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Nephrotic Syndrome Nicholls > Nephrotic Syndrome II > Flashcards

Nephrotic Syndrome II Flashcards

1
Q

What is an ApoL1 mutation?

A

mutations that cause truncations of apolipoprotein L1 which confers resistance to trypanosomiasis (african sleeping sickness) but leads to susceptibility to focal segmental glomerulosclerosis

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2
Q

What in the name of the good lord is soluble urokinase-type plasminogen activator receptor?

A

a circulating factor that binds to the podocyte anchor beta3-integrin and could be a common cause of focal segmental glomerulosclerosis.

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3
Q

What is M-type phospholipase A2 receptor?

A

podocyte membrane component that forms immune complexes with autoantibodies probably causing most primary membranous nephropathy

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4
Q

What are the two categories of Nephrotic syndromes?

A
  1. ) podocytopathies- those that occur on visceral epithelium
  2. ) Membranous nephropathy- immune complex deposition
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5
Q

Two different podocytopathies?

A
  1. ) Minimal Charge

2. ) Focal segmental glomerulosclerosis

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6
Q

Two different types of membranous nephropathy?

A
  1. ) Endogenous antigen immune complex

2. ) Filtered antigenimmune complex

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7
Q

What are some highlights of the epidemiology of minimal change disease?

A

Affects the very young and very hold. 75% of nephrotic syndrom in children. 25% in adults. more common in whites than blacks. Children = 2:1 male to female.

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8
Q

Minimal change disease is a nephrotic syndrome so it involves what

A

Proteinuria, ding dong

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9
Q

What type of protein is predominant in minimal change disease?

A

Albumin

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10
Q

How do you definitively diagnose minimal change disease?

A

renal biopsy….ight microscopy is negative but electron microscopy shows effacement of the podocyte foot processes

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11
Q

How do you treat minimal change disease?

A

glucocorticoids.

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12
Q

So if you see a kid with nephrotic syndrome it is most likely what?

A

Minimal change disease

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13
Q

What is focal segmental glomerulosclerosis?

A

Involves deposition of a basement membrane like material associated with podocyte injury only in certain glomeruli (hence focal) and only parts of that glomerulus (hence segmenal)

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14
Q

What does FSG progress to>

A

Sclerosis

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15
Q

Epidemiology of FSG?

A

Most common in adults, blacks, males. 64% of nephrotic syndrome in black adults

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16
Q

What mutation in African Americans puts them at risk for FSG?

A

ApoL1

17
Q

Can FSG be both secondary and Primary?

A

Yes…can follow things like glomerulonephritis, HIV, drug use, etc…

18
Q

What circulating factor could be a cause of FSG?

A

suPAR…soluble urokinase-type plasminogen activator binds to the podocyte anchor beta 3 integrin and can cause focal segmental glomerulosclerosis

19
Q

What is the type of FSG with the worst prognosis?

A

collapsing glomerulopathy

20
Q

What are the different subtypes of FSG>

A

Collapsing glomerulopathy, vascular pole, urinary pole, Glomerular tip (white ppl)

21
Q

Collapsing glomerulopathy is most predominant among what population?

A

African Americans, particularly kids

22
Q

Collapsing glomerulopathy is often associated with what?

A

HIV and drug use

23
Q

treatment of FSG?

A

corticosteroids

24
Q

Prognosis is worse in FSG or minimal change>?

A

FSG…importatn to differntiate between them

25
Q

What are the five major characteristics that differentiate FSG from Minimal change?

A

1) Older age
2) Hematuria
3) Non-specific proteinuria…mostly albumin in MC
4) hypertension
5) poor response to steroids

26
Q

What might immunoflourescnece show in FSG and not in MC?

A

IgM in glomerular segments

27
Q

Characterization of membranous nephropathy?

A

chronic, slow moving, BM thickening, subepithelial immune deposits

28
Q

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

A

Membranous nephropathy

29
Q

Most pathogenic mecahnism of Membranous nephropathy

A

filtered antibodies binding to intrinsic antigen

30
Q

Most common antigen?

A

PLA2R

31
Q

Presentation of membranous nephropathy>

A

Most present with nephrotic syndrome, the rest with proteinuria

32
Q

treatment of membranour nephropathy?

A

ACE inhibitorsor angiotensin receptor blockade

Nephrotic Syndrome Nicholls (2 decks)

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