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Nephrotic Syndrome
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Syndromes in NephrologySyndromes in Nephrology
Acute nephritisNephrotic syndrome
Asymptomatic urinary abnormalitiesAcute renal failure
Chronic kidney diseaseUrinary tract infection
Urinary tract obstructionNephrolithiasisHypertension
Renal tubular defects
Cross-Section of the KidneyCross-Section of the Kidney
R E N A L A N A E M I AR E N A L A N A E M I A
Renal VeinRenal VeinRenal ArteryRenal ArteryRenal PelvisRenal Pelvis
UreterUreter
Renal MedullaRenal MedullaPapillaPapilla
Renal CortexRenal Cortex
Branch of theBranch of theRenal VeinRenal Vein
Branch of theBranch of theRenal ArteryRenal Artery
NephronNephron
Manifold Tasks of the KidneyManifold Tasks of the Kidney
R E N A L A N A E M I AR E N A L A N A E M I A
Bone StructureBone StructureBone StructureBone Structure
Vitamin DVitamin DActivationActivation
CalciumCalciumBalanceBalance
Blood FormationBlood FormationBlood FormationBlood Formation
ErythropoietinErythropoietinSynthesisSynthesis
Cardiac ActivityCardiac ActivityCardiac ActivityCardiac Activity
PotassiumPotassiumBalanceBalance
Regulation of Blood pHRegulation of Blood pHRegulation of Blood pHRegulation of Blood pH
Recovery ofRecovery ofBicarbonateBicarbonate
Blood PressureBlood PressureBlood PressureBlood Pressure
Water BalanceWater Balance
SodiumSodiumRemovalRemoval
MetabolicMetabolicEnd ProductsEnd Products
MetabolicMetabolicEnd ProductsEnd Products
Removal ofRemoval of Urea, Creatinine etc. Urea, Creatinine etc.
FunctionsFunctions
NEPHROTIC SYNDROMENEPHROTIC SYNDROME
SYAKIB BAKRI, HASYIM KASIM, HAERANI RASYID
*Division of Nephrology, Department of Internal Medicine Faculty of Medicine, Hasanuddin University
GGlomerulopatlomerulopathhiesies
Glomerulopathy : a group of diverse conditions – including, but not limited Glomerulopathy : a group of diverse conditions – including, but not limited
to, glomerulonephritis – having in common the fact that the disease to, glomerulonephritis – having in common the fact that the disease
process begins in the glomerulus or that the glomerulus is the most process begins in the glomerulus or that the glomerulus is the most
importantly diseased part of the nephron.importantly diseased part of the nephron.
Glomerulopathies are the most common causes of end-stage renal diseaseGlomerulopathies are the most common causes of end-stage renal disease
Clinical presentation of glomerular diseaseClinical presentation of glomerular disease
I.I. Acute glomerulonephritisAcute glomerulonephritis
II.II. Rapidly progressive glomerulonephritisRapidly progressive glomerulonephritis
III.III. Chronic glomerulonephritisChronic glomerulonephritis
IV.IV. Persistent urinary abnormalities with few or no symptomsPersistent urinary abnormalities with few or no symptoms
V.V. Nephrotic syndromeNephrotic syndrome
Nephrotic syndromeNephrotic syndrome
• Clinical entity having miltiple causes and characterizedClinical entity having miltiple causes and characterized
by increased glomerular permeability manifested by increased glomerular permeability manifested
by massive proteinuria and lipiduria.by massive proteinuria and lipiduria.
• Massive proteinuria > 3.5 g/day/1.73mMassive proteinuria > 3.5 g/day/1.73m22 body surface area body surface area
in the absence of a depressed GFR.in the absence of a depressed GFR.
