Nephrotic Syndrome

Question 1

What are the features of nephrotic syndrome?

Answer 1

1. Proteinuria (ACR >250mg/mmol)
2. Hypoalbuminaemia (<30g/L)
3. Oedema
4. Frothy urine, hypercoagulability, hypercholesterolaemia.

Question 2

What is the pathophysiology of nephrotic syndrome in these conditions?

1. Minimal change disease
2. Membranous nephropathy
3. Focal segmental glomerulosclerosis
4. Membranoproliferative glomerulonephritis

Answer 2

1. Abnormal podocyte function
2. Immune mediated damage to podocytes
3. Podocyte injury/death
4. GBM/endothelial cell pathology

Question 3

What is this describing and how is it treated?

1. Commonest primary nephrotic glomerulonephropathy
2. Idiopathic, paraneoplastic, or associated with NSAIDs/lithium.
3. No renal failure
4. Light microscopy normal

Answer 3

1. Minimal change disease

2. Prednisolone 1mg/kg for 4-16 weeks

Question 4

What is this describing and how is it treated?

1. Commonest primary nephrotic glomerulonephropathy in Afro-Caribbean adults.
2. Primary (idiopathic) or secondary to HIV, heroin, lithium, lymphoma.
3. All at risk of progressive CKD and ESRF, disease recurs in 50% of renal transplants.

Answer 4

1. Focal segmental glomerulosclerosis

2. ACEi for BP and steroids in primary disease only

Question 5

What is this describing and how is it treated?

1. Commonest primary nephrotic glomerulonephropathy in Caucasian adults.
2. Primary (idiopathic) or secondary to malignancy, hep B/C, streptococcus, malaria, SLE, RA, sarcoidosis, drugs.
3. Anti-phospholipase A2 receptor Ab present in 70-80% of idiopathic

Answer 5

1. Membranous nephropathy
2. ACEi for BP, Ponticelli immunosuppression regime (prednisolone and cyclophosphamide in those at high risk of progression)

Question 6

What is this describing and how is it treated?

1. Immune-complex associated - deposit in kidney and activate complement. Infection, monoclonal gammopathy, autoimmunity.
2. C3 glomerulonephropathy - defect in alternative complement pathway.

Answer 6

1. Membranoproliferative glomerulonephritis

2. ACEi for BP, treat underlying cause in immune complex disease.

Question 7

What are the secondary causes of nephrotic syndrome?

Answer 7

SLE, hep B/C, HIV, diabetes mellitus, malignancy.

Question 8

How is oedema managed in nephrotic syndrome?

Answer 8

1. Fluid (1L/day) and salt restriction.
2. Loop diuretics (furosemide)
3. Aim 0.5-1kg weight loss per day
4. Add thiazide if refractory

Question 9

How is proteinuria reduced in nephrotic syndrome?

Answer 9

ACEi/ARB

Question 10

What are the complications of nephrotic syndrome?

Answer 10

1. Thromboembolism - increased clotting factors (give heparin/warfarin)
2. Infection - urine losses of Abs and immune mediators
3. Hyperlipidaemia - hepatic synthesis in response to low oncotic pressure