GU - Nephrotic Syndrome

Question 1

Defintion

Answer 1

Nephrotic syndrome occurs when the basement membrane in the glomerulus becomes highly permeable to protein
- Proteinuria with 3+/4+ on urine dipstick or a urine protein:creatinine ratio of 200mmg/mol
- Hypoalbuminaemia <25g/l
- Oedema

Question 2

Aetiology

Answer 2

• Primary is generally an idiopathic cause: Minimal change disease is the most common cause in children
• Secondary due to: FSGS, Membranoproliferative glomerulonephritis
• Secondary can be caused by systemic diseases such as HSP, SLE

Question 3

Signs and symptoms

Answer 3

• Periorbital oedema, often on awakening
• Scrotal, vulval, leg and ankle oedema
• SOB due to presence of pleural effusions and abdominal distension
• Frothy urine
• Recurrent infections
• Increased risk of VTE

Question 4

Diagnosis

Answer 4

Urinalysis: proteinuria predominates over haematuria; lipid casts
24-hour urine protein collection: > 3.5 g protein
Urine albumin-creatinine ratio (ACR): raised due to proteinuria
U&Es: monitor eGFR and creatinine to assess for renal failure
LFTs: hypoalbuminemia < 25 g/L
Lipid profile: hypercholesterolaemia and hypertriglyceridemia
Renal ultrasound: exclude structural pathology
Minimal change disease renal biopsy: Electron microscopy (EM) : effacement of foot processes

Question 5

Management

Answer 5

Medical
1. Corticosteroid therapy:
- Prednisolone 60 mg/m2 /day in a single morning dose (maximum 80mg/day) for 28 days.
- Then reduce dosage to 40mg/m2 /alternate day (maximum 50mg/alternate day) given once daily, for 28 days and then stop without tapering.
2. Diuretics may be needed to control oedema whilst the steroids are taking effect = furosemide 1-2mg/kg/day
3. Diet with reduced salty food diet
4. Pneumococcal immunisations = 23 valent pneumococcal polysaccharide vaccine

Question 6

Most children will have remissions and relapses however the relapses will generally become less frequent and may stop once they are in teenage years

Question 7

What is steroid resistant nephrotic syndrome

Answer 7

• Common in Asian boys = potential complication of nephrotic syndrome and requires specialist paediatric nephrologist involvement.
• It may resolve or can cause relapses and can progress to renal failure
• Some patients may respond to ACE-Inhibitors and Immunosuppression: cyclophosphamide, tacrolimus or rituximab
• Causes = focal segmental glomerulosclerosis (MC) and membranous nephropathy.

Question 8

Epidemiology

Answer 8

2 - 5 years
Presenting with frothy urine, generalised oedema and pallor

Question 9

Complications

Answer 9

Hypovolaemia
Thrombosis
Infection
Acute or chronic renal failure
Relapse