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Dr. Rai Muhammad Asghar
Associate Professor Pediatrics
Head of Pediatric Department
RMC Rawalpindi
NephroticNephrotic
SyndromeSyndrome
DEFINATIONDEFINATION
Massive Proteinuria (>40mg/mMassive Proteinuria (>40mg/m22
/hr)/hr)
Hypoalbuminemia (< 2.5 g/dl)Hypoalbuminemia (< 2.5 g/dl)
EdemaEdema
Hypercholesterolemia (>250mg/dl)Hypercholesterolemia (>250mg/dl)
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
EtiologyEtiology
Primary or Idiopathic-Primary or Idiopathic- 90%90%
1. Minimal change disease1. Minimal change disease 85%85%
2. Focal segmental glomerulosclerosis2. Focal segmental glomerulosclerosis 10%10%
3. Mesangial proliferative Glomerulonephritis 5%3. Mesangial proliferative Glomerulonephritis 5%
Secondary- 10%Secondary- 10%
a) Glomerulonephritisa) Glomerulonephritis
Membranous GlomerulonephritisMembranous Glomerulonephritis
Membranoproliferative GlomerulonephritisMembranoproliferative Glomerulonephritis
b) Systemic Diseasesb) Systemic Diseases
1. Systemic diseases1. Systemic diseases
Henoch Schonlein PurpuraHenoch Schonlein Purpura
SLESLE
Diabetes mellitusDiabetes mellitus
2. Infections2. Infections
Hepatitis B,& CHepatitis B,& C
Infective EndocarditisInfective Endocarditis
SyphilisSyphilis
MalariaMalaria
HIVHIV
3. Drugs-3. Drugs-
PenicillaminePenicillamine
Gold saltsGold salts
CaptoprilCaptopril
NSAID’sNSAID’s
4. Neoplasm's4. Neoplasm's
Hodgkin’s lymphomaHodgkin’s lymphoma
LeukemiaLeukemia
Wilms tumorWilms tumor
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
PathophysiologyPathophysiology
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%
SECONDARY NEPHROTIC SYNDROMESECONDARY NEPHROTIC SYNDROME
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
Clinical FeaturesClinical Features
Nephrotic syndrome.
Nephrotic syndrome.
Nephrotic syndrome.
Nephrotic syndrome.
Nephrotic syndrome.
Nephrotic syndrome.
Nephrotic syndrome.
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
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.
4.Renal Biopsy (Indications)4.Renal Biopsy (Indications)
Steroid Resistant Nephrotic SyndromeSteroid Resistant Nephrotic Syndrome
Frequent RelapsesFrequent Relapses
Steroids ToxicitySteroids Toxicity
Age at onset < 1or >8 yearsAge at onset < 1or >8 years
HypertensionHypertension
Gross HematuriaGross Hematuria
Low plasma C3Low plasma C3
Renal insufficiencyRenal insufficiency
Secondary Nephrotic SyndromeSecondary Nephrotic Syndrome
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.
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
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
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
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
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
4) Steroid Resistant Nephrotic Syndrome4) Steroid Resistant Nephrotic Syndrome
MethylprednisoloneMethylprednisolone
CyclophosphamideCyclophosphamide
ACE inhibitorsACE inhibitors
Angiotensin II BlockersAngiotensin II Blockers
ComplicationsComplications
1)Infections1)Infections
Spontaneous bacterial peritonitisSpontaneous bacterial peritonitis
PneumoniaPneumonia
UTIUTI
SepsisSepsis
CellulitesCellulites
2)Arterial and Venous Thrombosis2)Arterial and Venous Thrombosis
3) Others3) Others
Steroids and other drugs adverse effectsSteroids and other drugs adverse effects
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
33)) Steroid responsivenessSteroid responsiveness
90 % Minimal change disease90 % Minimal change disease
50 % Mesangial proliferation50 % Mesangial proliferation
20 % Focal Sclerosis20 % Focal Sclerosis
4) Poor prognostic factors are4) Poor prognostic factors are
HematuriaHematuria
HypertensionHypertension
HypocomplementemiaHypocomplementemia
Focal segmental sclerosisFocal segmental sclerosis
Steroid resistanceSteroid resistance
Thank YouThank You

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Nephrotic syndrome.

  • 1. Dr. Rai Muhammad Asghar Associate Professor Pediatrics Head of Pediatric Department RMC Rawalpindi
  • 3. DEFINATIONDEFINATION Massive Proteinuria (>40mg/mMassive Proteinuria (>40mg/m22 /hr)/hr) Hypoalbuminemia (< 2.5 g/dl)Hypoalbuminemia (< 2.5 g/dl) EdemaEdema Hypercholesterolemia (>250mg/dl)Hypercholesterolemia (>250mg/dl)
  • 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
  • 5. EtiologyEtiology Primary or Idiopathic-Primary or Idiopathic- 90%90% 1. Minimal change disease1. Minimal change disease 85%85% 2. Focal segmental glomerulosclerosis2. Focal segmental glomerulosclerosis 10%10% 3. Mesangial proliferative Glomerulonephritis 5%3. Mesangial proliferative Glomerulonephritis 5%
  • 6. Secondary- 10%Secondary- 10% a) Glomerulonephritisa) Glomerulonephritis Membranous GlomerulonephritisMembranous Glomerulonephritis Membranoproliferative GlomerulonephritisMembranoproliferative Glomerulonephritis b) Systemic Diseasesb) Systemic Diseases 1. Systemic diseases1. Systemic diseases Henoch Schonlein PurpuraHenoch Schonlein Purpura SLESLE Diabetes mellitusDiabetes mellitus
  • 7. 2. Infections2. Infections Hepatitis B,& CHepatitis B,& C Infective EndocarditisInfective Endocarditis SyphilisSyphilis MalariaMalaria HIVHIV 3. Drugs-3. Drugs- PenicillaminePenicillamine Gold saltsGold salts CaptoprilCaptopril NSAID’sNSAID’s 4. Neoplasm's4. Neoplasm's Hodgkin’s lymphomaHodgkin’s lymphoma LeukemiaLeukemia Wilms tumorWilms tumor
  • 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.
  • 23. 4.Renal Biopsy (Indications)4.Renal Biopsy (Indications) Steroid Resistant Nephrotic SyndromeSteroid Resistant Nephrotic Syndrome Frequent RelapsesFrequent Relapses Steroids ToxicitySteroids Toxicity Age at onset < 1or >8 yearsAge at onset < 1or >8 years HypertensionHypertension Gross HematuriaGross Hematuria Low plasma C3Low plasma C3 Renal insufficiencyRenal insufficiency Secondary Nephrotic SyndromeSecondary Nephrotic Syndrome
  • 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
  • 31. ComplicationsComplications 1)Infections1)Infections Spontaneous bacterial peritonitisSpontaneous bacterial peritonitis PneumoniaPneumonia UTIUTI SepsisSepsis CellulitesCellulites 2)Arterial and Venous Thrombosis2)Arterial and Venous Thrombosis 3) Others3) Others Steroids and other drugs adverse effectsSteroids and other drugs adverse effects
  • 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
  • 33. 33)) Steroid responsivenessSteroid responsiveness 90 % Minimal change disease90 % Minimal change disease 50 % Mesangial proliferation50 % Mesangial proliferation 20 % Focal Sclerosis20 % Focal Sclerosis 4) Poor prognostic factors are4) Poor prognostic factors are HematuriaHematuria HypertensionHypertension HypocomplementemiaHypocomplementemia Focal segmental sclerosisFocal segmental sclerosis Steroid resistanceSteroid resistance