Makindo Medical Notes

Related Subjects: |Encopresis in Children |Enuresis/Bedwetting in Children |Acute Glomerulonephritis in Children |Nephrotic Syndrome in Children |Acute Appendicitis in Children |Gastro-oesophageal reflux in Children |Intussusception in Children |Panayiotopoulos Syndrome in Children |Reflex anoxic attacks in Children

In nephrotic syndrome, protein leaks from blood to urine through glomeruli, causing hypoproteinaemia, including loss of immunoglobulins, and oedema. Before steroids (and antibiotics), many died from infections.

About

• Generalised Oedema
• Proteinuria (urinary protein-creatinine ratio (PCR) >200mg/mmol)
• Albumin <25g/L ±
• Hypercholesterolaemia

Graphic

Causes

• In 90% of cases, the cause is unknown.
• Any of the causes of nephritis can also cause nephrotic syndrome.
• Usually minimal change glomerulonephritis (GN), often associated with allergy and IgE production.
• Classified as: Steroid-sensitive (SS), Steroid-dependent (SD), Steroid-resistant (SR)

Symptoms:

• Insidious onset of oedema, typically starting periorbital and then becoming generalized.
• Anorexia, gastrointestinal disturbance, infections, irritability, ascites, oliguria.
• Urine: Frothy; albuminous ± casts; decreased sodium (secondary hyperaldosteronism).
• Blood: Decreased albumin (resulting in decreased total calcium); normal urea and creatinine levels.

Investigations

• Protein-to-Creatinine Ratio (PCR): >200 mg/mmol
• Albumin: <25 g/L ± hypercholesterolemia
• Renal Biopsy for older children with any of the following: Haematuria, Elevated blood pressure, Elevated urea levels, Unselective protein loss (large molecular weights as well as small), Treatment failures

General Management

• Get help and consult with a senior.
• Eat healthily with no added salt and no high-protein content.
• Fluid restriction to 800–1000 mL/day.
• Administer diuretics if very oedematous and no evidence of hypovolemia.
• Albumin infusion for symptomatic hypovolemia or severe diuretic-resistant oedema (discuss with senior).
• Pneumococcal vaccination if age >2 years.

Medical management

• Prednisolone: 60 mg/m²/day (max 80 mg) for 4 weeks, then 40 mg/m² every 48 hours for 4 weeks, followed by a taper over 4 months. 90% respond within 8 weeks. Most children with nephrotic syndrome respond to corticosteroids. Many have a relapsing course with recurrent oedema and proteinuria. Corticosteroids reduce mortality to 3%, with infection remaining the most important cause of death. If steroid toxicity and relapsing nephrotic syndrome, consider cyclophosphamide or ciclosporin. Note: Ciclosporin is nephrotoxic, so monitor blood pressure.
• Steroid-resistant proteinuria, Consider Enalapril >2yrs: dose example: 1 month–12yrs: initially 100mcg/kg/day (monitor BP carefully for 1–2h; increase as needed to 0.5mg/ kg/12h (maximum). In one study, urine protein electrophoresis showed a reduction of 80% and 70% in the total protein and albumin, respectively, after enalapril. Some patients become free of proteinuria. ACE-i are discontinued if renal failure occurs, eg during infections.

Complications

• Pneumococcal peritonitis or other infections due to renal loss of immunoglobulins.
• Increased risk of venous thrombosis and thromboembolism (VTE).