Nephrotic syndrome

Question 1

What is nephrotic syndrome?

Answer 1

Glomerular disorder - leaking protein from a damaged glomerulus

Question 2

Give the classic triad of nephrotic syndrome

Answer 2

Generalised oedema
Heavy proteinuria (>200mg/mmol)
Hypoalbuminemia (<25g/L)

Question 3

What is the incidence of nephrotic syndrome?

Answer 3

2 per 100,000

Question 4

Which ethnicity has a higher incidence?

Answer 4

Asian

Question 5

Which sex has a higher incidence of nephrotic syndrome?

Answer 5

Males

Question 6

What is nephritis

Answer 6

Inflammation involving any part of the nephron or interstitium

Question 7

What is the basement membrane made up of?

Answer 7

Specialised epithelial cells - podocytes which normally fuse together and prevent proteins being filtered

Question 8

What happens to the basement membrane structure in nephrotic syndrome?

Answer 8

Podocytes become flattened and start to allow leaking of proteins

Question 9

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

Answer 9

Minimal change disease

Question 10

List some other causes of nephrotic syndrome in children

Answer 10

Congenital nephrotic syndromes
Focal segmental glomerulosclerosis
Mesangiocapillary glomerulonephritis

Question 11

When is a diagnostic renal biopsy performed?

Answer 11

When there are atypical features of nephrotic syndrome

Question 12

List the atypical features of nephrotic syndrome

Answer 12

Age <1yo/>12yo

HTN

Impaired renal function

Frank haematuria

Steroid resistant nephrotic syndrome

Question 13

Give the differential diagnosis of nephrotic syndrome

Answer 13

Heart failure
Allergic reaction
Malnutrition

Question 14

List the investigations for nephrotic syndrome

Answer 14

Urine dip
Urinary protein: creatinine ratio
Urea and electrolytes
Full blood count
Serum albumin
Varicella zoster serology

Question 15

Which investigations would be ordered if presenting with atypical features?

Answer 15

Complement levels
Hepatitis serology
Anti-Streptolysin O titre (ASOT)
Autoimmune investigations - ANA, ANCA, anti-dsDNA

Question 16

Describe the management of nephrotic syndrome

Answer 16

Discuss with nephrologist if atypical features

High dose steroids

Low salt diet

Prophylactic antibiotics

Question 17

What is it called if the nephrotic syndrome responds to steroids?

Answer 17

Steroid sensitive nephrotic syndrome (SSNS)

Question 18

What is it called when a child has more than one relapse of nephrotic syndrome?

Answer 18

Steroid resistant or frequently relapsing nephrotic syndrome

Question 19

What additional treatment may children with steroid resistant nephrotic syndrome require?

Answer 19

Low dose maintenance steroids or immunomodulatory drugs (levamisole, cyclophosphamide, ciclosporin or tacrolimus)

Question 20

Why is a low salt diet required in nephrotic syndrome?

Answer 20

Avoid worsening the oedema

Question 21

Why are prophylactic antibiotics needed in nephrotic syndrome?

Answer 21

Leak immunoglobulins from the kidneys so at high risk of infection

Question 22

Describe the complications of nephrotic syndrome

Answer 22

Fluid management - may become intravascularly deplete

Infection

Thrombosis