C:\documents and settings\administrator\桌面\20100607 nephrotic syndrome
Upcoming SlideShare
Loading in...5
×
 

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

on

  • 4,944 views

 

Statistics

Views

Total Views
4,944
Views on SlideShare
4,943
Embed Views
1

Actions

Likes
2
Downloads
247
Comments
0

1 Embed 1

http://www.slideshare.net 1

Accessibility

Upload Details

Uploaded via as Microsoft PowerPoint

Usage Rights

© All Rights Reserved

Report content

Flagged as inappropriate Flag as inappropriate
Flag as inappropriate

Select your reason for flagging this presentation as inappropriate.

Cancel
  • Full Name Full Name Comment goes here.
    Are you sure you want to
    Your message goes here
    Processing…
Post Comment
Edit your comment

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