Primary Glomerulonephritis Flashcards

1
Q

3 layers of glomerular capillary wall

A

Endothelial cells, GBM, Epithelial cells

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2
Q

the last barrier resistance encounteted by water and filtered solute

A

epithelial filtration slit

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3
Q

dominant infiltrating cells in early and late chronic renal injury

A

macrophages

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4
Q

second most abundantly excreted protein

A

Immunoglobulin

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5
Q

mild transient proteinuria of less than 1g per day in stressful conditions

A

isolated proteinuria

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6
Q

absence of proteinuria in recumbent, less than 1-2 g protein during upright posture

A

orthostatic proteinuria

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7
Q

diagnosis of orthostatic proteinuria in split collection 16h vs 8h recumbent

A

protein during recumbency <50 mg

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8
Q

normal mesangiun contain about number of mesangial cells

A

2 to 4

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9
Q

Dose of prednisone for adult patients witg mcd and duration

A

1 mg/kg not to exceed 80 mg per day, 16weeks

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10
Q

predilection for sclerosis in the perihilar regions with hyalinosis, obesity

A

FSGS

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11
Q

segmental consolidation confined to the segment adjacent to the origin of the proximal tubule

A

Glomerular tip variant

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12
Q

Segmental collapse of capillaries with hypertrophy and hyperplasia of overlying podocytes and accumulation of protein resorption droplets

A

Collapsing variant

Most aggressive type of FSGS

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13
Q

Presence of mesangial immune complex deposuts with staining for C1q in a patient with no clinical and lab evidence for sle

A

C1q Nephropathy

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14
Q

Membranous nephropathy caused by antibodies specific for what antibodies?

A

M type Phospholipase A2 receptor

subepithelial immune complex deposits

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15
Q

mesangial dense deposits in MN

A

secondary MN

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16
Q

characteristic IF finding in MN

A

diffuse global granular capillary wall staining

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17
Q

most frequent and most intense IF staining in MN

A

IgG, IgG4

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18
Q

characteristic histologic abnormality by light microscopy of MN

A

diffuse global capillary wall thickening in absence of significant glomerular hypercellularity

Caused by immune complex localization in the subepithelial zone of glomerular capillaries

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19
Q

2 categories of MPGN

A

Immune complex mpgn and c3 glomerulopatht (alternative complement pathway)

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20
Q

Type 2 MPGN

A

dense deposit disease

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21
Q

tran tracking/basement membrane reduplication

lobular glomerulonephritis

A

MPGN Type 1

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22
Q

aggregates of immune complexes filling capillary lumens possibility of crypglobulinemia or lupus in mpgn

A

hyaline thrombi

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23
Q

characteristic pattern of if in MpGN

A

peripheral granular to bandlike staining for c3

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24
Q

mesangial interposition into an expanded subendothelial zone that contain electron dense immune complex deposits

A

MPGN type 1

mesangiocapillary gn

Type of MPGN with persistent depression of C3 and presence of nephritic syndrome Type 1 MPGN

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25
Q

resembles type 1 Mpgn but with irregularly thickened gbms with numerous intramembranous deposits

A

mpgn type 3

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26
Q

discontinuous electron dense bands within the gbm

intense capillary wall linear to bandlike staining for C3

A

dense deposit disease

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27
Q

most common mechanism in uncontrolled activation of C3 convertase

A

presence of C3NeF

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28
Q

psgn associated with skin infections

A

epidemic psgn

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29
Q

psgn with pharyngitides

A

sporadic psgn

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30
Q

IF pattern, numerous large, closely apposed, granular deposits along capillary walls; nephrotic range proteinuria

A

Garland Pattern

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31
Q

IF pattern, psgn
More scattered granular staining less severe disease

A

Starry sky pattern

32
Q

iF pattern, psgn, resolving phase with a mesangioproloferative light microscopic appearance

A

Mesangial pattern

33
Q

hallmark ultrastructural feature of psgn

A

subepithelial humplike dense deposits

34
Q

deposition predominantly of IgA in the mesangium with mesangial proliferation

focal or diffuse mesangial hyper cellularity without more complex endo capillary hypercellularity

A

IgA nephropathy

35
Q

four parameters predictive of clinical outcomes in IgA nephropathy

A

MEST
mesangial hypercellularity
endocapillary hypercellularity
Segmental glomerulosclerosis
tubular atrophy and interstial fibrosisb

