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    C:\documents and settings\administrator\桌面\20100607 nephrotic syndrome C:\documents and settings\administrator\桌面\20100607 nephrotic syndrome Presentation Transcript

    • Department of pediatrics The first affiliated hospital Sun Yat Sen University Sun Liangzhong ( 孙良忠 ) [email_address] Nephrotic Syndrome
    • Definition
      • Glomerular permeability↑
      • Clinical features
        • Massive proteinuria
        • Hypoproteinemia
        • Hyperlipidemia
        • Edema
    • Classification
      • Primary/Idiopathic (90%)
      • Secondary:
        • SLE, HBV, anaphylactoid purpura
      • Congenital/hereditary
    • Idiopathic Nephrotic Syndrome
      • Etiology and Pathogenesis
        • Immunologic mechanisms
        • T-lymphocyte Abnormalities
        • Glomerular permeability factor
    • a Basement membrane b Epithelial cell c Endothelial cell d Mesangial cell Glomerular filtration barrier
    • Glomerular filtration barrier a Basement membrane b Epithelial cell c Endothelial cell d Mesangial cell
    • Glomerular filtration barrier
        • Size-selective (aperture) barrier
        • Charge-selective barrier
    • aperture barrier Endothelium slit diaphragm
    •  
    • Charge-selective barrier Endothelium
    •  
    •  
    • Nonselective proteinuria
      • minimal change disease (MCD)
      • mesangial proliferative glomerulonephritis (MsPGN)
      • focal segmental glomerulosclerosis (FSGS)
      • membranous nephrosis (MN)
      • membranoproliferative glomerulonephritis (MPGN)
      Pathological changes in INS
    •  
    •  
    •  
    • Mild Moderate Mesangial proliferative glomerulonephritis
    • Focal segmental glomerulosclerosis (FSGS)
    • membranous nephrosis (MN)
    • Membranoprliferative glomerulonephritis
    • Pathological patterns of INS in children and adults
    • Pathophysiology Pathogenetic factor glomerular permeability↑ massive proteinuria Hypoproteinemia lipoproteins synthesis ↑ hyperlipidemia Plasma oncotic pressure↓ Intravascular volume ↓ RAA(aldosterone)↑ ADH↑ Water  sodium retention Edema Fluid Interstitial space Lipoprotein lipase ↓
    • Clinical manifestation
      • Epidemiology
        • Incidence, sex and age
      • Main symptoms and signs
      • Edema 、 ascites 、 pleural effusion
        • Urine, hematuria
        • Blood pressure
        • Renal function
        • Genaral situation
    •  
    •  
    •  
    •  
      • Urine
        • Urinalysis, 24h urinary protein excretion, urinary Pro/Cr
      • Serum
        • albumin, cholesterol, triglyceride
        • IgG, IgA, IgM, C3
        • BUN, Cr
        • sodium, potassium, calcium
      • Ultrasonography
      • renal biopsy
      Laboratory tests
    • Diagnosis and classifications
        • 24h urinary protein excretion > 50/40mg/kg/d
        • Serum albumin < 25g/L
        • Serum cholesterol > 5.72mmol/L
        • Edema
      • Hematuria
        • Urinary RBC≥10/HPF
      • Hypertension:
        • Preschool age child≥120/80mmHg
        • School age child≥130/90mmHg
      • Renal function insufficient
      • Hypocomplementemia
      Simple type and Nephritic type Clinical types
    • Differential diagnosis
      • What are the related diseases?
      • Edema caused by renal diseases ?
      • Nephrotic syndrome ?
      • Primary, secondary or congenital ?
      • Simple type or nephritic type ?
    • Treatment General treatment
      • Rest
      • Diet
        • Sodium and water
        • Protein
        • calcium and vitamin D
      • Diuresis
      • Education of the family
      • Steroid
        • Prednisone, methyl-prednisolone
      Treatment
      • Corticosteroid therapy
      • Scheme
        • Short course
        • Mediate course
        • Long course
      • Prednisone 1.5-2 mg/kg/d*6-8w
      • Prednisone 2mg/kg qod*4w
      • Prednisone dose (every 2-4w)
      • Course of treatment
      • 6m 9m
      • Intermediate long
      • steroid responsive/sensitive
      • steroid resistant / insensitive
      • steroid dependent
      • frequent relapse
      classification on curative effects
        • Metabolic disturbance
        • Hypertension
        • Infection, peptic ulcer
        • Euphoria, lunacy, induce epilepsy, insomnia
        • Osteoporosis, growth retardation
        • Cataract
        • abuse syndrome and adrenal insufficiency
      Side effects of corticosteroid
        • frequent relapse
        • steroid dependent
        • steroid resistant
        • unable to tolerate steroid treatment
      Indications for Alternative agents
    • Alternative agents for INS
      • Cyclophosphamide (CTX)
      • Cyclosporine (CsA) and tacrolimus (FK506)
      • Mycophenolate, MMF .
