Glomerular Disease Flashcards

1
Q

what are all possible patterns seen on light microscopy?

A
  1. normal
  2. too many cells
  3. too many cells plus inflammatory cells
  4. sclerosed
  5. thickened membrane
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2
Q

What are possible patterns seen in IF

A
  • linear GBM
  • finely granular
  • coarse on mesangium
  • coarse on GBM
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3
Q

what is the classical clinical symptom for nephritic

A

hematuria

red cell casts

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4
Q

name 3 diseases presenting as primarily nephritic syndrome

A
  1. acute proliferative (poststreptococcal) glomerulonephritis
  2. non-streptococcal acute glomerulonephritis
  3. rapidly progressive glomerulonephritis
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5
Q

When does acute proliferative (poststreptococcal) glomerulonephritis occurs

A

1-4 weeks post strep skin or pharynx infection

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6
Q

who does acute proliferative (poststreptococcal) glomerulonephritis occur in

A

6-10 yr old

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7
Q

What immune response is mediated in acute proliferative (poststreptococcal) Glomerulonephritis? what are anti–streptococcal Abs and complement levels

A

immune-Complex

  • elevated anti-streptococcal Abs
  • low complement levels
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8
Q

What is seen in light microscopy for acute proliferative (poststreptococcal) glomerulonephritis

A

Proliferation - enlarged hypercellular glomeruli

neutrophil and monocyte infiltration

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9
Q

What is seen in IF for acute proliferative (poststreptococcal) glomerulonephritis?

A

Granular deposits of IgG, IgM, C3, mesangium along GBM

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10
Q

What is seen in electron microscopy for acute proliferative (poststreptococcal) glomerulonephritis

A

“humps”

epithelial side of basement membrane (subepithelial)

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11
Q

What are some symptoms seen in children with proliferative (poststreptococcal) glomerulonephritis

A

fever, malaise, nausea, oliguria, hematuria

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12
Q

prognosis for children with proliferative (poststreptococcal) glomerulonephritis

A
  • 95% recover with conservative toxicity, Na, and water balance
  • 1% develop rapidly progressive glomerulonephritis
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13
Q

what are signs and symptoms for adults with proliferative (poststreptococcal) glomerulonephritis

A

sudden hypertension
edema
elevated BUN

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14
Q

What is type I rapidly progressive Glomerulonephritis ? correlated disease?

A

Anti- GBM antibodies

Goodpastures syndrome

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15
Q

What is type II rapidly progressive glomerulonephritis? Correlated Disease?

A

Immune complex deposition

  • SLE
  • Henoch-Schoenlein Purpura ( IgA nephropathy)
  • post infectious glomerulonephritis
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16
Q

What is type III rapidly progressive glomerulonephritis

A

Pauci-Immune

  • Wegener granulomatosis
  • Microscopic polyangiits
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17
Q

What does rapidly progressive glomerulonephritis look like under light microscopy

A

Crescents

  • infiltration of monocytes and macrophages
  • proliferation of parietal epithelial cells lining Bowman’s capsule
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18
Q

