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3: Paeds Y3/4 - Paediatrics [15] > [56] Nephrotic Syndrome > Flashcards

[56] Nephrotic Syndrome Flashcards

1
Q

What is nephrotic syndrome defined as?

A

A combination of:

  • Heavy proteinuria
  • Hypoalbuminaemia
  • Oedema
  • Hyperlipidaemia
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2
Q

What is considered heavy proteinuria?

A

PCR >200mg/mmol

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3
Q

What is considered hypoalbuminaemia?

A

Serum albumin <25g/L

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4
Q

What is the peak age of onset of nephrotic syndrome?

A

<6 years old

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5
Q

How can nephrotic syndrome be classified?

A
  • Primary vs Secondary

- Steroid sensitive vs Steroid dependant vs Steroid resistant

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6
Q

What are type are the majority of cases of nephrotic syndrome?

A

Steroid sensitive

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7
Q

What features suggest a steroid sensitive nephrotic syndrome?

A
  • Age 1-10 years
  • No macroscopic haematuria
  • Normal BP
  • Normal complement levels
  • Normal renal function
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8
Q

When can congenital nephrotic syndrome occur?

A

In the first 3 months of life

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9
Q

Is congenital nephrotic syndrome common?

A

No it is rare

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10
Q

Who is congenital nephrotic syndrome more common in in the UK?

A

Children of consanguineous parents

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11
Q

What is congenital nephrotic syndrome associated with?

A

High mortality

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12
Q

Why is congenital nephrotic syndrome associated with high mortality?

A

Due to combinations of hypoalbuminaemia

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13
Q

What happens in nephrotic syndrome?

A

The glomeruli are affected by inflammation or hyalinisation allowing proteins to pass through cell membranes and appear in the urine

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14
Q

What proteins can be lost in nephrotic syndrome?

A
  • Albumin
  • Anti-thrombin
  • Immunoglobulins
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15
Q

Why does oedema occur in nephrotic syndrome?

A

Albumin is the main protein that maintains oncotic pressure preventing leakage of fluid into the extracellular medium, its loss results in oedema

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16
Q

What is the common cause of steroid sensitive nephrotic syndrome?

A

Minimal change glomerulonephritis

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17
Q

What can cause steroid resistant nephrotic syndrome?

A
  • Focal segmental glomerulosclerosis
  • Membranoproliferative glomerulonephritis
  • Membranous nephropathy
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18
Q

What are the risk factors for developing nephrotic syndrome?

A
  • Male gender

- Indian subcontinental ethnicity

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19
Q

What is the main non-urinary symptom of nephrotic syndrome?

A

Breathlessness

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20
Q

What can cause breathlessness in nephrotic syndrome?

A

Pleural effusion and abdominal distension

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21
Q

What signs can be seen on examination of a child with nephrotic syndrome?

A
  • Periorbital oedema
  • Scrotal/vulval/leg/ankle oedema
  • Ascites
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22
Q

What blood tests should be performed when investigating nephrotic syndrome?

A
  • FBC
  • ESR
  • U&E’s
  • Creatinine and albumin
  • Complement levels (C3 and C4)
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23
Q

What urine tests should be conducted?

A
  • Dipstick for protein
  • Urine microscopy and culture
  • Urinary sodium concentration
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24
Q

If the patient has recently travelled, what test should be considered?

A

Malaria screen

25
What screening tests should be considered?
Hep B and C
26
What additional tests (not blood, urine or infection) should be considered?
- Antistreptolysin O - Anti-DNAase B - Throat swab - Renal biopsy
27
When may a renal biopsy be required in nephrotic syndrome?
If atypical or if not responding to steroid treatment
28
How does steroid sensitive nephrotic syndrome usually appear on light microscopy?
Normal
29
How does steroid sensitive nephrotic syndrome appear on electron microscopy?
Fusion of podocytes
30
What are the differentials of nephrotic syndrome?
- Nephritic syndrome - Cirrhosis - Severe malnutrition
31
What is the initial treatment of steroid sensitive nephrotic syndrome?
Oral corticosteroids (60mg/m^2/day prednisolone)
32
After 4 weeks of 60mg/m^2/day prednisolone how should treatment be adjusted?
Reduce dose to 40mg/m^2 on alternate days for 4 weeks
33
After 4 weeks of 40mg/m^2 on alternate days how should treatment be adjusted?
Wean or stop
34
What percentage of cases of nephrotic syndrome will be successfully treated with corticosteroids?
85-90%
35
Who should be involved if relapses are frequent or a high maintenance dose is required?
Nephrologist
36
Why should a nephrologist be involved in some cases?
To consider the use of steroid sparing medication
37
What steroid sparing medications can be used?
- Levamisole - Alkylating agents - Calcineurin inhibitors - Mycophenolate mofetil - Rituximab
38
Name an alkylating agent
Cyclophosphamide
39
Name two calcineurin inhibitors
- Tacrolimus | - Cyclosporin
40
What is rituximab
Anti-B cell monoclonal antibody
41
Who should handle the treatment of steroid resistant nephrotic syndrome?
Nephrologist
42
How is oedema managed in steroid resistant nephrotic syndrome?
- Diuretic therapy - Salt restriction - ACE inhibitors - NSAIDs
43
What testing can be used to help determine management efficacy in steroid resistant nephrotic syndrome?
Genetic testing
44
How is congenital nephrotic syndrome often needed to be treated?
Unilateral nephrectomy followed by dialysis until no longer nephrotic and old enough for renal transplant
45
What are the complications of nephrotic syndrome?
- Hypovolaemia - Respiratory compromise - Thrombosis - Hypercholesterolaemia
46
When does hypovolaemia usually occur in nephrotic syndrome?
In the initial phase of oedema formation due to fluid shift
47
What do children with hypovolaemia complain of?
Abdominal pain and faintness
48
]What are indications of hypovolaemia on testing?
- High packed cell volume of RBCs | - Low urinary sodium
49
What does a child with hypovolaemia need?
Urgent treatment with IV fluid
50
Why is urgent treatment with IV fluid required in hypovolaemia?
The child is at risk of vascular thrombosis and shock
51
What causes respiratory compromise in nephrotic syndrome?
Increasing peripheral oedema
52
What may be needed if respiratory compromise is severe?
Treatment with IV 20% albumin and furosemide
53
Why must care be taken when administering 20% albumin?
It can precipitate pulmonary oedema and hypertension from fluid overload
54
Why must care be taken when administering furosemide?
Can worsen hypovolaemia
55
What can thrombosis in nephrotic syndrome affect?
- Lungs - Brain - Limbs - Splanchnic circulation
56
Why is thrombosis a complication of nephrotic syndrome?
The patient can enter a hypercoagulable state
57
Why do patients with nephrotic syndrome often enter a hypercoagulable state?
- Urinary losses of antithrombin III - Thrombocytosis - Increased synthesis of clotting factors - Increased blood viscosity from raised haematocrit
58
What can exacerbate thrombocytosis?
Steroid therapy
59
Why can hypercholesterolaemia occur in nephrotic syndrome?
Cholesterol inversely correlates with serum albumin

3: Paeds Y3/4 - Paediatrics [15] (115 decks)

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