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B - Paediatrics MANAGEMENT > Nephrotic Syndrome > Flashcards

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
Q

What screening tests should be considered?

A

Hep B and C

26
Q

What additional tests (not blood, urine or infection) should be considered?

A
  • Antistreptolysin O
  • Anti-DNAase B
  • Throat swab
  • Renal biopsy
27
Q

When may a renal biopsy be required in nephrotic syndrome?

A

If atypical or if not responding to steroid treatment

28
Q

How does steroid sensitive nephrotic syndrome usually appear on light microscopy?

A

Normal

29
Q

How does steroid sensitive nephrotic syndrome appear on electron microscopy?

A

Fusion of podocytes

30
Q

What are the differentials of nephrotic syndrome?

A
  • Nephritic syndrome
  • Cirrhosis
  • Severe malnutrition
31
Q

What is the initial treatment of steroid sensitive nephrotic syndrome?

A

Oral corticosteroids (60mg/m^2/day prednisolone)

32
Q

After 4 weeks of 60mg/m^2/day prednisolone how should treatment be adjusted?

A

Reduce dose to 40mg/m^2 on alternate days for 4 weeks

33
Q

After 4 weeks of 40mg/m^2 on alternate days how should treatment be adjusted?

A

Wean or stop

34
Q

What percentage of cases of nephrotic syndrome will be successfully treated with corticosteroids?

A

85-90%

35
Q

Who should be involved if relapses are frequent or a high maintenance dose is required?

A

Nephrologist

36
Q

Why should a nephrologist be involved in some cases?

A

To consider the use of steroid sparing medication

37
Q

What steroid sparing medications can be used?

A
  • Levamisole
  • Alkylating agents
  • Calcineurin inhibitors
  • Mycophenolate mofetil
  • Rituximab
38
Q

Name an alkylating agent

A

Cyclophosphamide

39
Q

Name two calcineurin inhibitors

A
  • Tacrolimus

- Cyclosporin

40
Q

What is rituximab

A

Anti-B cell monoclonal antibody

41
Q

Who should handle the treatment of steroid resistant nephrotic syndrome?

A

Nephrologist

42
Q

How is oedema managed in steroid resistant nephrotic syndrome?

A
  • Diuretic therapy
  • Salt restriction
  • ACE inhibitors
  • NSAIDs
43
Q

What testing can be used to help determine management efficacy in steroid resistant nephrotic syndrome?

A

Genetic testing

44
Q

How is congenital nephrotic syndrome often needed to be treated?

A

Unilateral nephrectomy followed by dialysis until no longer nephrotic and old enough for renal transplant

45
Q

What are the complications of nephrotic syndrome?

A
  • Hypovolaemia
  • Respiratory compromise
  • Thrombosis
  • Hypercholesterolaemia
46
Q

When does hypovolaemia usually occur in nephrotic syndrome?

A

In the initial phase of oedema formation due to fluid shift

47
Q

What do children with hypovolaemia complain of?

A

Abdominal pain and faintness

48
Q

]What are indications of hypovolaemia on testing?

A
  • High packed cell volume of RBCs

- Low urinary sodium

49
Q

What does a child with hypovolaemia need?

A

Urgent treatment with IV fluid

50
Q

Why is urgent treatment with IV fluid required in hypovolaemia?

A

The child is at risk of vascular thrombosis and shock

51
Q

What causes respiratory compromise in nephrotic syndrome?

A

Increasing peripheral oedema

52
Q

What may be needed if respiratory compromise is severe?

A

Treatment with IV 20% albumin and furosemide

53
Q

Why must care be taken when administering 20% albumin?

A

It can precipitate pulmonary oedema and hypertension from fluid overload

54
Q

Why must care be taken when administering furosemide?

A

Can worsen hypovolaemia

55
Q

What can thrombosis in nephrotic syndrome affect?

A
  • Lungs
  • Brain
  • Limbs
  • Splanchnic circulation
56
Q

Why is thrombosis a complication of nephrotic syndrome?

A

The patient can enter a hypercoagulable state

57
Q

Why do patients with nephrotic syndrome often enter a hypercoagulable state?

A
  • Urinary losses of antithrombin III
  • Thrombocytosis
  • Increased synthesis of clotting factors
  • Increased blood viscosity from raised haematocrit
58
Q

What can exacerbate thrombocytosis?

A

Steroid therapy

59
Q

Why can hypercholesterolaemia occur in nephrotic syndrome?

A

Cholesterol inversely correlates with serum albumin

B - Paediatrics MANAGEMENT (106 decks)

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