-DR APOORVAPOSTGRADUATE

DEPT OF PEDIATRICS

MANAGEMENT OF NEPHROTIC SYNDROME

CASE DEFINITIONS RELATED TO NEPHROTIC SYNDROME

• Remission : Urine albumin nil or trace for 3 consecutive early morning specimens.

• Relapse : Urine albumin 3+ or 4+ (or proteinuria >40 mg/m2/h) for 3 consecutive early morning specimens,having been in remission previously.

• Frequent relapses : Two or more relapses in six months (or) four or more relapses in any twelve months.

• Steroid dependence : Two consecutive relapses when on alternate day steroids or within 14 days of its discontinuation.

• Steroid resistance : Absence of remission despite therapy with daily prednisolone at a dose of 2 mg/kg per day for 4 weeks

EVALUATION

• The height, weight and blood pressure should be recorded before starting treatment with corticosteroids.

• Regular weight record helps monitor the decrease or increase of edema.

• Physical examination is done to detect infections and underlying systemic disorders like SLE,HSP etc.

• Infections should be treated before starting therapy with corticosteroids.

INVESTIGATIONS• Urinalysis• CBP• Serum albumin• Serum cholesterol• Blood urea • Serum creatinine• Estimation of ASO titre and C3 levels is required in

patients with hematuria• Others : chest X-ray and tuberculin test, HBsAg, ANA

etc.

TREATMENT OF INITIAL EPISODE

• The standard medication for treatment is prednisolone or prednisone.

• Started at a dose of 2 mg/kg per day (maximum 60 mg) in single or divided doses for 6 weeks,

followed by 1.5 mg/kg (maximum 40 mg) as a single morning dose on

alternate days for the next 6 weeks.• Given after meal.

INFREQUENT RELAPSERS

• Prednisolone is administered at a dose of2 mg/kg/day (single or divided doses) until urineprotein is trace or nil for three consecutive days.

Followed by single morning dose of 1.5 mg/kg on alternate days for 4 weeks, and then discontinued.

FREQUENT RELAPSERS AND STEROID DEPENDENCE

• Pediatric nephrologist should be consulted.• The relapse is treated following which

prednisolone is gradually tapered to a dose of 0.5-0.7 mg/kg, administered for 9- 18 months.

• If the prednisolone threshold dose to maintain remission is high or if features of

corticosteroid toxicity are seen,following immuno-modulators are added :

1.Levamisole2.Cyclophosphamide3.Calcineurin inhibitors – cyclosporin,tacrolimus4.Mycophenolate mofetil

SUPPORTIVE CARE1.DIET : A balanced diet, adequate in protein (1.5-2

g/kg) and calories is recommended. • Patients with persistent proteinuria should receive

2-2.5 g/kg/day• Saturated fats to be avoided. • Reduction of salt intake (1-2 g per day) isadvised for those with persistent edema.

2.EDEMA :If edema is not responding to medication,a combination of a loop and thiazide diuretic, and/or a potassium sparing agent is started.

• If refractory edema,albumin (20%) is given as an infusion at a dose of 0.5-1 g/kg over

2-4 hrs, followed by administration of frusemide.

3.VACCINES : Patients receiving prednisolone at a dose of 2 mg/kg/day for more than 14 days are considered immunocompromised and therefore should not receive live attenuated vaccines.

• Inactivated or killed vaccines are safe.• Live vaccines are administered once the child is

off steroids for at least 4 weeks.• Optional vaccines against capsulated organisms

like PCV have to be given.

SPECIAL CASES

1.If the patient is exposed to a case of varicella,varicella zoster Ig should be given within 96hrs of exposure.

• Those who develop varicella should receive oral acyclovir (80 mg/kg/day in 4 doses) for 7-10 days.

• The dose of prednisolone should be tapered to 0.5 mg/kg/day or lower during the infection.

2. Patients with nephrotic syndrome who are Mantoux positive with no evidence of tuberculosis should receive INH prophylaxis for 6 mths.

• Those having active tuberculosis should receive standard therapy with anti tubercular drugs.

3.Patients with thrombotic complications require treatment with heparin (IV) or LMW heparin (subcutaneously), followed by oral anti-coagulants on the long-term.

4.Hypertension: Therapy is initiated with ACE inhibitors, calcium channel blockers.

5. Infections: Increased susceptibility to severe infections like peritonitis,cellulitis and pneumonia.Require prompt treatment with iv antibiotics for a period of 7-10 days.

6. Steroids during stress: require supplementation of steroids during surgery or serious infections as parenteral hydrocortisone at a dose of 2 mg/kg/day, followed by oral prednisolone at 1 mg/kg/day,given for the duration of stress and then tapered rapidly.

CONCLUSION

1.Prompt,adequate treatment for 12 weeks

2.Monitor for steroid toxicity features3.Prompt treatment of infections and

complications

Thank you!