36
Q

in MEST, not significantly predictive of the rate of decline of kidney function or survival from eskd

A

endo capillary hypercellularity

37
Q

size fibrils in fibrillary gn

A

20 nm

38
Q

size of microtubular structures jn immunotactoid gn

A

30-40 nm

39
Q

associated with lymphoproliferative disease and monoclonal gammopathy

A

fibrillary/immunotactoid gn

40
Q

most common cause of rpgn in children

A

immune complex crescentic gn

41
Q

least frequent cause of crescentic gn

A

Anti-GBM

42
Q

Linear GBM IF staining + lung hemorrhage

proliferative crescentic gn + pulmonary hemorrhage + anti gbm antibodies

A

good pasture syndrome mpo-anca

43
Q

Anca disease + vasculitis with no granuloma or asthma

A

microscopic polyangitis

44
Q

Vasculitis with granuloma no asthma

Earliest lesions are intracapillary thrombosis with deposition of eosinophilic fibrinoid material with endothelial cell swelling

A

granulomatosis with polyangitis

45
Q

Vasculitis with granulomas and asthma

A

Eosinophilic grabulomatosis with polyangitis

46
Q

hallmark ultrastructural finding in immune complex mediated gn

A

immune complex type electron dense deposit

47
Q

most common biopsy in antigbm

A

diffuse crescentic gn with more than 50% of glomeruli with exuberant circumferential crescents

48
Q

Intense and diffuse linear staining for igG

A

Anti-GBM

49
Q

major prognostic marker for progression to eskd in anti-gbm

A

serum crea at time of tx

50
Q

major pathogenic factor for pauci immune crescentic gn

A

anca igg

51
Q

endothelial tubuloreticular inclusions seen in

A

HIV

52
Q

most frequent genetic cause of steroid resistant nephrotic syndrome

A

NPHs2 podocin

53
Q

strongest indicators of progressive disease in MN

A

persistence of moderate proteinuria

54
Q

Dose of cyclophosphamide for mcd and duration

A

2 mk for 8-12 weeks

55
Q

Dose of cyclosporine for mcd

A

5 mk

56
Q

No of glomeruli for adequate diagnosis

A

glomerular - 5
tubulointerstitial 6-10
transplant: 7

57
Q

Ponticelli protocol

A

Methlypred 1g/day for the 1st 3 days of each month with daily oral methylpred 0.4 mkd, prednisone 0.5 mkd alternating monthly with chlorambucil 0.2 mkd

58
Q

when to use ponticelli protocol

A

Moderate (4 and 6g protein) or high risk (>8g protein)

59
Q

deposits in the retina Along brusch membrane

A

Dense deposit disease

60
Q

latent period in post pharyngitic cases in psgn

A

14 to 21 days

61
Q

Synpharingitic episode latency

A

less than 1 week

62
Q

latency period of psgn after skin infection

A

14 to 21 days

63
Q

Tx of pauci immune crescentic gn

A

steroids + cyclophosphamide

(mppt 7mkd x 3 days; pred 1 mkd 1st month, dc 4th-5th; cyclophosphamide 0.5 g/m2)

64
Q

most impt determinant of patient survival

A

pulmonary hemorrhage

65
Q

most impt predictor of outcome with respect to eskd in pauci immune

A

crea at time of initiation of tx

66
Q

Medication in gpa as induction only if disease is milld and controllable

A

methotrexate

67
Q

TNF a blocker in gpa with 80% remission rate but high rate of infections

A

Infliximab

68
Q

anti CD52 monoclonal antibody for gpa

A

alemtuzumab

69
Q

vasculitis induced by ptu

A

Mpa

70
Q

reduction to less than 3.5 g/day protein and 50% decrease from peak proteinuria

A

Partial remission

71
Q

cytotoxic agent acts largely through alkylation of purine bases

A

cyclophosphamide

72
Q

inhibitor of inosine monophosphate dehydrogenase

A

Azathioprine

73
Q

reversible inhibitor of inosine monophosphate dehydrogenase

A

mycophenolate acid, mycophenolate mofetil

74
Q

adverse effect of craniofacial malformations

A

MMF

75
Q

anti C5 monoclonal antibody

A

Eculizumab