Renal: Glomerular Diseases Flashcards Preview

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Flashcards in Renal: Glomerular Diseases Deck (97):
1

nephrotic syndrome is characterized by:

heavy proteinuria (>3.5 mg/day)

  • hypoalbuminemia (bc of heavy proteinuria)
  • edema
  • hyperlipidemia & lipiduria
  • normal complement levels

2

why is there hypercoagulability in nephrotic syndrome?

loss of anti-thrombin III

ATIII breaks up thrombin and coagulation factors so that you cannot make thrombin; therefore loss of ATIII = increased coagulation risk

3

list the renal diseases associated with nephrotic syndrome based on immunoglobulin deposition

4

____ is the most common cause of nephrotic syndrome in children

minimal change disease is the most common cause of nephrotic syndrome in children

5

describe the EM image seen in the condition in the image

minimal change disease

PAS-stained image = normal

EM: arrow = effacement of foot processes and absence of deposits

6

the condition seen in the image is associated with ____ (another condition)

the condition seen in the image is associated with Hodgkin's lymphoma

7

describe what is seen on physical exam in the condition seen in the image

normal BP, edema (periorbital and pedal)

8

>90% of children have complete remission proteinuria in the condition seen in the image within 8 weeks of ____ therapy

>90% of children have complete remission proteinuria in the condition seen in the image within 8 weeks of steroid therapy

9

in the condition seen in the, there is autoimmune destruction of ____ cells, facilitated by ____ cells

in the condition seen in the, there is autoimmune destruction of epithelial cells (podocytes), facilitated by T cells 

10

in the condition seen in the image, there is diffuse effacement of ____

in the condition seen in the image, there is diffuse effacement of foot processes of podocytes

11

the injury in the condition seen in the image results in increased ____ and subsequent massive ____

the injury in the condition seen in the image results in increased permeability and subsequent massive proteinuria

12

what condition shows the following results:

minimal change disease

13

the condition seen in the image can be secondary to:

  • secondary to:
    • HIV
    • morbid obesity
    • chronic reflux nephropathy
    • heroin use
    • malignancies (lymphoma)

14

the prognosis of the condition in the image depends on ____

the prognosis of the condition in the image depends on the degree of proteinuria

15

if a patient has HIV or does heroin and then develops nephrotic syndrome, which disease is most likely?

FSGS

16

describe the pathogenesis of the condition seen in the image

decreased renal mass 

compensatory hyperfiltration

↓​

intraglomerular HTN and hyperfiltration injury 

non-selective proteinuria

17

which condition shows the following lab results:

LM: segmental hyalinosis of some glomeruli

IF: negative (or non-specific IgM & C3)

EM: patchy fusion of the foot processes & effacement

FSGS

18

in minimal change disease, there is ___ fusion of the foot processes and effacement

whereas

in FSGS, there is ___ fusion of the foot processes and effacement

in minimal change disease, there is diffuse fusion of the foot processes and effacement

whereas

in FSGS, there is patchy fusion of the foot processes and effacement

19

describe presentation of the condition seen in the image

present with nephrotic syndrome

microscopic hematuria & HTN

20

the condition seen in the image does NOT respond to ___ and ultimately progresses to ____

the condition seen in the image does NOT respond to steroids and ultimately progresses to end-stage renal failure (ESRF)

21

describe what is seen in the EM image

membranous glomerulopathy

EM showing electron-dense deposits (arrow) along the epithelial side of the basement membrane (B); note the effacement of foot processes overlying deposits 

22

the condition seen in the image is caused by ___ deposition in the ____ zone

the condition seen in the image is caused by immune-complex deposition in the subepithelial zone

23

describe the pathogenesis of the condition seen in the image

membranous glomerulopathy

24

in the 2 conditions with the word "membranous", they are characterized by thickening of ____ due to ____ deposition

membranous nephropathy 

membranoproliferative glomerulonephritis

in the 2 conditions with the word "membranous", they are characterized by thickening of the membrane  due to immune-complex deposition

25

if a patient has hepatitis B, which nephrotic syndrome are they at risk of developing?

