Nephrotic Syndrome

Question 1

How can you classify nephrotic syndrome?

Answer 1

1. Steroid sensitive - patient enters remission after starting steroid Tx, usually within two weeks but can take up to 28 days
2. Steroid dependent - patient enters remission but relapses when prednisolone is weaned or within 14 days of prednisolone treatment being stopped (frequently = >2x in 6/12 period)
   - Relapse defined as 3 consecutive days of 3+ proteinuria, or 5 days of 2+ proteinuria on early urine dip
3. Steroid resistant - patient does not enter remission after 28 days of steroid use and after receiving 3 pulses of IV methylprednisolone sodium succinate

Question 2

What is the most common cause?

Answer 2

Minimal change disease (type of steroid-responsive nephrotic syndrome)
- Pure nephrosis (NO haematuria)
- Boys > girls, 1-10 years
- Normal BP, renal function, complement levels (if it isn’t normal - question Dx)
- Steroid responsive
> IF no response, question the diagnosis
> Advantage of treating the with steroids early - minimise risk of relapse
- EM: podocyte effacement

Question 3

What are the signs and symptoms?

Answer 3

1st = peri-orbital oedema (often misdiagnosed as allergy)

2nd = other delayed features of oedema (i.e. peripheral leg swelling), features of underlying diagnosis

Question 4

What investigations would you carry out for nephrotic syndrome?

Answer 4

• Urine dipstick testing, urea, U&Es, urine MC&S, urinary sodium
• FBC, ESR, creatinine, albumin
• Complement levels (C3, C4)
• Anti-streptolysin O or anti-DNase B titres (recent streptococcal throat infection)
• HBV, HCV, malaria screen

Question 5

What is the management for nephrotic syndrome?

Answer 5

1. oral prednisolone for 4-6 weeks (reduced dose from 4+ weeks)
   - Renal histology of steroid-sensitive nephrotic syndrome = normal on light microscopy
   - However, on electron microscopy, fusion of podocytes is seen (minimal change disease)
2. (unresponsive or atypical) - specialised renal biopsy

Pre admission: Hx + exam, Bloods + IV cannula,
urine for urine albumin/creatinine ratio
(microalbumin)
Admit to paediatric ward IF blood & urine results
confirm nephrotic syndrome
- 2x daily weights (morning & evening)
- Measure height and complete growth chart
- Strict fluid balance charts
- 4 hourly temperature, pulse, respiratory rate
and BP
- Chickenpox antibody status - Varicella Zoster
IgG screen
- Measles IgG if no Hx of MMR vaccination

Question 6

What are the complications of nephrotic syndrome?

Answer 6

Risk of thrombosis -> loss of AT-III in the urine -> hypercoagulable state
Risk of infection -> loss of immunoglobulin in urine -> infection risk (esp. NHS bacteria)
Hypercholesterolaemia -> urinary albumin loss -> less oncotic pressure -> hepatic cholesterol synthesis