Nephrotic Syndrome(NS),站,Definition NS is an accumulation of symptoms and signs and is characterized by proteinuria, hypoproteinemia, edema, and hyperlipidemia.,The vast majority patients (90% of cases) with NS of childhood are primary.,Definition In children under age 5 years the disease usually takes the form of idiopathic (primary) NS of childhood.,Conditions Of Attack Second only to acute nephri- tis Incidence age: at all ages, but most commonly between 35 years of age,Type 1. Clinical type Simple type NS ; Nephritic NS 2. Response to steroid therapy (P274),Type The initial response to cortico- steroids is a guide to prognosis Steroid-dependent NS Steroid-responsive NS Steroid-resistant NS,Type Total effect Partial effect Non-effect,Type 3. Pathologic type (P282) Minimal change disease, MCD 80% of all NS, little change on light microscopy,Type but electron microscopy shows effacement of foot processes (podocytes) of epithelial cells,Type Other forms (Non-MCNS) MSPGN, FSGN, MGN, MPGN, etc.,Pathogenesis The primary disorder is an increase in glomerular permeability to plasma proteinsloss of proteins, mainly albumin in urine,Pathogenesis Filtrated barrier Charge barrier Molecular barrier The loss of the negative charges on the GBM,Pathogenesis The underlying pathogenesis is unknown, but evidence strongly supports the impor- tance of immune mechanisms (P281),Pathophysiology 1. Proteinuria: fundamental and highly important change of pathophysiology,Pathophysiology 2. Hypoproteinemia: mainly albumin 3. Edema: nephrotic edema (pitting edema),Pathophysiology Hypoproteinemiaplasma oncotic pressure, result in a shift of fluid from intervas- cular to extravascular com- partment edema,Pathophysiology Plasma volumeactivates the reninangiotensinaldo- sterone system, also ADH secretion Na and water reabsorption,Pathophysiology 4. Hyperlipidemia (Hyper- cholesterolemia): Ch,TC, LDL-ch, VLDL-ch,Pathophysiology Caused by: 1. Hypoproteinemia stimulates liver protein synthesis including lipoproteins; 2. Lipid catabolism(lipoprotein lipases lost in urine?),Clinical Manifestations Peak age: 25 years Boys:girls = 3.7:1 1. Main manifestations Edema (varying degrees) is the most common symptom, +/- weight gain,Clinical Manifestations Edema of periorbital /face, pitting edema in lower limbs, perineum anasarca evident Perhaps oliguria are noticed Ascites, pleural effusion Frothy urine,Clinical Manifestations 2. General symptoms: pallid, anorexia, fatigue, diarrhea, abdominal pain,Laboratory Exam 1. Urinary protein: 2+4+ 24 h total urinary protein 50 mg/kg/d or 0.1g/m2/d ( the most are selective proteinuria ),Laboratory Exam UP/Ucr(mg/mg)3.5 (normal 0.2) May occur RBC (15%), granular and red cell casts,Laboratory Exam 2. Total serum protein, 30 g/L Albumin levels are low , often25 g/L,Laboratory Exam 3. Serum lipids cholesterol(CH) 5.7mmol/L triglycerides(TC) LDL, VLDL,Laboratory Exam 4. ESR100 mm/h 5. Serum protein electrophoresis Albumen, 2-G,-G, A/G inversion 6. Serum Ca+ ,Laboratory Exam 7. Serum complement: vary with clinical type 8. Renal function BUN & serum Cr,Complications 1. Infections Acute infection is a major complication in children with NS. It frequently trigger relapses,Complications Often precipitated by viral infections Site: respiratory tract(URI), skin, urinary tract and acute primary peritonitis,Complications Due to: immunity lower (urinary loss of IgG, etc.) severe edemamalcirculation protein malnutrition use steroid therapy,Complications 2. Electrolyte disturbances 2.1. Hyponatremia 2.2. Hypokalemia 2.3. Hypocalcemia,站,Complications 3. Thromboembolic phenomena ( Hypercoagulability ): Potential arterial & venous thrombosis, e.g. renal vein thrombosis,Complications Due to: urinary loss of antithrombin, hepatic fibrinogen synthesis, platelet aggregationetc.,Complications 4. Hypovolemia (Hypovolemic shook) 5. Acute renal failure 6. Stunting,Diagnosis 1. Diagnostic standard (P273) Four characteristics Excluding other renal disease (second NS),Diagnosis 2. Clinical type: Simple type NS Nephritic type NS,Management 1. General measures 1.1. Rest 1.2. Diet Hypertension and edema: low salt diet (2 g Na/ day) or salt-free diet,Management Generally do not restrict oral fluid intake Severe edema: restrict fluid intake and use diuretics,Management Increase proteins properly 1.52 g/kg/d While undergoing steroid treatment: give VitD 400 iu/d (or Rocaltrol) and calcium,Management 1.3. Preve