GU - Nephrotic Syndrome

Question 1

Defintion

Answer 1

• 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 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
• Ascites
• SOB due to presence of pleural effusions and abdominal distension

Question 4

Diagnosis

Answer 4

• Urine dipstick
• Urine protein:creatinine ratio
• Urine microscopy
• Urine cultures
• Bloods - FBC, U+E, albumin, bone profile

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.
• Can lead to hypovolaemia, thrombosis, infection and hypercholesterolaemia
• It may resolve or can cause relapses and can progress to renal failure
• Some patients may respond to cyclophosphamide, tacrolimus or rituximab
• Causes = focal segmental glomerulosclerosis (MC) and membranous nephropathy.