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