Nephrotic Syndrome (20-3) Flashcards

1
Q

Nephrotic Syndrome involves derangement of _____

A

Derangement of glomerular capillary wall

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2
Q

What are the 4 signs of Nephrotic Syndrome?

A
  1. Proteinuria > 3.5 g/day
  2. Edema
  3. Hypoalbuminemia
  4. Hyperlipidemia and lipiduria
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3
Q

What are the 4 signs of Nephrotic Syndrome?

A
  1. Proteinuria > 3.5g/day
  2. Edema
  3. Hypoalbuminemia
  4. Hyperlipidemia and lipiduria
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4
Q

With Nephrotic Syndrome, the albumin level is usually below?

A

3

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5
Q

What 2 things are those with Nephrotic Syndrome at an increased risk for and why?

A

Infection – losing Ig’s

Thrombosis – losing Antithrombin 3

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6
Q

What systemic disease usually causes nephrotic syndrome?

A

Diabetes

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7
Q

What are 4 causes of Nephrotic Syndrome?

A
  • Membranous Nephropathy
  • Minimal Change Disease
  • Focal Segmental Glomerulosclerosis
  • Membranoproliferative Glomerulonephritis
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8
Q

What are 4 causes of Nephrotic Syndrome?

A
  • Membranous Nephropathy
  • Minimal Change Disease
  • Focal Segmental Glomerulosclerosis
  • Membranoproliferative Glomerulonephritis
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9
Q

With Membranous Nephropathy, what thickens and what gets deposited?

A

Glomerular capillary wall thickens

– IgG4 gets deposited

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10
Q

With Membranous Nephropathy, there is antibodies to?

A

Renal autoantigen

= M type phospholipase A2 receptor (PLA2R)

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11
Q

With Membranous Nephropathy, there are antibodies to?

A

Renal autoantigen

= M type phospholipase A2 receptor (PLA2R)

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12
Q

With Membranous Nephropathy, what is the job of C5b - C9 of the complement?

A

(+) mesangial and epithelial cells

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13
Q

With Membranous Nephropathy, once the C5b-C9 activates mesangial and epithelial cells, what does that cause to be activated?

A

(+) proteases

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14
Q

With Membranous Nephropathy, what do (+) proteases do?

A

Cause capillary injury

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15
Q

Thickening of glomerular capillary wall with IgG4 deposits suggests?

A

Membranous Nephropathy

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16
Q

What things can cause secondary Membranous Nephropathy?

A

Drugs, autoimmune, SLE, tumors, infections

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17
Q

Minimal Change disease affects who primarily?

A

CHILDREN

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18
Q

What is the most common cause of Nephrotic Syndrome in children?

A

Minimal Change Disease

19
Q

What 2 causes of Nephrotic Syndrome cause effacement of foot processes of podocytes to cause glomerular injury?

A

Minimal Change Disease

Focal Segmental Glomerulosclerosis

20
Q

With effacement of foot processes of the podocytes, what does that cause to happen?

A

Loss of slit diaphragms

21
Q

What things is Minimal Change Disease associated with?

A
  • Respiratory infections and immunizations
  • Atopic disorders, HLA haplotypes
  • Hodgkin Lymphoma
22
Q

What is unique about the treatment of Minimal Change Disease?

A

DRAMATIC response to corticosteroid therapy

23
Q

If a child has a dramatic response to corticosteroid therapy, what is the likely disease causing nephrotic syndrome?

A

Minimal Change Disease

24
Q

What is the most common cause of Nephrotic Syndrome?

A

Focal Segmental Glomerulosclerosis (FSGS)

25
Q

What causes the sclerosis of some segments of some glomeruli with FSGS?

A

TGF-beta

26
Q

What is FSGS associated with?

A
  • HIV
  • Heroin use
  • Sickle cell
  • Loss of renal tissue
27
Q

The inherited forms of FSGS are associated with mutations in what 4 genes?

A

NPHS1
NPHS2
Alpha actin 4
TRPC6

28
Q

The inherited forms of FSGS are associated with mutations in what 4 genes?

A

NPHS1
NPHS2
Alpha actin 4
TRPC6

29
Q

In the sclerotic areas of FSGS, what will the immunofluorescence show?

A

IgM and C3

30
Q

What 3 things classify HIV associated Nephropathy?

A
  1. Collapsing variant of FSGS
  2. Focal cystic dilation of tubule segments
  3. Tubuloreticular inclusions
31
Q

What 3 things classify HIV Associated Nephropathy?

A
  1. Collapsing variant of FSGS
  2. Focal cystic dilation of tubule segments
  3. Tubuloreticular inclusions
32
Q

What are the 2 types of Membranoproliferative Glomerulonephritis’s?

A

Type 1 - IgG immune complex deposition

Type 2 - Dense deposit disease

33
Q

What are the 2 types of Membranoproliferative Glomerulonephritis’s?

A

Type 1 - IgG immune complex deposition

Type 2 - Dense deposit disease

34
Q

The symptoms of IgG immune complex deposition (Type 1) are _____

A

Unremitting

35
Q

With Type 1 Membranoproliferative Glomerulonephritis, the glomerluli are large and hypercellular. How does the GBM look and why?

A

Double contour appearance

– due to synthesis of another GBM

36
Q

What is a hallmark of Type 1 Membranoproliferative Glomerulonephritis (IgG immune complex deposition)?

A

Double contour GBM with cells between the 2 GBM layers

37
Q

Type 2 Membranoproliferative Glomerulonephritis (dense deposit disease) involves what on the GBM?

A

C3 of the complement

38
Q

What complement pathway is persistently active with Dense Deposit Disease?

A

Alternative pathway

39
Q

Dense Deposit Disease has an autoantibody to?

A

C3NeF

40
Q

What does C3NeF cause with Dense Deposit Disease?

A

Persistent alternative complement activation

41
Q

What things will be decreased in the serum with Dense Deposit Disease (type 2 membranoproliferative glomerulonephritis)?

A

Decreased C3, Factor B, Properdin

42
Q

What things will be decreased in the serum with Dense Deposit Disease?

A

Decreased C3, Factor B, Properdin

43
Q

Where is C3 put with Dense Deposit Disease?

A

GBM

44
Q

All of the diseases in this deck can cause?

A

Nephrotic Syndrome