12

Anti-GBM: what kind of staining on IF?

Answer 12

linear

Question 13

Goodpasture’s is associated with which nephritis? mechanism?

Answer 13

associated with Anti-GBM nephritis. the Anti-GBM antibodies cross-react with alveolar BMs in lung and can lead to hemorrhage

Question 14

membranous GN: where do immune complexes form and deposit?

Answer 14

on the foot process: deposit on the sub epithelial (podocyte) side of the BM

Question 15

circulating immune complex nephritis: what is seen on EM?

Answer 15

deposits in mesangium and/or sub epithelium.

Question 16

circulating immune complex nephritis: what is pattern of complex deposition?

Answer 16

granular

Question 17

what is EM good to look for?

Answer 17

immune complex deposits, podocyte changes (effacement), GBM changes (microfibrils, thinning)

Question 18

4 characteristics of nephrotic disease?

Answer 18

proteinuria, hypoalbuminemia, edema, hypercholesterolemia

Question 19

if a child has nephrotic sx, what is it likely to be?

Answer 19

minimal change disease

Question 20

minimal change disease: appearance on light microscopy?

Answer 20

normal

Question 21

minimal change disease: appearance on IF?

Answer 21

normal/negative

Question 22

minimal change disease: appearance on EM?

Answer 22

widespread effacement of podocytes

Question 23

minimal change disease: severity? treatment?

Answer 23

benign. responds to steroids

Question 24

FSGS: how to differentiate from minimal change?

Answer 24

10% of glomeruli will have segmental sclerosis (areas of cap collapse, expanded mesangium)

Question 25

FSGS: appearance on light micro?

Answer 25

focal, segmental cap collapse and sclerosis

Question 26

FSGS: appearance on IF?

Answer 26

normal/negative

Question 27

FSGS: appearance on EM?

Answer 27

moderately widespread effacement of podocytes

Question 28

if a child is thought to have MCD and does not respond to steroids, what is the likely disease?

Answer 28

FSGS

Question 29

membranous GN: where are immune deposits?

Answer 29

subepithelial deposits in capillary wall, which evolve over time as body tries to re-sorb them

Question 30

membranous GN: appearance of GBM?

Answer 30

may see spikes and holes of GBM

Question 31

membranous GN: appearance on EM?

Answer 31

subepi deposits with spike/fringe projections, eventual incorporation into BM (4 stages)

Question 32

membranous GN: appearance on IF?

Answer 32

granular pattern along the GBM (IgG and C3)

Question 33

membranous GN: what are 4 stages?

Answer 33

-small numerous deposits on BM -BM sends out projections between deposits -individual spikes surround deposits -deposits incorporated into thickened BM

Question 34

diabetic nephrosis: appearance on light micro?

Answer 34

early on: glomerular hypertrophy, thickened GBM, mesangial sclerosis later: advancement of the above, K-W nodules, micro aneurisms, hyalinization

Question 35

diabetic nephrosis: appearance on IF?

Answer 35

negative (no immune complexes) may have linear IgG and albumin in GBM??

Question 36

diabetic nephrosis: appearance on EM?

Answer 36

thickened GBM, mesangial sclerosis

Question 37

amyloidosis: nephrotic or nephritic?

Answer 37

nephrotic

Question 38

memory trick for the nephrotic diseases?

Answer 38

DAM Fine Man: Diabetes, Amyloid, MembranousGN, FSGS, MCD (all cause proteinuria)

Question 39

memory trick for the nephritic diseases?

Answer 39

Peeing blood Means Lupus: Postinfectious, Membranoproliferative, Lupus (all cause hematuria)

Question 40

memory trick for the hematuric diseases?

Answer 40

hemATurIa Alport, Thin bm disease, IgA nephropathy

Question 41

what unique stains are used to dx amyloidosis?

Answer 41

congo red, polarized light

Question 42

amyloidosis: appearance with congo red?

Answer 42

salmon-colored glomeruli, nodular

Question 43

amyloidosis: appearance with polarized light?

Answer 43

apple green birefringence

Question 44

amyloidosis: appearance with IF?

Answer 44

positive for kappa or lambda light chain

Question 45

amyloidosis: appearance with EM?

