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Department of pediatrics The first affiliated hospital Sun Yat Sen University Sun Liangzhong  ( 孙良忠 )   [email_address] Ne...
Definition <ul><li>Glomerular permeability↑ </li></ul><ul><li>Clinical features   </li></ul><ul><ul><li>Massive proteinuri...
Classification <ul><li>Primary/Idiopathic (90%) </li></ul><ul><li>Secondary:  </li></ul><ul><ul><li>SLE, HBV, anaphylactoi...
Idiopathic  Nephrotic Syndrome <ul><li>Etiology and Pathogenesis </li></ul><ul><ul><li>Immunologic mechanisms </li></ul></...
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 <ul><ul><li>Size-selective (aperture) barrier </li></ul></ul><ul><ul><li>Charge-selective ba...
aperture barrier Endothelium slit diaphragm
 
Charge-selective barrier Endothelium
 
 
Nonselective proteinuria
<ul><li>minimal change disease (MCD)  </li></ul><ul><li>mesangial proliferative glomerulonephritis (MsPGN)  </li></ul><ul>...
 
 
 
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 ↑ ...
Clinical manifestation <ul><li>Epidemiology </li></ul><ul><ul><li>Incidence, sex and age </li></ul></ul><ul><li>Main sympt...
 
 
 
 
<ul><li>Urine </li></ul><ul><ul><li>Urinalysis, 24h urinary protein excretion, urinary Pro/Cr </li></ul></ul><ul><li>Serum...
Diagnosis and classifications <ul><ul><li>24h urinary protein excretion > 50/40mg/kg/d </li></ul></ul><ul><ul><li>Serum al...
<ul><li>Hematuria  </li></ul><ul><ul><li>Urinary RBC≥10/HPF </li></ul></ul><ul><li>Hypertension:  </li></ul><ul><ul><li>Pr...
Differential diagnosis <ul><li>What are the related diseases? </li></ul><ul><li>Edema caused by renal diseases ? </li></ul...
Treatment General treatment <ul><li>Rest   </li></ul><ul><li>Diet   </li></ul><ul><ul><li>Sodium and water </li></ul></ul>...
<ul><li>Steroid </li></ul><ul><ul><li>Prednisone, methyl-prednisolone </li></ul></ul>Treatment
<ul><li>Corticosteroid therapy </li></ul><ul><li>Scheme </li></ul><ul><ul><li>Short course </li></ul></ul><ul><ul><li>Medi...
<ul><li>Prednisone 1.5-2 mg/kg/d*6-8w  </li></ul><ul><li>Prednisone 2mg/kg qod*4w </li></ul><ul><li>Prednisone dose (every...
<ul><li>steroid responsive/sensitive </li></ul><ul><li>steroid resistant / insensitive </li></ul><ul><li>steroid dependent...
<ul><ul><li>Metabolic disturbance </li></ul></ul><ul><ul><li>Hypertension </li></ul></ul><ul><ul><li>Infection, peptic ulc...
<ul><ul><li>frequent relapse </li></ul></ul><ul><ul><li>steroid dependent </li></ul></ul><ul><ul><li>steroid resistant </l...
Alternative agents   for INS <ul><li>Cyclophosphamide (CTX) </li></ul><ul><li>Cyclosporine (CsA) and tacrolimus (FK506)  <...
Cyclophosphamide (CTX) <ul><li>Cyclophosphamide prolongs the duration of remission and reduces the number of relapses in c...
<ul><ul><li>Leukopenia </li></ul></ul><ul><ul><li>Alopecia </li></ul></ul><ul><ul><li>Hepatic function disorder </li></ul>...
Cyclosporine and tacrolimus <ul><li>Cyclosporine   (3–6 mg/kg/24 hr divided q 12 hr)  </li></ul><ul><li>Tacrolimus  (0.15 ...
Side effects of  CSA and FK506 <ul><li>Hypertension </li></ul><ul><li>Nephrotoxicity </li></ul><ul><li>Hirsutism </li></ul...
Mycophenolate and ACEI <ul><li>Mycophenolate  may maintain remission in children with steroid-dependent or frequently rela...
Others <ul><li>Anticoagulant therapy </li></ul><ul><ul><li>Heparin, Persantine </li></ul></ul><ul><li>Immunologic regulato...
Complications <ul><li>Infection </li></ul><ul><li>Electrolyte disorder, Hypovolemia </li></ul><ul><li>Hypercoagulability a...
<ul><li>Infection </li></ul><ul><li>Manifestations </li></ul><ul><ul><li>URI,  spontaneous peritonitis , tuberculosis, cel...
<ul><li>Manifestations </li></ul><ul><ul><li>Hyponatremia, hypokalemia, Hypocalcemia </li></ul></ul><ul><ul><li>Hypovolemi...
<ul><li>Hypercoagulability and thrombosis </li></ul><ul><li>Manifestations </li></ul><ul><ul><li>thrombosis within kidney,...
 
