Glomerulonephritis 1 Flashcards

1
Q

4 main clinical patterns you will see

A

nephrotic, nephritic, or Acute renal failure or chronic renal failure

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2
Q

nephrotic syndrome has…

A

edema, swollen, proteinuria over 3.5g , high cholesterol

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3
Q

nephritic syndrome

A

RBC in urine, HTN possible, may be edematous, may have small amount of PRO

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4
Q

acute renal failure occurs in ___

A

days or weeks

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5
Q

nephrotic lab findings : 3

A

pro over 3.5 g in urine
liduria
fatty casts
generally few cells are found in urine

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6
Q

what pattern cast is associated with lipiduria

A

Maltese cross

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7
Q

nephritic pattern lab findings (3) and 2 main sx

A

RBC
RBC casts
Proteinuria

with HTN and edema

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8
Q

what is a cast?

A

clump of PRO material seen on microscopy that has certain cell types attached to it

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9
Q

RBC cast is pathonomonic for

A

nephritic syndrome - know you are dealing with a glomerulonephritis

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10
Q

3 main ways GN can occur

A
  1. immune complex deposits in situ
  2. deposits of antiglomerular basement membrane antibody
  3. deposit of immunoglobulin in glomerulus
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11
Q

antinuclear antibody

A

indicates lupus

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12
Q

ANCA:

A

found with vasculitis, antineutrophil cytoplasmic antibody

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13
Q

Anti GBM antibody

A

Good pasteurs dz

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14
Q

Antistreptolysin O titer

A

seen in strep

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15
Q

serum and PRO electrophoresis

A

multiple myelonma

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16
Q

if you see a kid with nephrotic syndrome think…

A

minimal change disease.. responsible for 70-90% pediatric nephrotic cases

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17
Q

are NSAIDS associated with minimal change?

A

yes can increase risk

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18
Q

what is main microscopic finding of minimal change?

A

effacement of foot process that supports podocytes, weakening of slit pore membranes

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19
Q

what is first line treatment for minimal change?

A

prednisone given daily or on alternate days for 3 mo

encourage: low sodium diet, diuretics, ace inhbitors

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20
Q

T/F immune deposits are found with minimal change?

A

false

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21
Q

4 things that associated with minimal change

A

durgs: NSAID, lithium bisphosphontaes
Neoplasm: Hodgkins lymphoma
Infections:
Allergies: bee stings

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22
Q

what does minimal change put patient at risk for?

A

thromboembolism, infections specifically pneumococcal

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23
Q

what is pathogenesis of minimal change

A

related to a T cell disorder due to associations..but overall unknown

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24
Q

about 10% of minimal change pts will not respond to treatment.. what does that mean?

A

most likely have FSGS

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25
Q

FSGS

A

focal segmental glomerulosclerosis

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26
Q

FSGS definition

A

non specific trapping of immunoglobulin C3 and IgM not thought to be pathogenic

Lots of fibrosis/scar tissue

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27
Q

what is circulating factor associated with FSGS

A

soluble urokinase receptor SuPAR

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28
Q

what are 4 conditions sometimes seen with FSGS

A

nephrotic range proteinuria
microscopic hematuria - with varying PRO levels
HTN
renal insufficiency

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29
Q

what is APOL1 associated with?

A

increased FSGS susceptibility of blacks to FSGS

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30
Q

what chromosome is APOL1 located on?

A

chromosome 22

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31
Q

what does FSGS prognosis depend on? 3 things

A

degree of proteinuria, severity of renal dysfunction and histologic findings

in addition to response to therapy

32
Q

FSGS

A

nonimmunosuppressive therapy: low sodium, ACEI and diuretics PLUS
glucocorticoids

33
Q

membranous glomerulonephritis

A

diffuse thickening of the glomerular basement membrane with essentially normal cellularity

34
Q

where do FSGS lesions usually occur

A

at corticomedullary junction.. so superficial biopsy can miss scar tissue if don’t go deep enough

35
Q

what would show on a silver stain with membranous glomerulonephritis

A

a spike due to new basement membrane growing between electron dense deposits

36
Q

membranous glomerulonephritis is associated with…

A

hep B, autoimmune SLE, malignancies, drugs Gold, Penicillamine, and NSAIDS

37
Q

phospholipase A2 receptor

A

a M type PLA2R transmembrane receptor that is highly expressed in glomerular podocytes, has been identified as major antigen in human idiopathic MN

this is associated with membranous gn

38
Q

what are 4 possible pt scenarios that would indicate immunosuppressive treatment for membranous glomerulonephritis?

