Nephrotic Syndrome (20-3)

Question 1

Nephrotic Syndrome involves derangement of _____

Answer 1

Derangement of glomerular capillary wall

Question 2

What are the 4 signs of Nephrotic Syndrome?

Answer 2

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

Question 3

What are the 4 signs of Nephrotic Syndrome?

Answer 3

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

Question 4

With Nephrotic Syndrome, the albumin level is usually below?

Answer 4

3

Question 5

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

Answer 5

Infection – losing Ig’s

Thrombosis – losing Antithrombin 3

Question 6

What systemic disease usually causes nephrotic syndrome?

Answer 6

Diabetes

Question 7

What are 4 causes of Nephrotic Syndrome?

Answer 7

• Membranous Nephropathy
• Minimal Change Disease
• Focal Segmental Glomerulosclerosis
• Membranoproliferative Glomerulonephritis

Question 8

What are 4 causes of Nephrotic Syndrome?

Answer 8

• Membranous Nephropathy
• Minimal Change Disease
• Focal Segmental Glomerulosclerosis
• Membranoproliferative Glomerulonephritis

Question 9

With Membranous Nephropathy, what thickens and what gets deposited?

Answer 9

Glomerular capillary wall thickens

– IgG4 gets deposited

Question 10

With Membranous Nephropathy, there is antibodies to?

Answer 10

Renal autoantigen

= M type phospholipase A2 receptor (PLA2R)

Question 11

With Membranous Nephropathy, there are antibodies to?

Answer 11

Renal autoantigen

= M type phospholipase A2 receptor (PLA2R)

Question 12

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

Answer 12

(+) mesangial and epithelial cells

Question 13

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

Answer 13

(+) proteases

Question 14

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

Answer 14

Cause capillary injury

Question 15

Thickening of glomerular capillary wall with IgG4 deposits suggests?

Answer 15

Membranous Nephropathy

Question 16

What things can cause secondary Membranous Nephropathy?

Answer 16

Drugs, autoimmune, SLE, tumors, infections

Question 17

Minimal Change disease affects who primarily?

Answer 17

CHILDREN

Question 18

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

Answer 18

Minimal Change Disease

Question 19

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

Answer 19

Minimal Change Disease

Focal Segmental Glomerulosclerosis

Question 20

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

Answer 20

Loss of slit diaphragms

Question 21

What things is Minimal Change Disease associated with?

Answer 21

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

Question 22

What is unique about the treatment of Minimal Change Disease?

Answer 22

DRAMATIC response to corticosteroid therapy

Question 23

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

Answer 23

Minimal Change Disease

Question 24

What is the most common cause of Nephrotic Syndrome?

Answer 24

Focal Segmental Glomerulosclerosis (FSGS)

Question 25

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

Answer 25

TGF-beta

Question 26

What is FSGS associated with?

Answer 26

- HIV - Heroin use - Sickle cell - Loss of renal tissue

Question 27

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

Answer 27

NPHS1 NPHS2 Alpha actin 4 TRPC6

Question 28

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

Answer 28

NPHS1 NPHS2 Alpha actin 4 TRPC6

Question 29

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

Answer 29

IgM and C3

Question 30

What 3 things classify HIV associated Nephropathy?

Answer 30

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

Question 31

What 3 things classify HIV Associated Nephropathy?

Answer 31

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

Question 32

What are the 2 types of Membranoproliferative Glomerulonephritis's?

Answer 32

Type 1 - IgG immune complex deposition | Type 2 - Dense deposit disease

Question 33

What are the 2 types of Membranoproliferative Glomerulonephritis's?

Answer 33

Type 1 - IgG immune complex deposition | Type 2 - Dense deposit disease

Question 34

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

Answer 34

Unremitting

Question 35

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

Answer 35

Double contour appearance | -- due to synthesis of another GBM

Question 36

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

Answer 36

Double contour GBM with cells between the 2 GBM layers

Question 37

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

Answer 37

C3 of the complement

Question 38

What complement pathway is persistently active with Dense Deposit Disease?

Answer 38

Alternative pathway

Question 39

Dense Deposit Disease has an autoantibody to?

Answer 39

C3NeF

Question 40

What does C3NeF cause with Dense Deposit Disease?

Answer 40

Persistent alternative complement activation

Question 41

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

Answer 41

Decreased C3, Factor B, Properdin

Question 42

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

Answer 42

Decreased C3, Factor B, Properdin

Question 43

Where is C3 put with Dense Deposit Disease?

Answer 43

GBM

Question 44

All of the diseases in this deck can cause?

Answer 44

Nephrotic Syndrome