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 â 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.
Refractory ascites
interfering with respiration or associated with breaks
in the skin may be removed by cautious paracentesisâŚalbumin c/iâŚ.hyperlipidemia need not be treated