Nephrotic syndrome- case definitons and treatment

different case definitions,evaluation and treatment of nephrotic syndrome for undergrads

1. - DR APOORVA POSTGRADUATE DEPT OF PEDIATRICS MANAGEMENT OF NEPHROTIC SYNDROME

2. 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 .

3. 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

4. EVALUATION The height , weight and blood pressure should be recorded b efore 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 .

5. 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.

6. 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.

7. INFREQUENT RELAPSERS Prednisolone is administered at a dose of 2 mg/kg/day (single or divided doses) until urine protein 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.

8. 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.

9. If the prednisolone threshold dose to maintain remission is high or if features of corticosteroid toxicity are seen,following immuno -modulators are added : 1.Levamisole 2.Cyclophosphamide 3.Calcineurin inhibitors – cyclosporin,tacrolimus 4.Mycophenolate mofetil

10. SUPPORTIVE CARE 1.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) is advised for those with persistent edema.

11. 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 .

12. 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.

13. 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.

14. 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.

15. 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.

16. 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.

17. CONCLUSION 1.Prompt,adequate treatment for 12 weeks 2.Monitor for steroid toxicity features 3.Prompt treatment of infections and complications

18. Thank you!