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CVPR: Renal (Exam 4) > Nephrotic Syndrome Pathology > Flashcards

Nephrotic Syndrome Pathology Flashcards

(54 cards)

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1
Q

If your only pathological finding is foot process fusion, what is the dx?

A

minimal change disease

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2
Q

The primary protein in the slit diaphragm has recently been identified and is termed _____.

A

nephrin

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2
Q

Why are pts with nephrotic syndrome at an increased risk of infection?

A

loss of IgG and complement (esp. factor B) into the urine

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2
Q

What is the prognosis for minimal change disease?

A

good

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3
Q

What is the major abnormality that defines nephritic syndrome?

A

active inflammation within the glomerulus that leads to damage and subsequent loss of filtration and a reduced GFR

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3
Q

Why do children with nephrotic syndrome have poor growth and osteomalacia?

A

loss of vitamin D and its binding protein in the urine

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4
Q

What causes FSGS?

A

a circulating factor that affects the podocyte (maybe suPAR)

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5
Q

Name 3 properties of mesangial cells.

A
  1. secretes a basement membrane-like matrix which acts as a structural support to the glomerulus
  2. has smooth muscle-like properties and can contract, thus affecting capillary surface area and filtration
  3. has some macrophage-like properties including the ability to secrete cytokines, growth factors, proteases and oxidants
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5
Q

What does a “Maltese cross” under polarized light indicate?

A

lipiduria, indicative of nephrotic syndrome

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6
Q

What is the most common cause of nephrotic syndrome in adults?

A

diabetic nephropathy

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7
Q

______ is a type of nephrotic syndrome and the most common one in young adults and African Americans.

A

FSGS

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8
Q

What are the following associations common to?

  • HBV
  • gold and penicillamine drugs
  • lupus (type V)
  • cancer
A

Membranous Nephropathy

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9
Q

What do these path findings indicate?

  • thickening of the GBM by light microscopy with dense, subepi deposits
  • Spikes and domes along the BM
  • IF positive for immunoglobulin and C3 and C5b-9
A

Membranous nephropathy

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9
Q

What is Type I MPGN?

A

IgG and C3 immune complex deposition in the capillary walls and mesangium

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10
Q

What is the tx for FSGS?

A

long term steroids and cyclosporine

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10
Q

How does Membranous nephropathy occur?

A

autoimmune mediated by an antibody directed against an antigen (PLA2) on the podocyte

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11
Q

A mutation in the ______ gene is responsible for the congenital nephrotic syndrome of the Finish type.

A

nephrin

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12
Q

Which nephrotic syndrome often presents like a nephritic syndrome?

A

MPGN

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13
Q

_______ is an immune mediated glomerular disease associated with immune complex deposits in the subepithelial space

A

Membranous nephropathy

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15
Q

The most important barrier to protein is the ______.

A

filtration slit diaphragm

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17
Q

Why are pts with nephrotic syndrome at an increased risk of thrombosis?

A
  • increase in synthesis of coagulation factors (fibrinogen, factors V, VIII, IX, X) by the liver
  • urinary loss of anti-thrombin III
  • increased platelet aggregability
  • impairment of the fibrinolytic system
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18
Q

These underlying conditions are all common to?

  • prior minimal change disease
  • heroin
  • HIV infection
  • sickle cell disease
  • parvo virus infection
  • obesity
19
Q

How does minimal change disease pt present?

Study These Flashcards
A
  • edema
  • hx of allergic disease
  • severe hypoalbuminemia
  • possible Hodgkin’s disease
20
Q

What are some known associations to MPGN development?

21
What is the prognosis for FSGS?
poor
22
How is vitamin D measured in the blood?
by measuring ionized calcium and 25-0H vitamin D levels
23
IgG and C3 immune complex deposition in the capillary walls and mesangium would indicate?
type I MPGN
24
A mutation in the nephrin gene is responsible for \_\_\_\_\_\_\_\_.
congenital nephrotic syndrome of the Finish type
25
What is the prognosis for MPGN?
poor
27
What is the tx for minimal change disease?
steroids (ie prednisone- should have rapid response. if not = prednisone and cyclophosphamide)
28
C3 deposition only with the BM replaced by dense deposits would indicate?
Type II MPGN
29
What do these path. findings indicate? * Light microscopy shows segmental scarring (sclerosis) in some (focal) glomeruli * Immunofluourescence shows some nonspecific staining of IgM and C3 * EM shows diffuse foot process fusion consistent with a generalized capillary wall defect
FSGS
30
What is the tx for Membranous Nephropathy?
* Steroids * cytotoxic drugs (chlorambucil, cyclophosphamide) * ACE-I
31
Nephritic syndrome presents with?
* microhematuria and occasionally red cell casts * non-nephrotic proteinuria * decreased GFR * HTN * edema
32
Name 4 systemic diseases associated with nephrotic syndrome.
1. Diabetes 2. Amyloid 3. Light Chain Deposition Disease 4. Lupus (SLE) Membranous Type V
33
Name 3 components of the glomerular basement membrane.
1. type IV collagen 2. laminin and entactin (glycoproteins important in endothelial and epithelial cell attachment) 3. heparan sulfate (proteoglycan important in providing a negative charge to the GBM)
35
\_\_\_\_\_\_ is the most common cause of idiopathic nephrotic syndrome in children.
Minimal change disease
37
The Nephrotic syndrome results as a consequence of marked albuminuria (\>3.5g/d), which leads to ____ and \_\_\_\_.
hypoalbuminemia and edema
37
What can minimal change disease progress into?
FSGS
38
What is thought to be the cause of minimal change disease?
a circulating T cell product that damages the podocyte and permeability layer
39
Nephrotic range proteinuria almost always means that there is a disruption of the \_\_\_\_\_\_.
slit diaphragm
40
\_\_\_\_\_\_\_ is the most common cause of idiopathic nephrotic syndrome in older adults, esp males.
Membranous nephropathy
40
What is Type II MPGN?
C3 deposition only; BM replaced by dense deposits
42
Name 4 associations with Membranous Nephropathy.
1. HBV 2. gold and penicillamine drugs 3. lupus (type V) 4. cancer
43
Why does hyperlipidemia occur in nephrotic syndrome?
* increased lipoprotein synthesis (VLDL, LDL) in the liver * decreased peripheral removal of VLDL
44
What is the major abnormality that defines nephrotic syndrome?
an excessive leak of protein through the glomerular capillary wall into the urinary space
46
These characteristics define what disorder?  * Proteinuria (\>3.5 gm/day or \>40 mg/hr/M2 in children) * Hypoalbuminemia ( * Edema * Hyperlipidemia * Lipiduria
nephrotic syndrome
47
What are some s/s of acute renal vein thrombosis?
* flank pain * an enlarged kidney by ultrasound * hematuria
48
Minimal change disease is the most common cause of idiopathic nephrotic syndrome in \_\_\_\_\_.
children
49
Nephritic or nephrotic: Minimal change disease
nephrotic
50
What is the prognosis for membranous nephropathy?
poor
51
What are some underlying associations to FSGS development?
* prior minimal change disease * heroin * HIV infection * sickle cell disease * parvo virus infection obesity
53
What is the tx for MPGN?
* alternate day steroids * anti-platelets * treatment of HCV if present
54
Membranous nephropathy is the most common cause of idiopathic nephrotic syndrome in \_\_\_\_\_\_.
older adults, esp males

CVPR: Renal (Exam 4) (29 decks)

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