 
<ul><li>Manifestations </li></ul><ul><ul><li>Oliguria or anuria , hypertension </li></ul></ul><ul><ul><li>Elevated serum C...
<ul><li>Renal tubular function disorder </li></ul><ul><li>Manifestations </li></ul><ul><ul><li>polyuria, nocturia,   Gluco...
Prognosis <ul><li>Relapse and resolve </li></ul><ul><li>Prognosis is depend on pathologic patterns </li></ul>
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  1. 1. Department of pediatrics The first affiliated hospital Sun Yat Sen University Sun Liangzhong ( 孙良忠 ) [email_address] Nephrotic Syndrome
  2. 2. Definition <ul><li>Glomerular permeability↑ </li></ul><ul><li>Clinical features </li></ul><ul><ul><li>Massive proteinuria </li></ul></ul><ul><ul><li>Hypoproteinemia </li></ul></ul><ul><ul><li>Hyperlipidemia </li></ul></ul><ul><ul><li>Edema </li></ul></ul>
  3. 3. Classification <ul><li>Primary/Idiopathic (90%) </li></ul><ul><li>Secondary: </li></ul><ul><ul><li>SLE, HBV, anaphylactoid purpura </li></ul></ul><ul><li>Congenital/hereditary </li></ul>
  4. 4. Idiopathic Nephrotic Syndrome <ul><li>Etiology and Pathogenesis </li></ul><ul><ul><li>Immunologic mechanisms </li></ul></ul><ul><ul><li>T-lymphocyte Abnormalities </li></ul></ul><ul><ul><li>Glomerular permeability factor </li></ul></ul>
  5. 5. a Basement membrane b Epithelial cell c Endothelial cell d Mesangial cell Glomerular filtration barrier
  6. 6. Glomerular filtration barrier a Basement membrane b Epithelial cell c Endothelial cell d Mesangial cell
  7. 7. Glomerular filtration barrier <ul><ul><li>Size-selective (aperture) barrier </li></ul></ul><ul><ul><li>Charge-selective barrier </li></ul></ul>
  8. 8. aperture barrier Endothelium slit diaphragm
  9. 10. Charge-selective barrier Endothelium
  10. 13. Nonselective proteinuria
  11. 14. <ul><li>minimal change disease (MCD) </li></ul><ul><li>mesangial proliferative glomerulonephritis (MsPGN) </li></ul><ul><li>focal segmental glomerulosclerosis (FSGS) </li></ul><ul><li>membranous nephrosis (MN) </li></ul><ul><li>membranoproliferative glomerulonephritis (MPGN) </li></ul>Pathological changes in INS
  12. 18. Mild Moderate Mesangial proliferative glomerulonephritis
  13. 19. Focal segmental glomerulosclerosis (FSGS)
  14. 20. membranous nephrosis (MN)
  15. 21. Membranoprliferative glomerulonephritis
  16. 22. Pathological patterns of INS in children and adults
  17. 23. 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 ↓
  18. 24. Clinical manifestation <ul><li>Epidemiology </li></ul><ul><ul><li>Incidence, sex and age </li></ul></ul><ul><li>Main symptoms and signs </li></ul><ul><li>Edema 、 ascites 、 pleural effusion </li></ul><ul><ul><li>Urine, hematuria </li></ul></ul><ul><ul><li>Blood pressure </li></ul></ul><ul><ul><li>Renal function </li></ul></ul><ul><ul><li>Genaral situation </li></ul></ul>
  19. 29. <ul><li>Urine </li></ul><ul><ul><li>Urinalysis, 24h urinary protein excretion, urinary Pro/Cr </li></ul></ul><ul><li>Serum </li></ul><ul><ul><li>albumin, cholesterol, triglyceride </li></ul></ul><ul><ul><li>IgG, IgA, IgM, C3 </li></ul></ul><ul><ul><li>BUN, Cr </li></ul></ul><ul><ul><li>sodium, potassium, calcium </li></ul></ul><ul><li>Ultrasonography </li></ul><ul><li>renal biopsy </li></ul>Laboratory tests
  20. 30. Diagnosis and classifications <ul><ul><li>24h urinary protein excretion > 50/40mg/kg/d </li></ul></ul><ul><ul><li>Serum albumin < 25g/L </li></ul></ul><ul><ul><li>Serum cholesterol > 5.72mmol/L </li></ul></ul><ul><ul><li>Edema </li></ul></ul>
  21. 31. <ul><li>Hematuria </li></ul><ul><ul><li>Urinary RBC≥10/HPF </li></ul></ul><ul><li>Hypertension: </li></ul><ul><ul><li>Preschool age child≥120/80mmHg </li></ul></ul><ul><ul><li>School age child≥130/90mmHg </li></ul></ul><ul><li>Renal function insufficient </li></ul><ul><li>Hypocomplementemia </li></ul>Simple type and Nephritic type Clinical types
