peds nephro Flashcards

1
Q

a mutation in the nephrin gene that causes nephrotic syndrome in the first year of life is

A

congenital NS of the Finnish type

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2
Q

Congenital NS of the finnish type is characterized by

A

Thrombosis and malnutrition

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3
Q

minimal change disease is usually associated with NS in children when?

A

after the first year of life

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4
Q

14 month old presenting with generalized edema, what can this be?

A

minimal change

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5
Q

in young children:

who gets more Minimal change disease

A

Males 3:2

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6
Q

what features suggest you are dealing with something other than Minimal change disease nephrotic syndrome

A

age 12

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7
Q

Initial treatment of minimal change disease includes

A

Prednisone over 3-4 months
control of edema
low salt

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8
Q

minimal change disease DOES NOT LEAD TO

A

chronic kidney disease

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9
Q

Steroid resistant nephrotic syndrome iS NOT

A

minimal change disease

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10
Q

long term remission from minimal change disease happens

A

after puberty

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11
Q

If urine is tea-colored think

A

nephritic syndrome

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12
Q

urine microscopy findings in nephritic syndrome

A

RBC and RBC casts

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13
Q

most common primary renal disease associated with hypocomplementemic GN

A

post infectious - post strep GN

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14
Q

most common systemic disease associated with hypocomplementemic GN

A

SLE

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15
Q

most common primary renal disease associated with normocomplementemic C3

A

IgA nephropathy

ANCA + pauci

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16
Q

most common systemic disease associated with normocomplementemic C3

A

ANCA+ disease
anti GBM
Henoch Schonlein purpura

17
Q

Low C3
Nephritic syndrome
evidence of previous infection suggests

A

Post-infectious GN

18
Q

when does post strep GN occur

A

10-14 days after a strep pharyngitis infection

up to 3 weeks after impetigo infection

19
Q

how do you NOT treat post-infectious GN

A

steroids! dont give,

give diuretics, antihypertensives

20
Q

how long for the C3 to be back to normal in post-infectious GN

A

6-8 wks

21
Q

classic triad of HUS

A

MAHA
Thrombocytopenia
Acute kidney Injury

22
Q

Main cause of acute kidney injury in children

A

HUS

23
Q

typical or diarrhea positive HUS is caused by

A

Shiga toxin producing E coli

over 90% of cases

24
Q

classic blood film features in HUS

A

shistocytes

25
Q

Primary event in the pathogenesis of HUS

A

damage to endothelial cells by the shiga toxin

26
Q

the cardinal lesion in HUS is

A

arteriolar and capillary microthrombi microangiopathy

RBC fragmentation

27
Q

TREATment for symptomatic HUS

A
  • dialysis for the aKI
  • platelet tansfusion
  • red cell transfusion
28
Q

Do not use plasma exchange or eculizumab for

A

Diarrhea positive HUS

29
Q

mortality from HUS is

A

5% during the acute phase