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 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.

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!