What is the IF for rapidly progressive IF for

Type I, II, and III

A

I: linear GBM, Ig and complement

II: granular immune deposits

III: little or no deposition

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19
Q

What is electron microscopy for rapidly progressive glomerulonephritis

A

ruptures in GMB

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20
Q

What are symptoms for goodpasture

A

hemoptysis and pulmonary hemorrhage

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21
Q

What antibody is circulating with goodpasture

A

anti-GBM abs

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22
Q

What antibody is circulating with Wegener

A

c- ANCA

PRC

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23
Q

What antibody is circulating with SLE

A

anti-nuclear abs

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24
Q

What are general signs and symptoms for rapidly progressive glomerulonephritis

A

hematuria

RBC casts

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25
What happens in nephrotic syndrome Pathophysiology
- increased basement membrane permeability - urinary loss of plasma protein - proteinura greater than 3.5 gm/day - edema
26
Name 4 diseases that are nephrotic syndromes
- membranous nephropathy - minimal-change disease - focal segmental glomerulosclerosis - membranoproliferative glomerulonephritis
27
What is the most common nephrotic syndrome in Caucasian adults
membranous nephropathy
28
what is the pathogenesis of membranous nephropathy
immune-complex mediated
29
What happens to Glomeruli in membranous nephropathy
diffuse thickening of glomerular capillary wall
30
What is IF for membranous nephropathy
Granular IgG and C3
31
What does membranous nephropathy look like in EM
immune-complex deposits between GBM and epithelial cells | "spikes and Domes"
32
What are clinical features of membranous nephropathy
insidious onset - hematuria - mild hypertension
33
What is the most common cause of nephrotic syndrome in children
minimal-change in disease
34
what is etiology and pathogenesis of minimal-change disease
immunologically mediated although lacks immune-complex mechanisms
35
What is a good treatment for minimal-change disease
steroids
36
If a child had this then they might get minimal-change disease
- previous respiratory infection | - immunization
37
what is the light microscopy for minimal-change disease
no change
38
what is the IF for minimal-change disease
No Ig or complement deposits | - maybe weak staining
39
What is Em for minimal change disease
effacement of foot processes of visceral epithelial cells | - no electron dense deposits
40
What is a clinical feature for minimal change disease
massive proteinuria
41
what is treatment responds rapidly with minimal-change disease
corticosteroid
42
Focal segmental glomerulosclerosis is associated with other sytmptoms
HIV heroin addiction sickle-cell disease morbid obesity
43
What is the pathogenesis of focal segmental glomerulosclerosis
visceral epithelial damage
44
What genes are involved with focal segmental glomerulosclerosis
NPHS1 | NPHS2
45
What is the light microscopy for focal segmental glomerulosclerosis
- sclerosing some glomeruli ( focal) - segmental - affected capillary loops collapse - segmental hyalinosis
46
What if the IF for focal segmental glomerulosclerossi
IgM and C3 in sclerotic areas
47
What is the EM for focal segmental glomerulosclerosis
- effacement of foot processes | - detachment of epithelial cells and denudation of GBM
48
What can be used to treat focal segmental glomerulosclerosis
poor response to corticosteroid
49
who has better prognosis with focal segmental glomerusclerosis
children
50
Secondary membranoproliferative glomerulonephritis is associated with what disease
``` SLE HepB HepC endocarditis HIV alpha 1 antitrypsin deficiency ```
51
What is the pathogenesis for Type I membranoproliferative glomerulonephritis
- immune complexes | - activation of classical and alternative complement pathways
52
what is the pathogenesis for Type II membranoproliferative glomerulonephritis
alternative complement pathway
53
What is seen in Light microscopy for membranoproliferative glomerulonephritis
- hypercellular: proliferation of mesangial cells and proliferation of capillary endothelium - glomerular capillary wall "tram-track" apperance
54
What is seen in IF for membranoproliferative glomerulonephritis Type I
- C3 in granular pattern | - IgG, C1q and C4
55
What is seen in IF for membranoproliferative glomerulonephritis Type II
C3 - irregular granular pattern or linear NO IgG or C1 or C4
56
What does membranoproliferative glomerulonephritis Type I look like in EM
subENDOthelial electron-dense deposit
57
What does membranoproliferative glomerulonephritis Type II look like in EM
- dense material into GBM | - 'ribbon-like'
58
who does membranoproliferative glomerulonephritis usually occur in
children, adolescents and young adults
59
Name 3 isolated glomerular abnormalities
1. IgA nephropathy 2. Alport syndrome 3. think basement membrane lesion
60
What is the pathogenesis of IgA nephropathy
- genetic or acquired abnormality of immune regulation
61
Where does IgA deposit in IgA nephropathy
mesangium
62
What is the light microscopy for IgA nephropathy
- Mesangial widening due to proliferation and endocapillary proliferation
63
What is IF for IgA nephropathy
1. Mesangial deposition of IgA | 2. C3, IgG, IgM often
64
What is IgA nephropathy look like on EM
dense deposits in mesangium
65
what is another name for IgA nephropathy
Berger syndrome
66
What is the classical symptoms for IgA nephropathy
hematuria
67
How is Alport syndrome inherited
X-linked
68
what are clinical symptoms of Alport syndrome
1. hematuria with progression to chronic renal failure 2. nerve deafness 3. Eye disorders
69
what is the pathogenesis for alport sydnrome
- Abnormal alpha3, alpha4 or alpha 5 chains in collagen IV | - defective assembly of collagen IV
70
What is the EM for alport syndrome
Early lesion: GBM thinning Fully developed: GBM alterating thick and thin; splitting and layering of lamina densa
71
What are clinical symptoms for alport syndrome
Hematuria | red cell casts
72
what are clinical symptoms of thin basement membrane lesion? prognosis
-asymptomatic hematuria +/_ proteinuria - renal function preserved - prognosis excellent
73
what disease shows subepithelial humps
acute proliferative glomerulonephritis
74
what disease shows epimembranous deposists
membranous nephropathy
75
what disease shows subendothelial deposists
membranoproliferative glomerulonephritis lupus nephritis
76
what disease has mesangial deposists
IgA nephropathy
77
what happens to the kidney in end stage renal disease
- sclerotic glomeruli - interstitial fibrosis and inflammation - hyalinized sclerotic vessels - atrophy of tubules
78
what is a potential cause chronic glomerulonephritis
dialysis change
79
who and when does Henoch-Scholein purpura show up
- children 3-8 yrs | - follows an upper respiratory infection
80
what is pathogenesis for Henoch-Schoenlein purpura
IgA deposited in glomerular mesangium
81
What is the clinical feature for Henoch-Schoenlein Purpura
skin: purpuric lesions on arms, legs, buttocks
82
what happens to the proteins in diabetic nephropathy
- nonenzymatic glycosylation of proteins
83
Where does amyloids deposit in amyloidosis
mesangium and glomerular capillary walls