Nephrotic Syndrome in Children

Clinical Description

Presence of nephrotic range proteinuria, oedema, hyperlipidemia, and hypoalbuminemia

Criteria

• 1. Edema
• 2. Proteinuria (at least 3+)
• 3. Hypoalbuminemia usually plus Hyperlipidemia

Causes

Primary:

• Minimal change disease, Focal segmental glomerulosclerosis, Membranoproliferative glomerulonephritis

Secondary:

• Hepatitis B, HIV, Lupus, Postinfectious GN, Subacute infective endocarditis

Congenital:

• Occur in children less than 1 year of age

Signs and Symptoms

• Signs of systemic disease like joint complaints, rash, fever
• Preceding symptoms or illnesses e.g. respiratory, skin or urinary tract infections
• Oedema
• Abdominal pain, flank pain
• Breathlessness and cough
• Past medical history of similar problem
• Family history of renal problems
• ascites

Investigations

• Urine dipstick and microscopy
• FBC,
• Urea, electrolytes and Creatinine
• albumin
• HIV test
• Hepatitis B and C
• VDRL
• Malaria parasites
• Stool microscopy
• Kidney ultrasound

Treatment

First episode

• Admit
• Salt restriction
• fluid restriction if fluid overload
• Stat dose Praziquantel
• Monitor daily: blood pressure, weight and urine dipstick until normal
• Encourage mobilization (bed rest may increase risk of venous thrombosis.)
• Steroids: Prednisolone 2mg/kg/day for 4-6 weeks, If no response in 4 -6 weeks, refer to tertiary facility.
• Diuretics: if severely fluid overloaded
• Furosemide 1mg/kg IV(maximum 40mg) 12-8 hourly
• If no response after few days,refer to tertiary facility
• Pneumococcal vaccine, if available, and not previously immunized
• Follow up all patients in paediatric/renal clinic

Relapses

Proteinuria at least ++ for more than 3 days

• Start treatment as above
• Refer to tertiary facility
• The same as above, except

Complications

• Infections
• Thromboembolism (Doppler Ultrasound)
• Acute kidney injury
• Hypovolemia
• Protein malnutrition
• Hyperlipidemia
• Spontaneous bacterial peritonitis

Referral criteria

• Not responding after 4 -6 weeks of steroids
• All cases of relapse
• Severe fluid overload not responding to diuretics
• Patients with complications

NEPHROTIC SYNDROME IN ADULTS

Adults

• Give Frusemide 40-80 mg as a single dose each morning
• Give Enalapril 10-20 mg every day (use with caution, stop if renal function deteriorates)
• A trial of steroids is indicated (responsiveness to steroids in adults is less than in children). Give Prednisolone 50-60 m every day for up to two months, tapering off is required after response
• Give Proton pump inhibitors and calcium carbonate plus vitamin D tablets for bone protection if long term steroid use is indicated

If Schistosomiasis is diagnosed or suspected as cause

• Give Praziquantel 40 mg/kg single dose

Complications and Referral Criteria

Refer all patients to a physician specialist, pediatrician or nephrologist immediately after diagnosis and stabilization.

If presentation is acute:

• Give Phenoxymethlyl Penincillin 500 mg 6 hourly for 7 days
• Refer to Nephrologist

Note: Furosemide should not be given to children as routine treatment. Steroids use in nephrotic syndrome should be discussed with nephrologist