Nephrotic syndrome

Question 1

What is nephrotic syndrome?

Answer 1

When the basement membrane in the glomerulus becomes highly permeable to protein, allowing proteins to leak from the blood into the urine.

Question 2

Which age group is nephrotic syndrome common in?

Answer 2

Between the ages of 2 and 5 year

Question 3

How does nephrotic syndrome present?

Answer 3

Frothy urine, generalised oedema, pallor.

Question 4

What is the nephrotic syndrome triad?

Answer 4

Low serum albumin, proteinuria (>3+ protein on urine dipstick), oedema.

Question 5

What are 3 other features that occur in patients with nephrotic syndrome?

Answer 5

• deranged lipid profile - with high levels of cholesterol, triglycerides and low density lipoproteins
• hypertension - activation of RAAS & sodium retention **
• hyper coagulability, with an increased tendency to form blood clots

Question 6

What is the most common cause of nephrotic syndrome in children?

Answer 6

Minimal change disease - in this, nephrotic syndrome occurs in isolation without any clear underlying condition or pathology.

Question 7

What are some underlying causes of nephrotic syndrome?

Answer 7

It can be secondary to intrinsic kidney disease:

• focal segmental glomerulosclerosis
• membranoproliferative glomerulonephritis

It can also be secondary to an underlying systemic illness:

• Henoch schonlein purpura
• diabetes
• infections such as HIV, hepatitis and malaria

Question 8

What is the cause of minimal change disease?

Answer 8

Can occur in otherwise healthy children, without any clear risk factors or reason for developing the condition. It’s not clear why it occurs in most cases.

Question 9

What investigations are done for minimal change disease?

Answer 9

Renal biopsy and standard microscopy in MCD is usually not able to detect any abnormality. Urinalysis will show small molecular weight proteins and hyaline casts.

Question 10

What is the management of minimal change disease?

Answer 10

Corticosteroids (e.g., prednisolone) with a good prognosis.

Question 11

What is the general management of nephrotic syndrome?

Answer 11

• high dose steroids eg. prednisolone
• low salt diet
• diuretics may be used to treat oedema
• albumin infusions may be required in severe hypoalbuminaemia
• antibiotic prophylaxis may be given in severe cases

Question 12

How long are high dose steroids given for?

Answer 12

4 weeks and then gradually weaned over the next 8 weeks.

Question 13

How do children respond to steroids?

Answer 13

• 80% will respond to steroids and are referred to as steroid sensitive
• 80% of steroid sensitive patients will relapse at some point and need further steroids
• patients that struggle to wean steroids due to relapses are referred to as steroid dependant
• patients that don’t respond to steroids are referred to as steroid resistant

Question 14

What is given to steroid resistant children?

Answer 14

ACE inhibitors, immunosuppressants such as cyclosporine, tacrolimus or rituximab.

Question 15

What are the complications of nephrotic syndrome?

Answer 15

• hypovolaemia occurs as fluid leaks from the intravascular space into the interstitial space causing oedema and low blood pressure
• thrombosis can occur bc proteins that normally prevent blood clotting are lost in the kidneys and because the liver responds to low albumin by producing pro-thrombotic proteins
• infection occurs as the kidneys leak immunoglobulins, weakening the capacity of the immune system to respond. This is exacerbated with mediations that suppress the immune system, such as steroids
• acute or chronic renal failure
• relapse