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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

most common pathogen in nephrotic syndrome

A

Strep pneumonia and E coli

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6
Q

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

A

isolated proteinuria

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7
Q

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

A

orthostatic proteinuria

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8
Q

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

A

protein during recumbency <50 mg

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9
Q

normal mesangiun contain about number of mesangial cells

A

2 to 4

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10
Q

effacemebt of podocyte foot processes

A

minimal change disease

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11
Q

Dose of prednisone for adult patients witg mcd and duration

A

1 mg/kg not to exceed 80 mg per day, 15 weeks

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12
Q

predilection for sclerosis in the perihilar regions with hyalinosis, obesity

A

FSGS

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13
Q

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

A

Glomerular tip variant

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14
Q

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

A

Collapsing variant

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15
Q

Most aggressive type of FSGS

A

collapsing variant

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16
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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17
Q

Membranous nephropathy caused by antibodies specific for what antibodies?

A

M type Phospholipase A2 receptor

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18
Q

pathologic sine qua non of membranous nephropathy

A

subepithelial immune complex deposits

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19
Q

mesangial dense deposits in MN

A

secondary MN

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20
Q

characteristic IF finding in MN

A

diffuse global granular capillary wall staining

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21
Q

most frequent and most intense IF staining in MN

A

IgG, IgG4

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22
Q

characteristic histologic abnormality by light microscopy of MN

A

diffuse global capillary wall thickening in absence of significant glomerular hypercellularity

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23
Q

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

A

Membranous Nephropathy

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24
Q

2 categories of MPGN

A

Immune complex mpgn and c3 glomerulopatht (alternative complement pathway)

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25
Type 2 MPGN
dense deposit disease
26
tran tracking/basement membrane reduplication
MPGN Type 1
27
lobular glomerulonephritis
Mpgn type 1
28
aggregates of immune complexes filling capillary lumens possibility of crypglobulinemia or lupus in mpgn
hyaline thrombi
29
characteristic pattern of if in MpGN
peripheral granular to bandlike staining for c3
30
mesangial interposition into an expanded subendothelial zone that contain electron dense immune complex deposits
MPGN type 1
31
Type of MPGN with persistent depression of C3 and presence of nephritic syndrome
Type 1 MPGN
32
Very rare primary immunodeficiency characterized by clinical triad of high serum ige, recurring staph skin abscesses and recurrent pneumonia with pneumatocoeles
buckley syndrome
33
resembles type 1 Mpgn but with irregularly thickened gbms with numerous intramembranous deposits
mpgn type 3
34
mesangiocapillary gn
mpgn type 1
35
discontinuous electron dense bands within the gbm
dense deposit disease
36
intense capillary wall linear to bandlike staining for C3
dense deposit disease
37
most common mechanism in uncontrolled activation of C3 convertase
presence of C3NeF
38
psgn associated with skin infections
epidemic psgn
39
psgn with pharyngitides
sporadic psgn
40
IF pattern, numerous large, closely apposed, granular deposits along capillary walls; nephrotic range proteinuria
Garland Pattern
41
IF pattern, psgn | More scattered granular staining less severe disease
Starry sky pattern
42
iF pattern, psgn, resolving phase with a mesangioproloferative light microscopic appearance
Mesangial pattern
43
hallmark ultrastructural feature of psgn
subepithelial humplike dense deposits
44
deposition predominantly of IgA in the mesangium with mesangial proliferation
IgA nephropathy
45
ubiquitous ultrastructural finding of IgA in em
Mesangial electron dense deposits
46
focal or diffuse mesangial hyper cellularity without more complex endo capillary hypercellularity
Iga nephropathy
47
four parameters predictive of clinical outcomes in IgA nephropathy
``` MEST mesangial hypercellularity endocapillary hypercellularity Segmental glomerulosclerosis tubular atrophy and interstial fibrosisb ```
48
in MEST, not significantly predictive of the rate of decline of kidney function or survival from eskd
endo capillary hypercellularity
49
size fibrils in fibrillary gn
20 nm
50
size of microtubular structures jn immunotactoid gn
30-40 nm
51
associated with lymphoproliferative disease and monoclonal gammopathy
fibrillary/immunotactoid gn
52
most common cause of rpgn in children
immune complex crescentic gn
53
least frequent cause of crescentic gn
Anti-GBM
54
Linear GBM IF staining + lung hemorrhage
good pasture syndrome
55
Anca disease + vasculitis with no granuloma or asthma
microscopic polyangitis
56
Vasculitis with granuloma no asthma
granulomatosis with polyangitis
57
Vasculitis with granulomas and asthma
Eosinophilic grabulomatosis with polyangitis
58
hallmark ultrastructural finding in immune complex mediated gn
immune complex type electron dense deposit
59
proliferative crescentic gn + pulmonary hemorrhage + anti gbm antibodies
good pasture syndrome
60
anca in anti-gbm
mpo-anca
61
most commin extrarenal findings in anti-gbm
pulmo ary
62
most common biopsy in antigbm
diffuse crescentic gn with more than 50% of glomeruli with exuberant circumferential crescents
63
Intense and diffuse linear staining for igG
Anti-GBM
64
major prognostic marker for progression to eskd in anti-gbm
serum crea at time of tx
65
major pathogenic factor for pauci immune crescentic gn
anca igg
66
endothelial tubuloreticular inclusions seen in
HIV
67
most frequent genetic cause of steroid resistant nephrotic syndrome
NPHs2 podocin
68
strongest indicators of progressive disease in MN
persistence of moderate proteinuria
69
Dose of cyclophosphamide for mcd and duration
2 mk for 8-12 weeks
70
Dose of cyclosporine for mcd
5 mk
71
No of glomeruli for adequate diagnosis
glomerular - 5 tubulointerstitial 6-10 transplant: 7
72
Ponticelli protocol
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
73
when to use ponticelli protocol
Moderate (4 and 6g protein) or high risk (>8g protein)
74
deposits in the retina Along brusch membrane
Dense deposit disease
75
latent period in post pharyngitic cases in psgn
14 to 21 days
76
Synpharingitic episode latency
less than 1 week
77
latency period of psgn after skin infection
14 to 21 days
78
Tx of pauci immune crescentic gn
steroids + cyclophosphamide | mppt 7mkd x 3 days; pred 1 mkd 1st month, dc 4th-5th; cyclophosphamide 0.5 g/m2
79
most impt determinant of patient survival
pulmonary hemorrhage
80
most impt predictor of outcome with respect to eskd in pauci immune
crea at time of initiation of tx
81
Earliest lesions are intracapillary thrombosis with deposition of eosinophilic fibrinoid material with endothelial cell swelling
Granulomatosis with polyangitis
82
Medication in gpa as induction only if disease is milld and controllable
methotrexate
83
TNF a blocker in gpa with 80% remission rate but high rate of infections
Infliximab
84
anti CD52 monoclonal antibody for gpa
alemtuzumab
85
vasculitis induced by ptu
Mpa
86
reduction to less than 3.5 g/day protein and 50% decrease from peak proteinuria
Partial remission
87
cytotoxic agent acts largely through alkylation of purine bases
cyclophosphamide
88
inhibitor of inosine monophosphate dehydrogenase
Azathioprine
89
reversible inhibitor of inosine monophosphate dehydrogenase
mycophenolate acid, mycophenolate mofetil
90
adverse effect of craniofacial malformations
MMF
91
anti C5 monoclonal antibody
Eculizumab

Nephrology (28 decks)

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