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Brenner > Primary Gn > Flashcards

Primary Gn Flashcards

1
Q

What is the autoimmune disease characterized by circulating IgG autoantibodies against two major podocyte antigens?

A

PRIMARY MEMBRANOUS NEPHROPATHY (PMN)

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2
Q

What are the 2 major podocyte antigens in Primary MN?

A
  1. PLA2R1 (70%)
  2. THSDA (5-10%)
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3
Q

What are the 2 new classification for MPGN

A
  1. IMMUNE COMPLEX-MEDIATED
  2. COMPLEMENT- MEDIATED OR C3 GLOMERULOPATHY
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4
Q

New classification of MPGN is based on:

A

IF

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5
Q

What type of MPGN, seen in IF where both immunoglobulin and complement are deposited

A

Immune complex-mediated MPGN

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6
Q

What type of MPGN with predominant C3 staining by IF?

A

Complement-mediated MPGN or C3 glomerulopathy

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7
Q

An autoimmune disease which results from autoantibodies targeting DNAJB9 (a putative autoantigen)

A

FIBRILLARY GN

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8
Q

Patients with immunotactoid glomerulonephritis should be evaluated for the underlying 3 disorders:

A
  1. Monoclonal gammopathy
  2. Malignancy
  3. Lymphoproliferative disorders
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9
Q

Autoantibodies in sporadic anti-GBM disease are directed against NC1 domain of what chain of type IV collagen?

A

ALPHA-3 CHAIN

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10
Q

What antibody in ANCA-associated vasculitis, which when present lung involvement, or upper respiratory tract involvement are more likely to relapse?

A

ANTI-PROTEINASE-3 (PR3) ANTIBODIES

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11
Q

Anti-GBM alloantibodies cause posttransplantation nephritis in X-linked Alport syndrome are directed against the NC1 domain of what chain of the type IV collagen?

A

ALPHA-5 CHAIN

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12
Q

IgA vasculitis was previously called as?

A

HENOCH-SCHONLEIN PURPURA

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13
Q

GPA (Granulomatosis with polyangiitis) was previously called?

A

WEGENER’S GRANULOMATOSIS

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14
Q

EGPA (Eosinophilic granulomatosis with polyangiitis) was previously called as?

A

CHURG-STRAUSS SYNDROME

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15
Q

What are the 4 glomerular disease with Nephrotic Features?

A
  1. MCD
  2. MN
  3. FSGS
  4. FIBRILLARY GN
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16
Q

What glomerular disease manifest purely with Nephrotic features?

A

MCD

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17
Q

Loss of charge selectivity is the cause of proteinuria in what type of GN?

A

MCD

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18
Q

Loss of size selectivity is the cause of proteinuria in what type of GN

A

MN

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19
Q

What is the strong evidence for glomerular bleeding?

A

ACANTHOCYTURIA

20
Q

What are the 4 differential diagnosis of glomerular hematuria without proteinuria, renal insufficiency, or red blood cell casts?

A
  1. IgA NEPHROPATHY
  2. TBMN
  3. HEREDITARY NEPHRITIS
  4. NORMAL GLOMERULI
21
Q

What is the protein excretion rate in Nephrotic syndrome?

A

> 3.5g/day

22
Q

Refers to the presence of lipid in the tubular epithelial cells and urine

A

“LIPOID NEPHROSIS”

23
Q

MCD was previously called as?

A

“LIPOID NEPHROSIS”

24
Q

What the the 6 factors that are increased in Nephrotic syndrome?

A

2, 5, 7, 8, 10, 13

25
Q

What are the 3 factors that are decreased in Nephrotic syndrome?

26
Q

The most consistent tubular lesion of increased protein & lipid resorption droplets in TECs is seen in what type of GN?

27
Q

What is the most frequent positive IF finding in MCD?

A

Low-level mesangial staining for IgM & C3

28
Q

What is the pathologic sine qua non of MCD in EM?

A

EFFACEMENT OF FOOT PROCESSES

29
Q

What are the LM, IF, EM findings in MCD?

A

LM: no glomerular lesions
*focal segmental mesangial prominence (not >3-4cells)
IF: no staining
*low level staining for IgM,C3
EM: effacement of foot processes

30
Q

In MCD, there is abnormal regulation of this cell subset:

A

T-CELL SUBSET

31
Q

What NSAID has been associated with MCD?

A

FENOPROFEN

32
Q

What type of lymphoma is associated with MCD?

A

CLASSIC HODGKIN LYMPHOMA (NODULAR SCLEROSIS TYPE)

33
Q

What is dose of prednisone for the treatment of MCD?

A

1mg/kg
Not to exceed 80mg/day

34
Q

What is considered a response to therapy in MCD?

A

No proteinuria by dipstick for at least 3 days

35
Q

In adult patients with MCD, a response to corticosteroid treatment may take up to how many weeks?

36
Q

In MCD, how long should treatment with corticosteroids continued after complete remission of proteinuria?

37
Q

What is the dose of oral cyclophosphamide in the treatment of MCD?

A

2mg/kg
8-12 weeks

38
Q

What is an alternative to cyclophosphamide therapy for relapsing or steroid-dependent MCD?

A

CYCLOSPORINE

39
Q

What is the duration of treatment with cyclosporine for relapsing/steroid-dependent MCD?

A

18-24 months

40
Q

What are the 2 types of CNI?

A
  1. Tacrolimus
  2. Cyclosporine
41
Q

B lymphocyte-depleting agent?

42
Q

What is the usual treatment regimen for MCD?

A
  1. Prednisone (induction of remission)
    Response: 15 weeks
    Continued after remission: 6 more weeks
  2. Cyclophosphamide (maintenance)
    8-12 weeks
43
Q

What is a differential diagnosis in steroid-resistant MCD?

44
Q

What are the 2 treatment options for steroid-resistant MCD?

A
  1. CYCLOSPORINE
  2. RITUXIMAB
45
Q

What are the 10 glomerular disease that cause Nephrotic syndrome & GN?

A
  1. MCD
  2. FSGS
  3. C1q NEPHROPATHY
  4. MN
  5. MPGN
  6. C3 GLOMERULOPATHY
  7. ACUTE PSGN
  8. IgAN
  9. FIBRILLARY GN
  10. IMMUNOTACTOID GN

Brenner (4 decks)

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