Answer 45

random thin fibrils

Question 46

amyloidosis: appearance with light micro?

Answer 46

thickened mesangium, thickened cap walls

Question 47

post-infectious GN: prototypical disease?

Answer 47

post-strep

Question 48

post-infectious GN: appearance on IF?

Answer 48

granular deposits along GBM, especially C3

Question 49

post-infectious GN: appearance on EM?

Answer 49

classic sub epithelial hump deposits: almost round

Question 50

post-infectious GN: cellularity on light micro?

Answer 50

inflammatory cells (PMNs), swollen, hypercellular

Question 51

Membranoproliferative GN: most common GN associated with what?

Answer 51

Hep C and Hep B

Question 52

Membranoproliferative GN: appearance on light micro?

Answer 52

proliferative, hypercellular glomeruli with leukocytes gloms often hyperlobulated, with separation between lobules

Question 53

Membranoproliferative GN: appearance on EM or silver stain?

Answer 53

train track appearance due to duplicated BM, large subendo deposits

Question 54

Membranoproliferative GN: appearance on IF?

Answer 54

smudgy appearance due to bulky deposits, positive for C3 can be granular or globular

Question 55

lupus: appearance on EM?

Answer 55

extensive mesangial and peripheral deposits

Question 56

lupus: appearance on IF?

Answer 56

Full House staining! positive for many different reactants (IgG, IgA, IgM, C3, light chains)

Question 57

lupus: what is the spectrum of the disease?

Answer 57

6 different classes encompassing wide disease spectrum

Question 58

Alport disease: appearance on EM?

Answer 58

basket weave pattern on GBM due to fragmentation, splitting

Question 59

Alport disease: clinical presentation?

Answer 59

hematuria, nerve deafness, ocular abnormalities

Question 60

Alport: cause of disease?

Answer 60

X linked recessive mutations to collagen type IV

Question 61

Thin Basement Membrane Disease: Appearance on EM?

Answer 61

generalized thinning of BM, some areas more than others

Question 62

Alport: progression of disease?

Answer 62

often progresses to ESRD

Question 63

Thin Basement Membrane Dz: progression?

Answer 63

benign, non progressive

Question 64

IgA nephropathy: appearance on IF?

Answer 64

positive for IgA staining in the paramesangial area

Question 65

IgA nephropathy: appearance on EM?

Answer 65

mesangial deposits (mesangial areas have more than 3 nuclei)

Question 66

most common cause of GN worldwide?

Answer 66

IgA nephropathy. particularly common in asians

Question 67

Crescentic GN: sign of what?

Answer 67

severe glomerular injury

Question 68

Crescentic GN: appearance on light micro?

Answer 68

cellular proliferation along inside of Bowman's capsule, fills part of Bowman's space

Question 69

Crescentic GN: what are the three types? what tests distinguish them?

Answer 69

Anti-GBM disease, immune complex mediated disease, Pauci-immune test: IF staining pattern

Question 70

Crescentic GN with Positive Linear IF: what disease?

Answer 70

Anti-GBM

Question 71

Crescentic GN with Positive Granular IF: what disease?

Answer 71

Immune complex mediated disease lumpy-bumpy on IF

Question 72

Crescentic GN with Negative IF: what disease?

Answer 72

Pauci-immune

Question 73

If Pauci-immune disease, what should I test for? what will results tell me?

Answer 73

test for ANCA (anti-neutrophilic circulating antibodies) if Positive, there is association with systemic vascular disease

Question 74

Dx?

Answer 74

Amyloid

Question 75

Dx?

Answer 75

Crescentic GN

Question 76

dx?

Answer 76

crescentic GN

Question 77

dx?

Answer 77

DM

Question 78

dx?

Answer 78

DM

Question 79

Dx?

Answer 79

DM

Question 80

dx?

Answer 80

FSGS

Question 81

dx?

Answer 81

IgA nephropathy

Question 82

dx?

Answer 82

minimal change disease

Question 83

dx?

Answer 83

MGN

Question 84

dx?

Answer 84

MPGN

Question 85

dx?

Answer 85

MPGN

Question 86

dx?

Answer 86

PIGN

Question 87

dx?

Answer 87

PIGN

Question 88

dx

Answer 88

lupus