Paediatric Nephrotic Syndrome

Question 1

In summary, what causes proteinuria/haematuria in children?

Answer 1

glomerular disease - nephrotic syndrome

nephritic syndrome

Question 2

In summary, what causes acute kidney injury in children?

Answer 2

haemolytic uraemic syndrome

Question 3

In summary, what causes chronic kidney disease in children?

Answer 3

developmental anomalies leading to reflux nephropathy

Question 4

What is a normal GFR in neonates?

Answer 4

20-30ml/min/1.73m2

Question 5

What is a normal GFR in children aged 2?

Answer 5

same as adult: 80-120ml/min/1.73m2

Question 6

What are the 3 layers of the glomerular filtration barrier?

Answer 6

• fenestrated endothelial cell
• glomerular basement membrane
• podocytes

Question 7

Is it more common to acquire or have a congenital glomerulopathy?

Answer 7

more common to have an acquired glomerulopathy

Question 8

When considering a glomerulopathy, what dictates the clinical presentation and most likely diagnosis?

Answer 8

blood and protein in varying amounts

Question 9

Take home message. What does proteinuria signify?

Answer 9

glomerular injury

Question 10

Why can glomerulopathies have a progressive nature?

Answer 10

hypertension and the proteinuria can worsen condition

Question 11

Nephrotic syndrome and nephritic syndrome are 2 overlapping ways that may be used to narrow down diagnoses. What makes conditions have a nephritic syndrome presentation compared to classical nephrotic syndrome presentation?

Answer 11

• nephritic syndrome is characterised by increasing haematuria and intravascular overload
• nephrotic syndrome is characterised by increasing proteinuria and intravascular depletion

Question 12

What 3 signs define nephrotic syndrome?

Answer 12

• nephrotic range proteinuria (>250mg/mmol)
• hypoalbuminaemia (normal is 36-44g/l)
• oedema

Question 13

Why does oedema occur in nephrotic syndrome disease processes?

Answer 13

damage in glomeruli results in protein leaking out which allows water to follow it

Question 14

Where can the oedema occur in nephrotic syndrome?

Answer 14

periorbital, pitting oedema of legs, ascites

Question 15

What are 3 ways you can test for proteinuria?

Answer 15

• dipstix (+++ usually indicates abnormal)
• protein creatinine ratio (use early morning urine and are looking for nephrotic range proteinuria)
• 24hr urine collection

Question 16

How would you diagnose a patient with nephrotic syndrome?

Answer 16

• oedema
• proteinuria on dipstix
• protein creatinine ratio
• urine sodium- 10
• bloods (low albumin and normal creatinine)

Question 17

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

Answer 17

minimal change disease

Question 18

What is minimal change disease?

Answer 18

• have nephrotic syndrome but only on electron microscopy can the effacement of the podocyte for processes be seen
• this allows the proteinuria
• 85% will have minimal change disease

Question 19

How would you diagnose/ treat minimal change disease?

Answer 19

• recognise young child with nephrotic syndrome is likely to have minimal change disease
• treat with steroids (prednisolone for 8 weeks) and if responsive then has MCD
• only if the steroids do not work should you consider renal biopsy

Question 20

What is another common (but much less common than MCD) cause of nephrotic syndrome?

Answer 20

focal segmental glomerulosclerosis (FSGS)

Question 21

What is focal segmental glomerulosclerosis?

Answer 21

• when some (focal) glomeruli in the kidney become affected by scarring change (sclerosis) in only a segment (segmental) of the glomerulus
• the glomeruli that are affected by the scarring allow proteins to pass through their filtration barrier, ultimately resulting in nephrotic syndrome

Question 22

How would FSGS be treated?

Answer 22

• can be steroid resistant!

- so tend to use second lin treatment which would be immunosuppression e.g. cyclosporin, tacrolimus

Question 23

What tends to be the outcome of nephrotic syndrome disease?

Answer 23

• MCD tends to result in remission but can recur

- FSGS can result in remission ht sometimes can progress and result in chronic kidney disease

Question 24

What are 3 causes of acquired nephrotic syndrome?

Answer 24

• HSP/ IgA
• lupus
• post infectious