Clinical Features of The Nephrotic SyndromeClinical Features of The Nephrotic Syndrome
Manifestations of the nephrotic syndrome itselfManifestations of the nephrotic syndrome itself
Signs and symptoms determined by the underlying disease Signs and symptoms determined by the underlying disease involving the kidneyinvolving the kidney
Clinical manifestation of nephrotic syndrome :Clinical manifestation of nephrotic syndrome :
OedemaOedema
HypertensionHypertension
DyslipidemiaDyslipidemia
Hypercoagulable stateHypercoagulable state
Hypoproteinemia / proteinuriaHypoproteinemia / proteinuria
Progressive renal failureProgressive renal failure
Trace metal deficienciesTrace metal deficiencies
Endocrine disturbancesEndocrine disturbances
Infectious / immunodeficiency statesInfectious / immunodeficiency states
Pathophysiology of the Nephrotic SyndromePathophysiology of the Nephrotic Syndrome
Classification of the disease states associated with the development of Classification of the disease states associated with the development of nephrotic syndromenephrotic syndrome
I. Idiopathic nephrotic syndrome due to Primary Glomerular DiseaseI. Idiopathic nephrotic syndrome due to Primary Glomerular Disease
II.Nephrotic syndrome associated with spesific etiologic events or in which II.Nephrotic syndrome associated with spesific etiologic events or in which glomerular disease arises as a complication of other diseaseglomerular disease arises as a complication of other disease
1.1. MedicationsMedications
2.2. AllergensAllergens
3.3. Infection ( bacterial, viral, protozoal, helminthic )Infection ( bacterial, viral, protozoal, helminthic )
4.4. Neoplasmic ( solid tumors, leukemia and lymphoma )Neoplasmic ( solid tumors, leukemia and lymphoma )
5.5. Multisystem diseaseMultisystem disease
6.6. Heredofamilial and metabolic diseaseHeredofamilial and metabolic disease
7.7. MiscellaneousMiscellaneous
Diagnostic approach in nephrotic syndromeDiagnostic approach in nephrotic syndrome
I.I. ClinicalClinical
II.II. Laboratory studiesLaboratory studies
III.III. Renal biopsyRenal biopsy
I. ClinicalI. Clinical
HistoryHistoryPreexisting diseasePreexisting diseasePrevious infectionPrevious infectionDrug ingestionDrug ingestionArthritis, rashArthritis, rashCurrent pregnancyCurrent pregnancyFamily history of renal diseaseFamily history of renal disease
Physical examinationPhysical examinationSevere obesitySevere obesityRash, arthritisRash, arthritisDiabetic retinopathyDiabetic retinopathyHypertensionHypertensionEvidence of malignancyEvidence of malignancyLipodystrophyLipodystrophyLymphoadenopathy/hepatosplenomegalyLymphoadenopathy/hepatosplenomegaly
II. Laboratory StudiesII. Laboratory Studies
Urinalysis
In all cases ( nondiagnstic )Creatinine clearanceSerum protein electrophoresisSerum tota;cholesterol, lipoproteinSerum ionized calciumParathyroid hormone
In selected cases ( to establis the diagnosis )Complement levelAntinuclear antibody assay CryoglobulinsHepatitis and HIV serologySerum and urine immunoelectrophoresis
III. Renal biopsyIII. Renal biopsy
• Minimal change diseaseMinimal change disease• Focal segmental glomerulosclerosisFocal segmental glomerulosclerosis• Membranous nephropathyMembranous nephropathy• Membranoproliferative glomerulonephritisMembranoproliferative glomerulonephritis• Other glomerulonephritisOther glomerulonephritis
Suggested approach for initial treatmentSuggested approach for initial treatment( Minimal change disease )( Minimal change disease )
ChildrenChildrenPrednisone 60 mg/mPrednisone 60 mg/m22/day until remission, then 40 mg/m/day until remission, then 40 mg/m22/48 h for /48 h for 12 weeks, then reduce by 5-10 mg/m12 weeks, then reduce by 5-10 mg/m22/48 h every month./48 h every month.
AdultsAdultsPrednisone 1mg/kg/day until remission or for 6 weeks, then 1.6 mg/kg/48 hPrednisone 1mg/kg/day until remission or for 6 weeks, then 1.6 mg/kg/48 hfor 1 month, then reduce by 0.2-0.4 mg/kg/48 h.for 1 month, then reduce by 0.2-0.4 mg/kg/48 h.
ElderlyElderlyPrednisone 1 mg/kg/day until remission or for 4 weeks, then 0.8 mg/kg/day Prednisone 1 mg/kg/day until remission or for 4 weeks, then 0.8 mg/kg/day for 2 weeks, then 1.6 mg/kg/48 h for 2 weeks. Then reduce by 0.4 mg/kg/48 hfor 2 weeks, then 1.6 mg/kg/48 h for 2 weeks. Then reduce by 0.4 mg/kg/48 hevery 2 weeks. If no remission continue with 1.2 mg/kg/48 h for another every 2 weeks. If no remission continue with 1.2 mg/kg/48 h for another 4 weeks then reduce.4 weeks then reduce.
Contraindications to prednisoneContraindications to prednisoneCyclophosphamide 2 mg/kg/day or chlorambucil 0.15mg/kg/day for 8-12 Cyclophosphamide 2 mg/kg/day or chlorambucil 0.15mg/kg/day for 8-12 weeksweeks
T H A N K T H A N K Y O UY O U