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M2 Renal/FEK > 10. Path of Glomerular Disease > Flashcards

10. Path of Glomerular Disease Flashcards

0
Q

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

A

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

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1
Q

which cells in the glomerulus have negative charges?

A

both epithelial/podocytes and the endothelial capillary wall

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2
Q

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

A

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

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4
Q

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

A

Alport syndrome

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5
Q

Filtration governed by what 4 factors?

A

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

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6
Q

most cases of glomerulonephritis involve what kind of mechanisms?

A

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

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7
Q

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

A

-in situ -circulating immune complex nephritis

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8
Q

what kind of injury is anti-GBM nephritis?

A

in situ, ab-associated injury

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9
Q

what kind of injury is membranous GN?

A

in situ, ab-associated injury

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10
Q

anti-GBM nephritis: antibodies are directed against what?

A

antigens intrinsic to the GBM

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11
Q

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

A

in the GBM

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12
Q

Anti-GBM: what kind of staining on IF?

A

linear

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13
Q

Goodpasture’s is associated with which nephritis? mechanism?

A

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

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14
Q

membranous GN: where do immune complexes form and deposit?

A

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

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15
Q

circulating immune complex nephritis: what is seen on EM?

A

deposits in mesangium and/or sub epithelium.

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16
Q

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

A

granular

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17
Q

what is EM good to look for?

A

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

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18
Q

4 characteristics of nephrotic disease?

A

proteinuria, hypoalbuminemia, edema, hypercholesterolemia

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19
Q

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

A

minimal change disease

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20
Q

minimal change disease: appearance on light microscopy?

A

normal

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21
Q

minimal change disease: appearance on IF?

A

normal/negative

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22
Q

minimal change disease: appearance on EM?

A

widespread effacement of podocytes

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23
Q

minimal change disease: severity? treatment?

A

benign. responds to steroids

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24
Q

FSGS: how to differentiate from minimal change?

A

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

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25
Q

FSGS: appearance on light micro?

A

focal, segmental cap collapse and sclerosis

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26
Q

FSGS: appearance on IF?

A

normal/negative

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27
Q

FSGS: appearance on EM?

A

moderately widespread effacement of podocytes

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28
Q

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

A

FSGS

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29
Q

membranous GN: where are immune deposits?

A

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

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30
Q

membranous GN: appearance of GBM?

A

may see spikes and holes of GBM

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31
Q

membranous GN: appearance on EM?

A

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

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32
Q

membranous GN: appearance on IF?

A

granular pattern along the GBM (IgG and C3)

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33
Q

membranous GN: what are 4 stages?

A

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

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34
Q

diabetic nephrosis: appearance on light micro?

A

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

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35
Q

diabetic nephrosis: appearance on IF?

A

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

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36
Q

diabetic nephrosis: appearance on EM?

A

thickened GBM, mesangial sclerosis

37
Q

amyloidosis: nephrotic or nephritic?

A

nephrotic

38
Q

memory trick for the nephrotic diseases?

A

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

39
Q

memory trick for the nephritic diseases?

A

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

40
Q

memory trick for the hematuric diseases?

A

hemATurIa Alport, Thin bm disease, IgA nephropathy

41
Q

what unique stains are used to dx amyloidosis?

A

congo red, polarized light

42
Q

amyloidosis: appearance with congo red?

A

salmon-colored glomeruli, nodular

43
Q

amyloidosis: appearance with polarized light?

A

apple green birefringence

44
Q

amyloidosis: appearance with IF?

A

positive for kappa or lambda light chain

45
Q

amyloidosis: appearance with EM?

A

random thin fibrils

46
Q

amyloidosis: appearance with light micro?

A

thickened mesangium, thickened cap walls

47
Q

post-infectious GN: prototypical disease?

A

post-strep

48
Q

post-infectious GN: appearance on IF?

A

granular deposits along GBM, especially C3

49
Q

post-infectious GN: appearance on EM?

A

classic sub epithelial hump deposits: almost round

50
Q

post-infectious GN: cellularity on light micro?

A

inflammatory cells (PMNs), swollen, hypercellular

51
Q

Membranoproliferative GN: most common GN associated with what?

A

Hep C and Hep B

52
Q

Membranoproliferative GN: appearance on light micro?

A

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

53
Q

Membranoproliferative GN: appearance on EM or silver stain?

A

train track appearance due to duplicated BM, large subendo deposits

54
Q

Membranoproliferative GN: appearance on IF?

A

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

55
Q

lupus: appearance on EM?

A

extensive mesangial and peripheral deposits

56
Q

lupus: appearance on IF?

A

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

57
Q

lupus: what is the spectrum of the disease?

A

6 different classes encompassing wide disease spectrum

58
Q

Alport disease: appearance on EM?

A

basket weave pattern on GBM due to fragmentation, splitting

59
Q

Alport disease: clinical presentation?

A

hematuria, nerve deafness, ocular abnormalities

60
Q

Alport: cause of disease?

A

X linked recessive mutations to collagen type IV

61
Q

Thin Basement Membrane Disease: Appearance on EM?

A

generalized thinning of BM, some areas more than others

62
Q

Alport: progression of disease?

A

often progresses to ESRD

63
Q

Thin Basement Membrane Dz: progression?

A

benign, non progressive

64
Q

IgA nephropathy: appearance on IF?

A

positive for IgA staining in the paramesangial area

65
Q

IgA nephropathy: appearance on EM?

A

mesangial deposits (mesangial areas have more than 3 nuclei)

66
Q

most common cause of GN worldwide?

A

IgA nephropathy. particularly common in asians

67
Q

Crescentic GN: sign of what?

A

severe glomerular injury

68
Q

Crescentic GN: appearance on light micro?

A

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

69
Q

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

A

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

70
Q

Crescentic GN with Positive Linear IF: what disease?

A

Anti-GBM

71
Q

Crescentic GN with Positive Granular IF: what disease?

A

Immune complex mediated disease lumpy-bumpy on IF

72
Q

Crescentic GN with Negative IF: what disease?

A

Pauci-immune

73
Q

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

A

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

74
Q

Dx?

A

Amyloid

75
Q

Dx?

A

Crescentic GN

76
Q

dx?

A

crescentic GN

77
Q

dx?

A

DM

78
Q

dx?

A

DM

79
Q

Dx?

A

DM

80
Q

dx?

A

FSGS

81
Q

dx?

A

IgA nephropathy

82
Q

dx?

A

minimal change disease

83
Q

dx?

A

MGN

84
Q

dx?

A

MPGN

85
Q

dx?

A

MPGN

86
Q

dx?

A

PIGN

87
Q

dx?

A

PIGN

88
Q

dx

A

lupus

M2 Renal/FEK (20 decks)

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