4. EpidemiologyEpidemiology
15 times more common in children than adults15 times more common in children than adults
Incidence is 2-3/ 100,000 children per yearIncidence is 2-3/ 100,000 children per year
8. PATHOPHYSIOLOGYPATHOPHYSIOLOGYPermeability of glomerular capillary membrane Proteinurea
Hepatic protein
synthesis including
lipoproteins.
Hyperlipidemia Transudatio
n of fluid
from
intravascular
compartment
to interstitial
space.
Plasma
oncotic
pressure.
Intravascular
volume
ADH Renal
perfusio
n
pressure
Water
reabsorption
in collecting
ducts
Activate renin angiotensin
aldosterone system
Tubular reabsorption of
sodium & water↑
Edema.
Hypoalbuminimia
10. IDIOPATHIC NEPHROTIC SYNDROMEIDIOPATHIC NEPHROTIC SYNDROME
MINIMAL CHANGE DISEASE FOCAL
SEGMENTAL
SCLEROSIS
AGE 2-6yrs 2-10yrs
SEX 2:1 male 1:3:1 male
HEMATURIA 10-20% 60-80%
HYPERTENSION 10% 20%
RENAL FAILURE No progression 10yrs
ASSOCIATIONS None None
SERUM CREATININ Inc. in 15-30% Inc. in 20-40%
IIMMUNOGENETICS HLA-B8, B12 None
LIGHT MICROSCOPY Normal Focal sclerosis
IMMUNOFLUORESCENCENegative IgM & C3 in lesions
ELECTRON MICRO Foot process fusion Foot process fusion
STEROID RESPONSE 90% 15-20%
12. Clinical FeaturesClinical Features
Periorbital PuffinessPeriorbital Puffiness
More marked is the morningMore marked is the morning
Edema later become generalizedEdema later become generalized
Scrotal EdemaScrotal Edema
Plural effusion and Ascites is the late featurePlural effusion and Ascites is the late feature
Decrease urine outputDecrease urine output
Hypertension and Hematuria are absentHypertension and Hematuria are absent
21. InvestigationsInvestigations
1. Urinalysis1. Urinalysis
Proteinuria 3+ or 4+Proteinuria 3+ or 4+
Urinary Protein excretion (>40mg/mUrinary Protein excretion (>40mg/m22
/hr)/hr)
Urinary Protein & Creatinine ratio > 3Urinary Protein & Creatinine ratio > 3
Microscopic Hematuria 10%Microscopic Hematuria 10%
Pus Cells :Pus Cells : Underlying UTIUnderlying UTI
Cellular Casts:Cellular Casts: not in minimal change disease, common in othernot in minimal change disease, common in other
formsforms
22. 2.Serum2.Serum
AlbuminAlbumin < 2.5 g/dl< 2.5 g/dl
CholesterolCholesterol >250mg/dl>250mg/dl
Normal C3Normal C3
Normal renal functionNormal renal function
3.Others3.Others
C.B.C. usually normal, ESR raisedC.B.C. usually normal, ESR raised
Mantoux test to rule out TBMantoux test to rule out TB
Chest X-Ray to rule out Pulmonary pathology or PleuralChest X-Ray to rule out Pulmonary pathology or Pleural
effusion.effusion.
24. Important DefinitionsImportant Definitions
RemissionRemission
Urine trace or negative for protein for 3 consecutive daysUrine trace or negative for protein for 3 consecutive days
Steroid resistantSteroid resistant
If the child continues to have Proteinuria (2 plus or more) on dailyIf the child continues to have Proteinuria (2 plus or more) on daily
steroid therapy after 8 wks.steroid therapy after 8 wks.
RelapseRelapse
Proteinuria 3-4 + with Oedema.Proteinuria 3-4 + with Oedema.
Steroid dependentSteroid dependent
Relapse while on alternate day therapy or within 28 days of stoppingRelapse while on alternate day therapy or within 28 days of stopping
Steroid therapy.Steroid therapy.
Frequent relapserFrequent relapser
Four or more relapses in 12 months.Four or more relapses in 12 months.
25. ManagementManagement
SupportiveSupportive
1) Hospitalization (Indications)1) Hospitalization (Indications)
InfectionInfection
Marked EdemaMarked Edema
2) Diet2) Diet
A balanced diet adequate in proteins and caloriesA balanced diet adequate in proteins and calories
Salt and fluid restriction when edemaSalt and fluid restriction when edema
3) Infection3) Infection
AntibioticsAntibiotics
26. 4) Diuretics4) Diuretics
Indications:Indications:
Pleural EffusionPleural Effusion
AscitesAscites
Severe Genital EdemaSevere Genital Edema
Treatment (Edema)Treatment (Edema)
Sodium restrictionSodium restriction
Fluid restrictionFluid restriction
DiureticsDiuretics
25 % Salt poor human albumin infusion25 % Salt poor human albumin infusion
27. SpecificSpecific
1)1) Steroids( Oral Prednisolone)Steroids( Oral Prednisolone)
60 mg/60 mg/mm22
/day for 4 weeksday for 4 weeks
40 mg/40 mg/mm22
/AD for 4 weeksAD for 4 weeks
WithdrawalWithdrawal
Gradual over next 2-3 monthsGradual over next 2-3 months
Dose decreased every 2 weeks by 15 mg/mDose decreased every 2 weeks by 15 mg/m22
28. 2)2) Treatment of steroid dependant and FrequentTreatment of steroid dependant and Frequent
relapsersrelapsers
6-12 months AD single dose6-12 months AD single dose
DoseDose School going 0.5 mg/kg ADSchool going 0.5 mg/kg AD
PreschoolPreschool 1 mg/kg/AD1 mg/kg/AD
29. 3) Alternative Therapy3) Alternative Therapy
IndicationsIndications
A) Relapse on Prednisolone dosage > 1 mg/kg ADA) Relapse on Prednisolone dosage > 1 mg/kg AD
OROR
B) Relapse on Prednisolone dosage > 0.5 mg/kg ADB) Relapse on Prednisolone dosage > 0.5 mg/kg AD
PlusPlus
Steroid Toxicity or Severe RelapseSteroid Toxicity or Severe Relapse
DrugsDrugs
CyclophosphamideCyclophosphamide
LevamisoleLevamisole
CyclosporinCyclosporin
ChlorambucilChlorambucil
30. 4) Steroid Resistant Nephrotic Syndrome4) Steroid Resistant Nephrotic Syndrome
MethylprednisoloneMethylprednisolone
CyclophosphamideCyclophosphamide
ACE inhibitorsACE inhibitors
Angiotensin II BlockersAngiotensin II Blockers
32. PROGNOSISPROGNOSIS
11)) Responders (78%)Responders (78%)
92 % Minimal Change92 % Minimal Change
8 % Others8 % Others
Non responders (22 %)Non responders (22 %)
25 % Minimal change25 % Minimal change
25%Focal Sclerosis25%Focal Sclerosis
25 % Mesangial Proliferation25 % Mesangial Proliferation
25 % Others25 % Others
22) Response Time) Response Time
10 % by the end of 110 % by the end of 1stst
WeekWeek
70 %by the end of 270 %by the end of 2ndnd
WeekWeek
85 %by the end of 385 %by the end of 3rdrd
WeekWeek
92 %by the end of 492 %by the end of 4thth
WeekWeek