Nephrotic and Nephritic Syndrome

In this condition, the glomerular basement membrane becomes very leaky to proteins, leading to the production of frothy urine, generalised oedema, and pallor. It most commonly occurs between 2 - 5 years.

The most common cause in children is Minimal Change Disease (MCD). Over 80% of patients have an excellent prognosis with treatment and, even though most relapse, they make a full recovery.

Secondary causes include:

• Intrinsic Kidney Disease - Focal segmental glomerulosclerosis, Membranoproliferative glomerulonephritis

• Systemic diseases – SLE, Henoch schonlein purpura (HSP), Diabetes

• Infections - HIV, Hepatitis, Malaria, Syphilis

• Drugs – NSAIDs, Heroin

Presentation

• Proteinuria

• Hypoalbuminaemia

• Generalised Oedema – Usually peri-orbital first, followed by peripheral oedema, ascites and perineal oedema

• Hyperlipidaemia

• Hyper-coagulability

• HTN

Investigations

• Renal biopsy - Typically only done if not responding to steroids

• Urinalysis – Proteinuria (Raised ACR)

• Lipid profile

Renal biopsy in MCD shows:

• No changes on light microscopy

• Podocyte effacement on electron microscopy

Management

The mainstay of treatment is High-dose steroids. Most respond really well within 4 weeks, and then are weaned off it over the next 8 weeks.

Other aspects of management includes:

• Low salt diet

• Diuretics if severely oedematous

• Albumin infusion in cases of severe hypoalbuminaemia

Complications

The 2 main complications that occur here are:

• Infection – urinary loss of immunoglobulins, therefore suppressing the immune system; treatment with immunosuppressants exacerbates this

• Thrombosis – urinary loss of anti-thrombotic proteins

Other complications include:

• Hypovolaemia – due to oedema

• Renal failure

• Relapse