Nephrotic Syndrome

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Nephrotic Syndrome. Etiology Idiopathic nephrotic syndrome (90%) Minimal change disease (85%) Focal segmental glomerulosclerosis (10%) Mesangial proliferation (5%) Glomerulonephritis; Membranous nephropathy; and membranoproliferative glomerulonephritis (10%). - PowerPoint PPT Presentation

Text of Nephrotic Syndrome

Prognosis

Nephrotic SyndromeEtiologyIdiopathic nephrotic syndrome (90%)Minimal change disease (85%)Focal segmental glomerulosclerosis (10%)Mesangial proliferation (5%)Glomerulonephritis; Membranous nephropathy; and membranoproliferative glomerulonephritis (10%)1Idiopathic Nephrotic SyndromeApproximately 90% of children with nephrotic syndrome have idiopathic nephrotic syndromeMale:female ratio (2:1) Peak incidence: 2 6 yrThe initial episode and subsequent relapses may follow minor infections and, occasionally, reactions to insect bites, bee stings, or poison ivy.more common in males than in females (2:1) and most commonly appears between the ages of 2 and 6 yr.

2Idiopathic Nephrotic SyndromeMinimal Change DiseaseMesangial ProliferationFocal segmental glomerulosclerosisLight MicroscopyNormal or minimal increase in mesangial cells and matrix

Diffuse increase in mesangial cells and matrix

Mesangial proliferation and segmental scarring

Idiopathic Nephrotic SyndromeMinimal Change DiseaseMesangial ProliferationFocal segmental glomerulosclerosisImmunofluorescenceNegative

Trace to 1+ mesangial IgM and/or IgA staining

IgM and C3 staining in the areas of segmental sclerosisIdiopathic Nephrotic SyndromeMinimal Change DiseaseMesangial ProliferationFocal segmental glomerulosclerosisElectron MicroscopyEffacement of epithelial foot processes

Increased numbers of mesangial cells and matrix; effacement of the epithelial cell foot processesSegmental scarring of the glomerular tuft with obliteration of the glomerular capillary lumenIdiopathic Nephrotic SyndromeMinimal Change DiseaseMesangial ProliferationFocal segmental glomerulosclerosisSteroid response>95%

50%20%Focal segmental glomerulosclerosis The disease is frequently progressive, ultimately involving all glomeruli, and leads to end-stage renal failure in most patients.6CharacteristicsProteinuria>3.5 g/24 hr in adults>40 mg/m2/hr in childrenSpot urine protein to creatinine ratio >2.0Hypoalbuminemia