This lecture was conducted during the Nephrology Unit Grand Ground by Medical Student under Nephrology Division, Department of Medicine in King Saud University. Nephrology Division is not responsible for the content of the presentation for it is intended for learning and /or education purpose only.

Nephrotic Syndrome Presented by: Hawra AL-Muslim

Nephrotic syndrome is a nonspecific disorder in which the kidney are damaged, causing them to leak large amounts of protein from the blood into the urine.

Proteinurea oedema hypoalbuminaemia

1- Proteinurea: >3.5g/24h Or a spot urine protein:creatinine ratio of > mg/mmol 2- Hypoalbuminaemia: albumin <25g/L 3- Oedema Severe hyperlipidemia is often present: total cholesterol >10mmol/L

Pathophysiology Structural damage to the glomerular basement membrane leads to loss of electrostatic and physical barriers, which normally prevent the passage of large molecular weight proteins into the glomerular filtrate. proteinurea

Increased protein loss increased catabolism of protein in the kidney hypoalbuminaemia.

Salt & water moves into the extravascular compartment renin-angiotensin system plasma oncotic pressure Oedema(poorly understood) Hypoalbuminaemia Na & water reabsorption in distal nephron.

Causes Primary: 1/Minimal change disease children 2/Focal segmental glomerulosclerosis 3/Membranous nephropathy adult

Secondary: SLE DM Infections (HIV, hepatitis B, hepatitis C, bacterial,…) Drugs (captopril, heroin, heavy metals, NSAIDs, penicillamine,gold) malignancy

Signs & symptoms 1/ Puffiness around the eye, pitting edema in the leg, pleural effusion, ascites, generalized edema (anasarca) >> weight gain

2/ (mostly) normal BP, but may be hypertention. 3/ Anemia (transferrin loss) 4/ dyspnea (Pleural Effusion or Ascites) 5/ features of the underlying cause ( rash in SLE, neuropathy in DM).

complications Increase susceptibility to infection (20%): loss of immunoglobulin in urine & to immunosuppressive treatments. Thromboembolism (40%): clotting factors & platelet abnormalities, loss of certain anticoagulants in the urine >> DVT/PE, renal vein thrombosis. Kidney failure: SOB, weakness, easy fatigability(anemia) & loss of appetite.

Diagnosis Urine test: Urinalysis : urine dipstick (protein 3+,4+) microscopic examination of urine sediment (fatty casts) 24 hour urinary total protein estimation (gold standard)

Blood tests Serum albumin Lipid profile ( high LDL ) Electrolytes, urea & creatinine FBS & HA1c>>DM Serologic studies Antinuclear antibody >> SLE,RA tests for hepatitis B and C, HIV Kidney biopsy (if the cause is not clear)

Treatment Symptomatic Rx: Na intake <3g/day, fluid intake 1.5 L/day Diuretics ( furosemide mg/24h ± metolazone or spironolactone ) >> edema ACE-I>> proteinurea Low protein intake Treat any underlying cause or complication Monitor U&E, BP, fluid balance and weight (0.5-1 kg/day loss) regularly.

Primary causes Rx: Minimal change glomerulonephritis: Steroids Cyclophosphamide or ciclosporin>> relapses, steroid SE/dependence Prognosis excellent, 1% ESRF

Membranous nephropathy: Steroids+ Cyclophosphamide or chlorambucil Prognosis fair to good 40% spontaneous remission, RF 33%

Focal segmental glomerulosclerosis: Corticosteroids 30% of cases Cylophosphamide or ciclosporin >> steroid resistant Prognosis fair to poor, 30-50%ESRD