Nephrotic Syndrome Flashcards

1
Q

What is nephrotic syndrome?

A

Glomerular disorders characterized by proteinuria characterized by:

Hypoalbuminemia - pitting edema

Hypogammaglobulinemia - increased ris kof infection

Hypercoagulable state - due to loss of antithrombin III

Hyperlipidemia and hypercholesterolemia - may result in faty casts in urine

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2
Q

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

A

Minimal change disease

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3
Q

What is the etiology of minimal change disease?

A

Idiopathic usually

May be associatedwith Hodgkin Lymphoma

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4
Q

What is seen on H&E stain, EM, and IF of MCD?

A

H&E: Normal glomeruli; Lipid may be seen in proximal tubule cells

EM: effacement of foot processes

IF: no immune complex deposits, negaitive IF

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5
Q

What kind of protein is lost in the urine in MCD?

A

Selective proteinuria for albumin. No loss of immunoglobulin

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6
Q

Tx for MCD

A

Excellent response to steroids (damage is mediated by cytokines from T cells)

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7
Q

What is focal segmental glomerulosclerosis (FSGS)?

A

MCC of nephrotic syndrome in hispanics, and African Americans

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8
Q

Etiology of FSGS

A

Usually idiopathic

May be associated with HIV, heroin use, and sickle cell disease

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9
Q

What is seen on the H&E stain, EM and IF of FSGS?

A

H&E stain - Focal (some glomeruli) and segmental (involving only part of the glomerulus) sclerosis

EM - effacement of foot processes

IF - negative IF (no immune complex deposits)

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10
Q

Tx for FSGS?

A

Poor response to steroids - usually progresses to chronic renal failure

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11
Q

MCC of nephrotic syndrome in Caucasian adults?

A

membranous nephropathy

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12
Q

Cause of membranous nephropathy?

A

Usually idiopathic

May be associated with Hep B/C, solid tumors, SLE or drugs

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13
Q

Does SLE normally develop nephrotic or nephritic syndrome?

A

SLe usually develops nephritic syndrome but when it does develop nephrotic, it will be membranous

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14
Q

What is seen on H&E stain, EM and If of membranous nephropathy?

A

H&E - thick glomerular BM on H&E

IF - immune complex deposition

EM - subepithelial deposits with “spike and dome” appearance

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15
Q

Does membranous nephropathy respond to steroids?

A

Poor response to steroids

Progresses to chronic renal failure

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16
Q

What proliferates in membranoproliferative glomerulonephritis?

A

Mesangial cells proliferate

17
Q

What is seen on H&E stain and IF of membranoproliferative glomerulonephritis?

A

H&E - thick glomerular BM often with tram-track apperance (more often seen with Type I)

IF - granular due to immune complex deposits

18
Q

What are the two types of membranoproliferative glomerulonephritis?

A

Type I - subendothelial deposits; associated with HBV and HCV

Type II (dense deposit disease) - intramembranous; associated iwth C3 nephritic factor

19
Q

Does MPGN lead to nephritic or nephrotic syndrome?

A

Either or both

20
Q

Does MPGN respond to steroids?

A

Poor response; progresses to chronic renal failure

21
Q

How does DM lead to nephrotic syndrome?

A

High serum glucose causes nonenzymatic glycosylation of the vascular BM resulting in hyaline arteriolosclerosis

This affects the efferent arteriole more than the afferent arteriole leading to a high GFR

Eventually progresses to nephrotic syndrome due to sclerosis of mesangial cells

22
Q

How is DM nephrotic ysndrome characterized?

A

Characterized by sclerosis of the mesangium with formation of Kimmelstiel-wilson nodules

23
Q

Tx for DM nephrotic syndrome

A

ACE inhibitors

24
Q

How does systemic amyloidosis cause nephrotic syndrome?

A

Kidney is the most commonly involved organ in systemic amyloidosis

Amyloid deposits in the mesangium, resulting in nephrotic syndrome

25
Q

How is systemic amyloidosis nephrotic syndrome characterized?

A

Apple-green birefringence under polarized light after staining with congo red

26
Q

what conditions cuse both nephritic and nephrotic syndrome?

A

Mebranoproliferative glomerulonephritis

Diffuse proliferative glomerulonephritis