membranous nephropathy

26

if a patient has SLE, which nephrotic syndrome are they at risk of developing?

membranous nephropathy 

27

which condition shows the following lab results:

28

on EM of the condition seen in the image, there are subepithelial immune-complex deposits w/ alternating ___ and ____

on EM of the condition seen in the image, there are subepithelial immune-complex deposits w/ alternating spikes and domes

29

on immunofluorescence of the condition seen in the image, there are subepithelial deposits of ___ and ____

on immunofluorescence of the condition seen in the image, there are subepithelial deposits of IgG and C3

30

describe complications of the condition seen in the image

infections, hypercoagulability (ischemic heart disease), renal failure

31

what would indicate a poor prognosis of the condition seen in the image

males, >50 yrs old, >10 gm of proteinuria

32

what is the FIRST change seen in diabetic nephropathy?

high serum glucose leads to non-enzymatic glycosylation (NEG) of vascular basement membranes, resulting in hyaline arteriosclerosis

arteriosclerosis preferentially in efferent arterioles → increased backpressure in glomerulus → 

33

in diabetic nephropathy, initially hyperglycemia leads to ____

in diabetic nephropathy, initially hyperglycemia leads to hyperfiltration

34

the earliest lesions seen in diabetic nephropathy is expansion of ____ and thickening of ____

the earliest lesions seen in diabetic nephropathy is expansion of mesangial matrix and thickening of GBM

35

later lesions seen in diabetic nephropathy is diffuse global ____ with:

diffuse increase in ____

and diffuse thickening of ____

later lesions seen in diabetic nephropathy is diffuse global glomerulosclerosis with:

diffuse increase in mesangial matrix

and diffuse thickening of GBM

36

in diabetic nephropathy, ____ nodules can be seen which contain ___ & ____

in diabetic nephropathy, Kimmelstiel-Wilson nodules can be seen which contain lipids & fibrin

37

amyloid fibrils are composed of various normal soluble proteins that have undergone a misfolding event resulting in formation of abnormal protein with ____ structures

amyloid fibrils are composed of various normal soluble proteins that have undergone a misfolding event resulting in formation of abnormal protein with B-pleated sheet structures

38

in amyloidosis, organ damage & dysfunction is due to ____ and replacement of normal organ architecture with consequent loss of cellularity

in amyloidosis, organ damage & dysfunction is due to infiltration by amyloid fibrils and replacement of normal organ architecture with consequent loss of cellularity

39

which condition shows the following lab results:

amyloidosis

40

describe what condition is seen in the images

41

describe what is seen in the urine in nephritic syndrome

  • RBCs and/or RBC casts
  • granular casts
  • variable proteinuria
  • possibly WBC

42

list the normal complement level nephritic disorders (4)

  • IgA nephropathy/Henoch-Schonlein purpura
  • Alport's syndrome (hereditary nephritis)
  • SLE (class I, II, V)
  • benign hematuria

43

list the low complement level nephritic disorders (4)

  • PSGN
  • membranoproliferative glomerulonephritis
  • SLE (class III, IV)
  • bacterial endocarditis/infected ventriculoatrial shunt
  • cryoglobulinemia

44

____ is the most common cause of nephritic syndrome

mesangioproliferative glomerulonephritis (IgA nephropathy/Berger's disease) is the most common cause of nephritic syndrome

45

list the conditions associated with IgA nephropathy

  • hepatic cirrhosis
  • gluten enteropathy
  • HIV infection
  • minimal change disease
  • others: membranous, Wegener's, ankylosing spondylitis, small cell ca.

46

the condition seen in the image in children is part of ____

the condition seen in the image in children is part of Henoch-Schonlein Purpura Nephritis

  • kidneys = IgA nephropathy
  • skin = non-blanching purpuric rash
  • GI = mesenteric vasculitis → red infarct
  • joints = arthralgia

47

the condition seen in the image presents in adults 1-3 days after ____

the condition seen in the image presents in adults 1-3 days after respiratory (or GI) infection

resp. + GI are lined by mucous membranes and therefore secrete IgA

48

the condition seen in the image is associated with ____

the condition seen in the image is associated with liver cirrhosis, Celiac's disease, HIV and minimal change disease

