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NEPHROTIC SYNDROME

NEPHROTIC SYNDROME. AETIOLOGY,DIAGNOSIS AND MANAGEMENT. INTRODUCTION. NS is a syndrome comprising of heavy proteinuria, (>3g/24 hrs), hypoalbuminemia(alb <30g/L) and oedema.Hypercholesterol other features: Lipiduria, ↑renal catabolism, hypercoagulability.

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NEPHROTIC SYNDROME

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  1. NEPHROTIC SYNDROME AETIOLOGY,DIAGNOSIS AND MANAGEMENT

  2. INTRODUCTION. • NS is a syndrome comprising of heavy proteinuria, (>3g/24 hrs), hypoalbuminemia(alb <30g/L) and oedema.Hypercholesterol other features: Lipiduria, ↑renal catabolism,hypercoagulability. • Key components: Proteinuria. Results from altered permeability of the GF barriers for protein ie GBM, podocytes and their slit diaphragm.

  3. Contd. • Underfilling hypothesis. ↓ protein →↓ oncotic P0 → leakage of fluid to interstitium → ↓intravascular vol. → stimulation of RAAS, synthesis and release of vasopressin (ADH), supp. of atria natriuretic peptide release. All lead to restoration of IVS → further leakage.

  4. AETIOLOGY • Primary NS. • Secondary NS. • Primary : • Minimal change glomerular DZ. 20%. No progression to chronic renal failure. • Focal segmental glomerulosclerosis.15 – 25% • Membranous Glomerulopathy 30 - 40 %. Peak 30 – 50 yrs. • Membranoproliperative GN. 20 – 30 %. • Fibrillary – immunotactoid 1%. • Mesangial proliferative GN. 5 – 10%

  5. Contd. • Secondary NS. • DM • Sickle cell nephropathy. • Amyloidosis. MM. • Systemic vasculitides – SLE, HSP, GPD. • Drugs: Penicillamine, Captopril, Probenecid trimethadione, mercury, chlormethiazole. • Allergies. Bee stings, hays, etc. • Malignancies. Malignant lymphoproliferative disorders

  6. DIAGNOSIS. • History. • Exposure to drugs, allergies, family hx. Atopy. • Acute or chronic infections. • Frothy urine. • Systemic symptoms. Fever, wt. loss.

  7. Contd. • Examination. • Oedema: leg, arm, face. • Ascites, hepatomegaly, pleural effussion. • ↑ BP in some. • Xanthelasma, Xanthomata. • Features of underlying disorders – SLE, HSP,DM, SCD, malignancies.

  8. Contd. • Investigations. • Urine. • Dipstick (Haematuria, proteinuria) • Microscopy (RBCs, Casts), culture and sensitivity • 24 hr. urine collection. • Urine electrophoresis • Blood. • FBC + ESR or CRP • SEUC • LFT

  9. contd • Lipid profile • HBsAg, HCV • FBS + 2HPP • ASO titres • Throat swab • Serum C3 complement • Selective protein clearance; IgG/ alb or trans • Serum protein

  10. Contd. • Serum electrophoresis. • Immunoglobulins. • Serum ANA, ANCA, anti GBM antibodies. • Cryoglobulinaemia. • Immaging. • CXR • Renal ultrasound. • Renal biopsy.

  11. TREATMENT • Monitor SEUC, BP, Fluid balance, wt. • Fluid restriction (1 – 1.5 L/day.) • Salt restriction 5gm/day. • Normal protein approx. 1g/24hrs of HBV. • Diuretics – frusemide, thiazides, spironolactone ± salt poor alb. • Treatment for HBP • Treat proteinuria • Treat infxn. Prophylaxis. • Prophylactic heparin, warfarin, ± streptokinase.

  12. Contd. • Corticosteroids • Immunomodulators.

  13. COMPLICATIONS • Hyperlipidaemia • Hypercoagulability: antithrombin III, protein s and c, hyperfibrinogenaemia ( ↑ hepatic synthesis), impaired fibrinolysis, ↑ platelet aggregation. • Renal vein thrombosis.(memb. Memb.pro.and amyloidosis. • ARF • Hypocalcaemia & 20 hyperparathyroidism: Loss of cholecalciferol binding protein → Vit D Deff. • Infections. IgG loss. • Others : ↓ thyroxine, protein mal., Fe. Rest. anae

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