Nephrotic Syndrome Definition : a clinic-laboratory condition characterized by: Massive proteinuria Hypoalbuminemia Generalized edema Hyperlipidemia high cholesterol

terol and TG (not necessarily present)

Etiology Primary/Idiopathic 90% o Most common histological types MCNS (85%) FSGS (10%) Mesangeal proliferative (5%) o Causes Unknown (the name is idiopathic, of course unkown cause..duh!!) In MCNS there usually is Genetic predisposition Autoimmune Secondary NS o Any causes of glomerulonephritis w/ heavy proteinuria Systemic lupus nephritis Henoch-schonlein purpura Infection HBV, HCV, shistosomiasis o Drugs D penicillamine & heavy metals (eg: gold) Phenytoin & procainamide o Tumor Hodgkin lymphoma

Idiopathic Nephrotic Syndrome Pathogenesis Proteinuria o Intrinsic factor 1ry glomerular defect (fusion & effacement of foot process & pseudovillus formation) o Extrinsic factor Circulating permeability factor A soluble factor is thought to increase the permeability of albumin The nature of this factor is unknown Immunological basis

Hypoalbuminemia o Proteinuria o Excess catabolism Edema o Underfill theory Proteinuria & hypoalbuminemia causes Decreased oncotic pressure translocation of plasma water into the interstitial space edema Decreased intravascular volume activation of the RAS salt and water retention edema o Overfill theory There is a primary defect in the distal tubules It causes sodium retention edema Hyperlipidemia o Hypoproteinemia hepatic protein synthesis (including lipoprotein) o Excess loss of lipoprotein lipase through urine

C/p: In MCNS peak age = 2-8 y.o. and boys : girls = 2:1 Generalized edema o Starts as morning periorbital puffiness then progress towards the lower extremities o Soft and pitting edema o May be complicated w/ pleural effusion & ascites Mild hypertension compensatory mech. Towards hypovolemia Urine changes o Olguria o Hematuria usu. Microscopic, gross hematuria only 3% o Frothy urine May be asymptomatic Presentation of complications

Investigation Urine exam o Heavy proteinuria - >40mg/m2/hour o Hematuria o Hyaline cast Blood test o Low s. Albumin - <2.5g/dl o High cholesterol and LDL o S. Creatinine usu. Normal o C3 (normal in 1ry NS but low in some causes of 2ry)

Renal biopsy indicated in o Abn. Presentation Age : <1 or >10 Low C3 Gross hematuria Persistent HTN Presentation of systemic disease Impaired kidney function (kidney failure) o Steroid resistant o Steroid dependant or frequent relapse o 2ry NS

Complication Short term o Infection (capsulated bacteria & viruses) o IV thrombosis o AKI o Anemia Long term o Growth failure o Cataracts o CVS

Management General measures o Diet Salt restriction Fat restriction o Activity that is tolerable o Diuretics with albumin supplements only indicated in Severe edema Infection o Prevention of complication Specific measure o Corticosteroids (prednisone)