Nephrotic Syndrome (20-3) Flashcards
Nephrotic Syndrome involves derangement of _____
Derangement of glomerular capillary wall
What are the 4 signs of Nephrotic Syndrome?
- Proteinuria > 3.5 g/day
- Edema
- Hypoalbuminemia
- Hyperlipidemia and lipiduria
What are the 4 signs of Nephrotic Syndrome?
- Proteinuria > 3.5g/day
- Edema
- Hypoalbuminemia
- Hyperlipidemia and lipiduria
With Nephrotic Syndrome, the albumin level is usually below?
3
What 2 things are those with Nephrotic Syndrome at an increased risk for and why?
Infection – losing Ig’s
Thrombosis – losing Antithrombin 3
What systemic disease usually causes nephrotic syndrome?
Diabetes
What are 4 causes of Nephrotic Syndrome?
- Membranous Nephropathy
- Minimal Change Disease
- Focal Segmental Glomerulosclerosis
- Membranoproliferative Glomerulonephritis
What are 4 causes of Nephrotic Syndrome?
- Membranous Nephropathy
- Minimal Change Disease
- Focal Segmental Glomerulosclerosis
- Membranoproliferative Glomerulonephritis
With Membranous Nephropathy, what thickens and what gets deposited?
Glomerular capillary wall thickens
– IgG4 gets deposited
With Membranous Nephropathy, there is antibodies to?
Renal autoantigen
= M type phospholipase A2 receptor (PLA2R)
With Membranous Nephropathy, there are antibodies to?
Renal autoantigen
= M type phospholipase A2 receptor (PLA2R)
With Membranous Nephropathy, what is the job of C5b - C9 of the complement?
(+) mesangial and epithelial cells
With Membranous Nephropathy, once the C5b-C9 activates mesangial and epithelial cells, what does that cause to be activated?
(+) proteases
With Membranous Nephropathy, what do (+) proteases do?
Cause capillary injury
Thickening of glomerular capillary wall with IgG4 deposits suggests?
Membranous Nephropathy
What things can cause secondary Membranous Nephropathy?
Drugs, autoimmune, SLE, tumors, infections
Minimal Change disease affects who primarily?
CHILDREN
What is the most common cause of Nephrotic Syndrome in children?
Minimal Change Disease
What 2 causes of Nephrotic Syndrome cause effacement of foot processes of podocytes to cause glomerular injury?
Minimal Change Disease
Focal Segmental Glomerulosclerosis
With effacement of foot processes of the podocytes, what does that cause to happen?
Loss of slit diaphragms
What things is Minimal Change Disease associated with?
- Respiratory infections and immunizations
- Atopic disorders, HLA haplotypes
- Hodgkin Lymphoma
What is unique about the treatment of Minimal Change Disease?
DRAMATIC response to corticosteroid therapy
If a child has a dramatic response to corticosteroid therapy, what is the likely disease causing nephrotic syndrome?
Minimal Change Disease
What is the most common cause of Nephrotic Syndrome?
Focal Segmental Glomerulosclerosis (FSGS)
What causes the sclerosis of some segments of some glomeruli with FSGS?
TGF-beta
What is FSGS associated with?
- HIV
- Heroin use
- Sickle cell
- Loss of renal tissue
The inherited forms of FSGS are associated with mutations in what 4 genes?
NPHS1
NPHS2
Alpha actin 4
TRPC6
The inherited forms of FSGS are associated with mutations in what 4 genes?
NPHS1
NPHS2
Alpha actin 4
TRPC6
In the sclerotic areas of FSGS, what will the immunofluorescence show?
IgM and C3
What 3 things classify HIV associated Nephropathy?
- Collapsing variant of FSGS
- Focal cystic dilation of tubule segments
- Tubuloreticular inclusions
What 3 things classify HIV Associated Nephropathy?
- Collapsing variant of FSGS
- Focal cystic dilation of tubule segments
- Tubuloreticular inclusions
What are the 2 types of Membranoproliferative Glomerulonephritis’s?
Type 1 - IgG immune complex deposition
Type 2 - Dense deposit disease
What are the 2 types of Membranoproliferative Glomerulonephritis’s?
Type 1 - IgG immune complex deposition
Type 2 - Dense deposit disease
The symptoms of IgG immune complex deposition (Type 1) are _____
Unremitting
With Type 1 Membranoproliferative Glomerulonephritis, the glomerluli are large and hypercellular. How does the GBM look and why?
Double contour appearance
– due to synthesis of another GBM
What is a hallmark of Type 1 Membranoproliferative Glomerulonephritis (IgG immune complex deposition)?
Double contour GBM with cells between the 2 GBM layers
Type 2 Membranoproliferative Glomerulonephritis (dense deposit disease) involves what on the GBM?
C3 of the complement
What complement pathway is persistently active with Dense Deposit Disease?
Alternative pathway
Dense Deposit Disease has an autoantibody to?
C3NeF
What does C3NeF cause with Dense Deposit Disease?
Persistent alternative complement activation
What things will be decreased in the serum with Dense Deposit Disease (type 2 membranoproliferative glomerulonephritis)?
Decreased C3, Factor B, Properdin
What things will be decreased in the serum with Dense Deposit Disease?
Decreased C3, Factor B, Properdin
Where is C3 put with Dense Deposit Disease?
GBM
All of the diseases in this deck can cause?
Nephrotic Syndrome