A

older age at onset 50+
male
nephrotic range of protein (esp if above 8 or 10 g /day)
increased serum creatinine at presentation

39
Q

what does membranoproliferative GN look like?

A

lobular glomerular tuft with focal areas of increased cellularity
mesangial expansion
narrowing of capillary lumens
diffuse thickening of glomerular capillary walls

40
Q

how many membranoproliferative GN types are there

A

3

41
Q

what causes MPGN?

A

complement dysregulation that leads to persistant activation
or endothelial injury

42
Q

what can trigger MPGN immune dysregulation?

A

infections - hep B and hep C virus
SLE, Sjogren syndrome, sclerosis
monoclonal gammopathy

43
Q

IgA nephropathy

A

large globular mesangial IgA deposits

44
Q

what is most common cause of primary glomerulonephritis in developed countries?

A

IgA

45
Q

what race has higher reports of IgA nephropathy

A

Asians
Caucasians
rare in blacks

46
Q

what is gender ratio Iga?

A

2:1 male to female

47
Q

about 50% of ppl with Iga will have just had what..

A

upper respiratory infection and visible hematuria

48
Q

when would renal biopsy be indicated with IgA?

A

persistent protein excretion above 1000 mg/day which may increase over time.. or elevated serum creatinine concentration

49
Q

what are clinical predictors of IgA progression (4)

A

look at histology
proteinuria over 1 g
hypertension
elevated creatinine

50
Q

Iga tx

A

control BP: ACE or ARB

51
Q

__ is one of the most common forms of systemic vasculitis in kids

A

Henoch Schonlein purpura aka IGA vasculitis

52
Q

signs of IGA vasculitis

A
  • palpable purpura without coagulopathy or thrombocytopenia
  • arthritis/arthralgia
  • abdominal pain
  • renal disease
53
Q

HSP occurs between ages of __ and ___

A

3 and 15

54
Q

there is ___ predominance in Henoch Schonlein Purpura

A

male

55
Q

what are characteristics of Henoch Schonlein Purpura

A

leukocytoclastic vasculitis with IgA immune complexes within affected organs

56
Q

HSP treament

A

support plus acetominoophen or NSAID for pain

57
Q

diffuse proliferative GN

A

post infectious glomerulonephritis with granular deposition of complement in the glomerular tuft

58
Q

T/F post infectious glomerulonephritis starts as nephritic

A

true: red to brown urine, proteinuria, edema, HTN, AKI

59
Q

___most common cause of acute nephritis in kids

A

PSGN

60
Q

what to 5 order for post infectious glomerulonephritis

A

ASO, AHase, ASKase, anti-NAD, Anti-DNAse B antibodies

61
Q

PSGN tx

A

supportive

62
Q

Good Pastures dz

A

hypercellular circumferential CRESCENT that compresses glomerular tuft closing the capillary lumens

63
Q

how does goodpastures show up on immunoflorescence

A

linear deposition of IGg in anti GBM antibody disease

64
Q

prognosis of acute GN from anti GBM antibody dz is…

A

poor

65
Q

treatment goodpastures

A

Plasmapheresis with prednisone and cyclophsophamide

66
Q

how does goodpasteurs present

A

AKI, proteinuria, nephritic sediment with dysmorphic red cells, white cells, red cell, and granular casts

and see alveolar hemorrhage ..affects about 30-40% of patients

67
Q

vasculitis definition

A

fresh segmental necrotizing lesions with bright red fibrin deposition

68
Q

Wegners granulomatosis name changed to

A

granulomatosis with polyangitis which can be abbreviated as GPA

69
Q

what are some signs that separate GPA from other vasculitis

A

nasal or oral inflammation, bloody discharge
abnormal CXR with nodules, infiltrates, cavities
abnormal urinary sediment
granulomatous inflammation on biopsy of an artery or perivascular area

70
Q

focal vasculitis

A

at least 50% of glomeruli are normal

71
Q

crescentic

A

50% are crescents that are cellular or fibrotic

72
Q

sclerotic

A

at least 50% of glomeruli are globally sclerotic

73
Q

mixed

A

less than 50% of glomeruli are normal, less than 50% are crescentic and less than 50% are globally scleortic

74
Q

if you think its vasculitis order…

A

ANCA

GPA is associated with PR3-ANCA

75
Q

when you can confirm diagnosis of MPA or GPA with….

A

biopsy

76
Q

GPA tx

A

oral or pulse cyclophosphamide

reduce steroids after 2 mo