      • Angiotensin-converting enzyme inhibitors (ACEI) and angiotensin II blockers
    • Cyclophosphamide (CTX)
      • Cyclophosphamide prolongs the duration of remission and reduces the number of relapses in children with frequently relapsing and steroid-dependent nephrotic syndrome .
        • Leukopenia
        • Alopecia
        • Hepatic function disorder
        • Hemorrhagic cystitis
        • Sterility
        • Disseminated varicella infection
        • Inappropriate ADH secretion
      Side effects of CTX
    • Cyclosporine and tacrolimus
      • Cyclosporine (3–6 mg/kg/24 hr divided q 12 hr)
      • Tacrolimus (0.15 mg/kg/24 hr divided q 12 hr)
      • Both are also effective in maintaining prolonged remissions in children with nephrotic syndrome and are useful as steroid-sparing agents.
    • Side effects of CSA and FK506
      • Hypertension
      • Nephrotoxicity
      • Hirsutism
      • gingival hyperplasia
    • Mycophenolate and ACEI
      • Mycophenolate may maintain remission in children with steroid-dependent or frequently relapsing nephrotic syndrome.
      • ACEI and angiotensin II blockers may be helpful as adjunct therapy to reduce proteinuria in steroid-resistant patients.
    • Others
      • Anticoagulant therapy
        • Heparin, Persantine
      • Immunologic regulators
      • Chinese medicine
    • Complications
      • Infection
      • Electrolyte disorder, Hypovolemia
      • Hypercoagulability and thrombosis
      • Acute renal failure
      • Renal tubular function disorder
      • Infection
      • Manifestations
        • URI, spontaneous peritonitis , tuberculosis, cellulitis, urinary tract infection
      • Cause
        • Immunoglobulin and complement factor↓
        • protein malnutrition, edema,
        • immunosuppressive therapy
      • Management and Prophylaxis
        • high index of suspicion, prompt evaluation
        • early initiation of therapy
        • polyvalent neumococcal vaccine
      • Manifestations
        • Hyponatremia, hypokalemia, Hypocalcemia
        • Hypovolemic shock
      • Cause
        • salt intake restriction
        • diuretic treatment
        • vomit, diarrhea, intestinal reabsorbtion
        • Loss of calcium binding protein
      • Prophylaxis
        • Avoiding aggressive diuretic therapy
        • Inappropriate salt intake restriction
      E lectrolytes disorder and hypovolemia
      • Hypercoagulability and thrombosis
      • Manifestations
        • thrombosis within kidney, extremities, brain and lung
      • Cause
        • coagulation factorsⅡ, Ⅴ, Ⅶ, Ⅷ, Ⅹ↑, platelet aggregation↑, antithrombin Ⅲ↓
        • Hyperlipidemia, diuretic and steroid therapy
      • prophylaxis
        • Avoiding puncture of deep veins
        • Prophylactic anticoagulation drugs
    •  
    •  
      • Manifestations
        • Oliguria or anuria , hypertension
        • Elevated serum Cr and BUN levels
      • Cause
        • Intravascular blood volume↓
        • Obstruction, crescent formation
        • Acute interstitial nephritis, drugs
      • Prophylaxis
        • Avoiding use of renal toxic drugs
        • Avoiding aggressive diuretic therapy
      Acute renal failure
      • Renal tubular function disorder
      • Manifestations
        • polyuria, nocturia, Glucosuria,
        • aminoaciduria, Fanconi syndrome
      • Cause
        • Progress of the glomerular disease
        • Persistent massive proteinuria
      • prophylaxis
        • avoiding excessive albumin transfusion
    • Prognosis
      • Relapse and resolve
      • Prognosis is depend on pathologic patterns
    • Thank You