Nephrotic syndrome is characterized by generalized edema as a result of hypoproteinemia due to excessive protein loss in the urine. Hypercholesterolemia is commonly associated with MCNS. The causes of increased permeability of glomeruli to proteins is not known in 90% of children with nephrotic syndrome hence it is called as idiopathic or primary nephrotic syndrome. Majority i.e. 80 - 85% of NS respond to steroid therapy and if biopsied will show minimal change.

Diagnosis of nephrotic syndrome is by simple urine examination in a child who presents with edema. The edema is generalized involving eyes, abdominal wall, genitals and ankles; ascites may be present and B.P is normal in majority of cases.

Urine examination shows presence of proteins- 3+ to 4+ by sulphosalicylic acid test or heat test or dipstick; RBC's absent or 10 - 12/HPF; no macrohematuria or persistent hematuria, no pyuria. Fatty or waxy casts may be present.

In the first attack, hospitalize the patient to come to a complete diagnosis and to plan the treatment. It is important to do the blood tests for serum proteins - Total proteins and albumin (serum alb < 2.5g/dl); serum cholesterol ( > 200mg/dl) and BUN/S.creatinine (normal). Although steroid therapy is the backbone of therapy for nephrotic syndrome; oral prednisolone should be started after checking for infection, hypovolemia and secondary causes.

Check list prior to steroid therapy:

Presence of infection: Mantoux test, X-ray chest, CBC, urine culture, hepatitis B test. Steroids flare up the infections and in presence of infection steroids fail to give good response. Treatment of infection is advised before starting steroid therapy.

Severe edema with oliguria can be associated with hypovolemia (detected clinically by poor capillary fill and raised hematocrit and BUN), which requires IV Fluids before starting steroids; otherwise a nephrotic child can go into shock. Use of diuretics like furosemide is not advised in presence of hypovolemia or shock.

Presence of joint involvement, skin rash, anemia, and purpura are rare but when present demand attention to rule out systemic lupus erythematosus or Henoch Schonlein Purpura etc.

Steroid therapy for the initial or first attack should be started as follows:

Oral prednisolone 2mg/kg/day in 2 - 3 divided doses after food is given for 4 - 6 weeks daily. Urine should be tested for albumin and recorded in a diary every day till protein in urine reduces to nil or trace for 3 - 4 days. Steroid response is achieved in 10-15 days during which daily weight, B.P and urine output should be recorded. Decrease in weight (edema), increase in urine output with maintenance of normal BP is associated with a good response and child may be treated on OPD basis. After 4 weeks, the child may show moon facies or mild elevation of BP while on daily dose of steroids, hence weekly BP, weight, clinical examination along with urine test for proteinuria is essential till the course of steroids is completed.

After 4-6 weeks of daily oral prednisolone therapy, the dose is reduced to 1.5mg/kg as a single dose on alternate day or every 48 hours for 4-6 weeks. During the alternate day therapy, weekly weight, BP, urine examination (to ensure that proteinuria is controlled) should be continued.

If there are no side effects of steroid therapy like high BP, striae, Cushingoid facies, myopathy, infections, abdominal pain due to gastritis, reduced dose of oral prednisolone (1mg/kg) on alternate days is advised for 4 more weeks in the treatment of initial episode of nephrotic syndrome. Intensive therapy of the first attack is known to reduce the risk of future relapse of Nephrotic syndrome.