Nephrotic syndrome - MCD Flashcards

1
Q

GFR values - neonates

adult

A
  • 20-30ml/min/1.73m²

- 90-120

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2
Q

5 key functions of the kidney

A

Waste handling - urea, creatinine

Water handling

Salt balance - sodium and calcium phosphate

Acid base control - bicarbonate

Endocrine
red cells / blood pressure / bone health

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3
Q

what proteins are in the GBM?

A

Type IV collagen (COL4) and laminin

- MESENGIAL CELLS WITHIN

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4
Q

Podocytes - proteins within wall?

A

Podocin, nephrin

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5
Q

Mesangial cells - what do they regulate?

A

glomerular capillaries

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6
Q

how do patients present

A

haematuria and proteinuria

- frothy urine

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7
Q

what does proteinuria signify?

A

glomerular injury

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8
Q

Nephritic syndrome tends to have?

A

Increasing haematuria

Intravascular overload

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9
Q

Nephrotic syndrome tends to have?

A

Increasing proteinuria

Intravascular depletion

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10
Q

what component is affected in Acquired Glomerulopathy

- what disease does this cause?

A
Epithelial cell (podocyte)
-Minimal Change Disease
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11
Q

Basement Membrane condition

A

Post Infectious Glomerulonephritis (PIGN)

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12
Q

Endothelial cell condition

A

PIGN, Haemolytic Uraemic Syndrome (HUS)

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13
Q

Mesangial cell condition

A

HSP / IgA nephropathy

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14
Q

Congenital nephrotic syndrome proteins?

A

Podocin (AR), nephrin (AR)

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15
Q

Congenital basement membrane proteins - syndromes? (2)

A
Alport syndrome (XL)
Thin basement membrane disease (AD)
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16
Q

Endothelial/microvascular integrity  - what proteins?

A

Complement regulatory proteins (MPGN)

17
Q

what is a abnormal dipstick reading?

A

> 3

18
Q

proteinuria assessment in children?

A

Protein Creatinine Ratio (practical)

Early morning urine (best)

19
Q

Creatine range normal ?

A

20mg/mmol

20
Q

protein creatine ratio - nephrotic range?

A

250mg/mmol

21
Q

gold standard test for proteinuria? and what are the normal values - adults and children

A

24hr urine collection (gold standard)
normal <60mg/m²/24hrs

Nephrotic range>1g/m²/24hrs
Adults >3.5g/24hrs

22
Q

what is nephrotic syndrome

A
  • Nephrotic range proteinuria
  • Hypoalbuminaemia, low blood albumin
  • Oedema - increasing 3rd space fluid volume
23
Q

Typical presentation of nephrotic syndrome or MCD?

A

age 2 and a half
Gastroenteritis 10days prior
3 -4 days
Swollen face (worse in mornings), one eye closed in mornings, legs

24
Q

presentation of nephrotic syndrome in kids?

A
Looked well, pale
Inflated weight 14.9kg
Periorbital oedema, pitting oedema legs, ascites, small pleaural effusions
BP 98/56 (can be raised)
Frothy urine
25
Q

how do you know when it is nephrotic syndrome?

A

Oedema
Proteinuria >3

Protein Creatinine Ratio – 1200mg/mmol creatinine - high
Urine Na – 10mmol/l - low

bloods - albumin low

26
Q

microscopic heamaturia sign?

A

minimal change disease

27
Q

Typical features of nephrotic syndrome - MCD? (4)

A

Age 1 - 10
Normal blood pressure
No Frank haematuria
Normal renal function

28
Q

Atypical features of MCD - nephrotic syndrome

A

Suggestions of autoimmune disease
Abnormal renal function
Steroid resistance

renal biopsy

29
Q

Drug treatment for MCD?

A

Prednisolone 8 weeks

30
Q

Prednisolone side effects? (5)

A
personality changes
greater infection rate
hypertension
GI distress - increased acid
growth
31
Q

infection risk of glucocorticoids (Prednisolone)

A

Varicella status
Pneumococcal vaccination
Antibiotic prophylaxis

32
Q

if steroids don’t work for MCD - what condition is this more indicitive of?

A

Focal Segmental Glomerulosclerosis - podocyte loss

33
Q

pathogenesis of nephrotic syndrome?

A

Interaction between lymphocytes (T and B cells) and podocytes

34
Q

congenital FSGS - Mutations in what genes?

A

NPHS1 – nephrin
NPHS 2 – podocin
Podocyte loss