49

describe the presentation of the condition seen in the image

episodic gross hematuria w/ a background of persistent hematuria, oliguria

50

describe what is seen on microscopy of the urine in the condition seen in the image

RBC casts

51

blood investigations in the condition seen in the image would show normal ____ levels, elevated ____ levels and _____

blood investigations in the condition seen in the image would show normal complement levels, elevated IgA levels and azotemia

52

describe what is seen on LM in the condition seen in the image

segmental areas of increased mesangial matrix & hypercellularity

53

describe what is seen on IF in the condition seen in the image

coarse granular (lumpy-bumpy) with mesangial & subendothelial IgA and C3

54

describe what is seen on EM in the condition seen in the image

mesangial and subendothelial IgA & C3

55

describe the complication of the condition seen in the image

can progress to Type II RPGN 

56

the condition seen in the image can occur 10 days after ____ or 3 weeks after ____

the condition seen in the image can occur 10 days after pharyngitis or 3 weeks after impetigo 

57

diagnosis of the condition seen in the image is with elevated titers of ___ or ____ in association with low ____

diagnosis of the condition seen in the image is with elevated titers of anti-streptolysin O Ab or anti-DNAse B in association with low complement levels

58

in the condition seen in the image, initially, there are ____ deposits which then become ____ which is responsible for ___

in the condition seen in the image, initially, there are subendothelial deposits which then become subepithelial humps which is responsible for epithelial cell damage & proteinuria

59

on LM of the condition seen in the image, what is seen?

  • hypercellular glomeruli: neutrophils + monocytes
  • proliferation of: mesangial, endothelial, epithelial cells
  • process is diffuse (entire lobules of all glomeruli)
  • closure of capillary loops due to proliferation & swelling of endothelial cells & leukocytes infiltration

60

in the condition seen in the image, there is closure of ____

in the condition seen in the image, there is closure of capillary loops due to proliferation & swelling of endothelial cells & leukocytes infiltration

61

describe what is seen on IF in the condition seen in the image

coarse, granular, (lumpy bumpy) deposits of IgG & C3 in mesangium & along capillary walls

62

in the EM of the condition seen in the image, there are ___

in the EM of the condition seen in the image, there are electron-dense deposits in subepithelial space; humps

63

a complication of the condition in the image is that it can progress to ____

a complication of the condition in the image is that it can progress to type II RPGN

64

the outcome of most cases of the condition seen in the image is ____

the outcome of most cases of the condition seen in the image is complete resolution

65

on LM of the condition seen in the image,  there is ___ expansion and ____

on LM of the condition seen in the image,  there is mesangial expansion and hypercellularity

66

what is a characteristic finding in the condition seen in the image?

duplication of the GBM = "tram-track" appearance

67

describe what is seen on EM in type I of the condition seen in the image

type I = subendothelial deposits (C3, +/- IgG, C1q, C4)

68

describe what is seen on EM in type II of the condition seen in the image

deposition of dense material along GBM (complement deposition, no immune complexes)

69

describe what is seen on EM in type III of the condition seen in the image

subendothelial, mesangial, subepithelial deposits (C3 +/- IgG)

70

describe what is seen in the image

MPGN: type I

note the discrete, electron-dense deposits (arrows) incorporated into the glomerular capillary wall between duplicated (split) BMs (double arrows)

71

describe what is seen in the image

MPGN: type II

dense-deposit disease (type II MPGN) 

there are dense homogenous deposits within the BM

72

in type I of the condition seen in the image, there is ___ activation via the ___ pathway

in type I of the condition seen in the image, there is complement activation via the classical pathway

73

in type II of the condition seen in the image, there is activation of the ___ pathway which leads to depressed ____ levels

in type II of the condition seen in the image, there is activation of the alternate pathway which leads to depressed C3 levels

74

list the 4 ways the condition seen in the image can present

  1. hematuria or proteinuria discovered on urinalysis
  2. acute nephritic syndrome with hematuria, HTN and edema
  3. recurrent episodes of gross hematuria
  4. insidious onset of edema and nephrotic syndrome

