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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)Hypoa...
EpidemiologyEpidemiology
 15 times more common in children than adults15 times more common in children than adults
 Inci...
EtiologyEtiology
Primary or Idiopathic-Primary or Idiopathic- 90%90%
1. Minimal change disease1. Minimal change disease 85...
Secondary- 10%Secondary- 10%
a) Glomerulonephritisa) Glomerulonephritis
Membranous GlomerulonephritisMembranous Glomerulon...
2. Infections2. Infections
Hepatitis B,& CHepatitis B,& C
Infective EndocarditisInfective Endocarditis
SyphilisSyphilis
Ma...
PATHOPHYSIOLOGYPATHOPHYSIOLOGYPermeability of glomerular capillary membrane Proteinurea
Hepatic protein
synthesis includin...
PathophysiologyPathophysiology
IDIOPATHIC NEPHROTIC SYNDROMEIDIOPATHIC NEPHROTIC SYNDROME
MINIMAL CHANGE DISEASE FOCAL
SEGMENTAL
SCLEROSIS
AGE 2-6yrs 2-1...
SECONDARY NEPHROTIC SYNDROMESECONDARY NEPHROTIC SYNDROME
Clinical FeaturesClinical Features
Periorbital PuffinessPeriorbital Puffiness
More marked is the morningMore marked is the...
Clinical FeaturesClinical Features
InvestigationsInvestigations
1. Urinalysis1. Urinalysis
Proteinuria 3+ or 4+Proteinuria 3+ or 4+
Urinary Protein excretion...
2.Serum2.Serum
AlbuminAlbumin < 2.5 g/dl< 2.5 g/dl
CholesterolCholesterol >250mg/dl>250mg/dl
Normal C3Normal C3
Normal ren...
4.Renal Biopsy (Indications)4.Renal Biopsy (Indications)
Steroid Resistant Nephrotic SyndromeSteroid Resistant Nephrotic S...
Important DefinitionsImportant Definitions
RemissionRemission
Urine trace or negative for protein for 3 consecutive daysUr...
ManagementManagement
SupportiveSupportive
1) Hospitalization (Indications)1) Hospitalization (Indications)
InfectionInfect...
4) Diuretics4) Diuretics
Indications:Indications:
Pleural EffusionPleural Effusion
AscitesAscites
Severe Genital EdemaSeve...
SpecificSpecific
1)1) Steroids( Oral Prednisolone)Steroids( Oral Prednisolone)
60 mg/60 mg/mm22
/day for 4 weeksday for 4 ...
2)2) Treatment of steroid dependant and FrequentTreatment of steroid dependant and Frequent
relapsersrelapsers
6-12 months...
3) Alternative Therapy3) Alternative Therapy
IndicationsIndications
A) Relapse on Prednisolone dosage > 1 mg/kg ADA) Relap...
4) Steroid Resistant Nephrotic Syndrome4) Steroid Resistant Nephrotic Syndrome
MethylprednisoloneMethylprednisolone
Cyclop...
ComplicationsComplications
1)Infections1)Infections
Spontaneous bacterial peritonitisSpontaneous bacterial peritonitis
Pne...
PROGNOSISPROGNOSIS
11)) Responders (78%)Responders (78%)
92 % Minimal Change92 % Minimal Change
8 % Others8 % Others
Non r...
33)) Steroid responsivenessSteroid responsiveness
90 % Minimal change disease90 % Minimal change disease
50 % Mesangial pr...
Thank YouThank You
Nephrotic syndrome.
Nephrotic syndrome.
Nephrotic syndrome.
Nephrotic syndrome.
Nephrotic syndrome.
Nephrotic syndrome.
Nephrotic syndrome.
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Nephrotic syndrome.

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Rawalpindi Medical College

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Nephrotic syndrome.

  1. 1. Dr. Rai Muhammad Asghar Associate Professor Pediatrics Head of Pediatric Department RMC Rawalpindi
  2. 2. NephroticNephrotic SyndromeSyndrome
  3. 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. 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. 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. 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. 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. 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
  9. 9. PathophysiologyPathophysiology
  10. 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%
  11. 11. SECONDARY NEPHROTIC SYNDROMESECONDARY NEPHROTIC SYNDROME
  12. 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
  13. 13. Clinical FeaturesClinical Features
  14. 14. 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
  15. 15. 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.
  16. 16. 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
  17. 17. 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.
  18. 18. 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
  19. 19. 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
  20. 20. 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
  21. 21. 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
  22. 22. 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
  23. 23. 4) Steroid Resistant Nephrotic Syndrome4) Steroid Resistant Nephrotic Syndrome MethylprednisoloneMethylprednisolone CyclophosphamideCyclophosphamide ACE inhibitorsACE inhibitors Angiotensin II BlockersAngiotensin II Blockers
  24. 24. 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
  25. 25. 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
  26. 26. 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
  27. 27. Thank YouThank You

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