Nephrotic Syndrome (Nephrosis) Characteristics : Proteinuria ( urine protein loss > 2 gm/day ) Hypo-proteinemia ( serum albumin < 2.5 gm/dL ) Edema Hyperlipidemia Etiology : Idiopathic nephrotic syndrome (90 %) Minimal-change disease (85 %) Focal sclerosis (10 %)  End-stage renal failure Mesangial proliferation ( 5 %) Glomerulonephphritis; Membranous nephropathy & Membranoproliferative glomerulonephritis (10 %)

Nephrotic Syndrome (Nephrosis) Pathology : Idiopathic nephrotic syndrome (90 %) Minimal-change disease (85 %) miminal increase in Mesangial cells & matrix 95 % : respond to corticosteroid Focal sclerosis (progressive) (10 %)  End-stage renal failure normal or mesangial proliferation juxta-medullary segmental scarring 20 % : respond to corticosteroid Mesangial proliferation ( 5 %) diffuse increase in Mesangial cells & matrix mesangial IgM & C3 deposits 50~60 % : respond to corticosteroid

Nephrotic Syndrome (Nephrosis) Clinical manifestations : Idiopathic nephrotic syndrome (90 %) Boys : girls = 2 : 1 peak age = 2 ~ 6 yr initial episode & subsequent relapses : follow an apparent Viral URI Edema : eyes, lower extremities “ pitting ” Weight gain Ascites or Pleural effusions (dependent sites) Declining urine output Anorexia Abdominal pain Diarrhea

Nephrotic Syndrome (Nephrosis) Diagnosis : Urinalysis = 3+ ~ 4+ proteinuria Microscopic hematuria Gross hematuria (rare) low Ccr Urine Protein loss > 2 gm/day Serum albumin < 2.5 gm/dL decreased Total serum Ca Normal C3 *

Nephrotic Syndrome (Nephrosis) Complications : Infections Spontaneous peritonitis (Streptococcus ; GNB ) * Polyvalent pneumococcal vaccine Sepsis Pneumonia Cellulitis Urinary tract infections Arterial & Venous thrombosis * inhibitors of frinolysis * decreased plasma Anti-thrombin III * increased PLT aggregation * deficiency of coagulation factors IX, XI & XII

Nephrotic Syndrome (Nephrosis) Treatments : Divided-dose therapy time needed for response to prednisolone : 2 wk * response : urine becames free of protein * relapse : recurrence of edema, not simply of proteinuria Intermittent proteinuria that resolves spontaneously Alternate-day therapy 5 days after the urine becomes free of protein continued 3~6 mo

Nephrotic Syndrome (Nephrosis) Treatments : * Steroid-resistant : Proteinuria > 2+, after 1 mo of continuous daily divided-dose prednisolone * Steroid-dependent : respond to daily divided-dose therapy, but having relapses shortly after switching to or after terminating alternate-day therapy

Nephrotic Syndrome (Nephrosis) Treatments : Corticosteroid toxicity : Cushingoid appearance Hypertension Growth failure Immunosuppressive drugs : ( WBC > 5000/mm3 ) Prolong the duration of remission prevent relapses in children with frequency relapsing nephrotic syndrome * Steroid-resistent group: Pulse methylprednisolone Immunosuppressive drugs

Nephrotic Syndrome (Nephrosis) Treatments : Most children with steroid-responsive nephrosis have repeated relapses till the disease resolves spontaneously toward the end of the 2 nd decade of life *