75

on LM of the condition seen in the image, there is proliferative GN with prominent ____ formation +/- segmental ____

on LM of the condition seen in the image, there is proliferative GN with prominent crescent formation +/- segmental necrosis

76

describe type I of the condition seen in the image; what kind of staining is seen on IF?

linear staining on IF

Anti-GBM disease, such as Goodpasture's syndrome

77

describe type II of the condition seen in the image; what kind of staining is seen on IF?

granular staining on IF

immune-complex disease; found in SLE, post-infectious, IgA, Henoch-Schonlein Purpura

78

describe type III of the condition seen in the image; what kind of staining is seen on IF?

no staining on IF; pauci-immune GN

Wegener's (c-ANCA)

microscopic polyangitis (p-ANCA)

Churg-Strauss (p-ANCA)

 

79

what are the crescents in the image composed of?

crescents = fibrin + macrophages + proliferating parietal cells

80

the best prognosis of the condition seen in the image is with patients with ___

the best prognosis of the condition seen in the image is with patients with treatable underlying disorders (such as SLE) or one that spontaneously remits (such as post-strep)

81

describe what is seen in the image

type I RPGN; anti-BM disease, such as Goodpasture's

82

on IF examination of lupus nephritis, it is positive for ____ which is also called a ____

on IF examination of lupus nephritis, it is positive for IgG, IgA, C3, IgM which is also called a full house

83

describe class I of lupus nephritis

minimal mesangial lupus nephritis

  • LM = normal 
  • IF & EM = mesangial immune deposits

84

describe class II lupus nephritis

mesangial proliferative lupus nephritis

  • mesangial immune deposits resulting in expansion & hypercellularity
  • clinical: mild disease, microscopic hematuria, proteinuria, nephrotic syndrome

85

describe class III lupus nephritis

focal segmental proliferative lupus nephritis

  • <50% glomeruli affected on LM
  • subendothelial & mesangial IC deposits; complement activation, influx of inflam. cells
  • clinical: hematuria, nephrotic syndrome, HTN, renal failure

86

describe class IV of lupus nephritis

diffuse proliferative lupus nephritis

  • >50% glomeruli affected on LM
  • marked deposition of IC in subendothelial and mesangium
  • crescents and necrotizing lesions
  • clinical: most common and severe form; hematuria, proteinuria, nephrotic syndrome, renal failure, low complements, high anti-DNA levels

87

describe class V lupus nephritis

membranous lupus nephritis

  • subepithelial immune complex deposits
  • diffuse thickening of GBM
  • clinical: same as idiopathic membranous; nephrotic syndrome, normal C3/C4, negative anti-DNA
  • IC deposits in blood vessels

88

describe class VI lupus nephritis

advanced sclerosing lupus nephritis

  • global sclerosis of >90% of glomeruli
  • advanced interstitial fibrosis and tubular atrophy
  • represents healing of prior inflammatory injury, advanced stages of chronic class III, IV, V lupus nephritis

89

disease activity of lupus nephritis can be monitored by serial measurements of ____ (4 things)

disease activity of lupus nephritis can be monitored by serial measurements of:

  • complements
  • anti-dsDNA Ab
  • ESR
  • CRP

90

the condition seen in the image is also called _____

the condition seen in the image is also called inherited nephritic syndrome

91

the condition seen in the image has a defect in ____

the condition seen in the image has a defect in α-5 subunit of type IV collagen

92

the genes associated with the condition seen in the image are....

COL4A3, COL4A4, COL4A5

COL4 = collagen 4

93

describe the presentation of the condition seen in the image

  • hematuria, periorbital edema, oligura
  • cataracts
  • sensorineural deafness

94

describe the blood levels of the condition seen in the image

NORMAL complements levels, azotemia (increased BUN + increased creatinine without uremic symptoms)

95

describe what is seen on LM in the condition in the image

initially normal → hyperfiltration → FSGS

96

describe what is seen on EM in the condition in the image

basket-weave appearance due to alternating thickening and thinning of lamina densa in BM

97

list complications of the condition seen in the image

  • can progress to nephrotic FSGS → anasarca & 3.5g of protein & fatty casts
  • deafness, blindness