  22. 32. Differential diagnosis <ul><li>What are the related diseases? </li></ul><ul><li>Edema caused by renal diseases ? </li></ul><ul><li>Nephrotic syndrome ? </li></ul><ul><li>Primary, secondary or congenital ? </li></ul><ul><li>Simple type or nephritic type ? </li></ul>
  23. 33. Treatment General treatment <ul><li>Rest </li></ul><ul><li>Diet </li></ul><ul><ul><li>Sodium and water </li></ul></ul><ul><ul><li>Protein </li></ul></ul><ul><ul><li>calcium and vitamin D </li></ul></ul><ul><li>Diuresis </li></ul><ul><li>Education of the family </li></ul>
  24. 34. <ul><li>Steroid </li></ul><ul><ul><li>Prednisone, methyl-prednisolone </li></ul></ul>Treatment
  25. 35. <ul><li>Corticosteroid therapy </li></ul><ul><li>Scheme </li></ul><ul><ul><li>Short course </li></ul></ul><ul><ul><li>Mediate course </li></ul></ul><ul><ul><li>Long course </li></ul></ul>
  26. 36. <ul><li>Prednisone 1.5-2 mg/kg/d*6-8w </li></ul><ul><li>Prednisone 2mg/kg qod*4w </li></ul><ul><li>Prednisone dose (every 2-4w) </li></ul><ul><li>Course of treatment </li></ul><ul><li>6m 9m </li></ul><ul><li>Intermediate long </li></ul>
  27. 37. <ul><li>steroid responsive/sensitive </li></ul><ul><li>steroid resistant / insensitive </li></ul><ul><li>steroid dependent </li></ul><ul><li>frequent relapse </li></ul>classification on curative effects
  28. 38. <ul><ul><li>Metabolic disturbance </li></ul></ul><ul><ul><li>Hypertension </li></ul></ul><ul><ul><li>Infection, peptic ulcer </li></ul></ul><ul><ul><li>Euphoria, lunacy, induce epilepsy, insomnia </li></ul></ul><ul><ul><li>Osteoporosis, growth retardation </li></ul></ul><ul><ul><li>Cataract </li></ul></ul><ul><ul><li>abuse syndrome and adrenal insufficiency </li></ul></ul>Side effects of corticosteroid
  29. 39. <ul><ul><li>frequent relapse </li></ul></ul><ul><ul><li>steroid dependent </li></ul></ul><ul><ul><li>steroid resistant </li></ul></ul><ul><ul><li>unable to tolerate steroid treatment </li></ul></ul>Indications for Alternative agents
  30. 40. Alternative agents for INS <ul><li>Cyclophosphamide (CTX) </li></ul><ul><li>Cyclosporine (CsA) and tacrolimus (FK506) </li></ul><ul><li>Mycophenolate, MMF . </li></ul><ul><li>Angiotensin-converting enzyme inhibitors (ACEI) and angiotensin II blockers </li></ul>
  31. 41. Cyclophosphamide (CTX) <ul><li>Cyclophosphamide prolongs the duration of remission and reduces the number of relapses in children with frequently relapsing and steroid-dependent nephrotic syndrome . </li></ul>
  32. 42. <ul><ul><li>Leukopenia </li></ul></ul><ul><ul><li>Alopecia </li></ul></ul><ul><ul><li>Hepatic function disorder </li></ul></ul><ul><ul><li>Hemorrhagic cystitis </li></ul></ul><ul><ul><li>Sterility </li></ul></ul><ul><ul><li>Disseminated varicella infection </li></ul></ul><ul><ul><li>Inappropriate ADH secretion </li></ul></ul>Side effects of CTX
  33. 43. Cyclosporine and tacrolimus <ul><li>Cyclosporine (3–6 mg/kg/24 hr divided q 12 hr) </li></ul><ul><li>Tacrolimus (0.15 mg/kg/24 hr divided q 12 hr) </li></ul><ul><li>Both are also effective in maintaining prolonged remissions in children with nephrotic syndrome and are useful as steroid-sparing agents. </li></ul>
  34. 44. Side effects of CSA and FK506 <ul><li>Hypertension </li></ul><ul><li>Nephrotoxicity </li></ul><ul><li>Hirsutism </li></ul><ul><li>gingival hyperplasia </li></ul>
  35. 45. Mycophenolate and ACEI <ul><li>Mycophenolate may maintain remission in children with steroid-dependent or frequently relapsing nephrotic syndrome. </li></ul><ul><li>ACEI and angiotensin II blockers may be helpful as adjunct therapy to reduce proteinuria in steroid-resistant patients. </li></ul>
  36. 46. Others <ul><li>Anticoagulant therapy </li></ul><ul><ul><li>Heparin, Persantine </li></ul></ul><ul><li>Immunologic regulators </li></ul><ul><li>Chinese medicine </li></ul>
  37. 47. Complications <ul><li>Infection </li></ul><ul><li>Electrolyte disorder, Hypovolemia </li></ul><ul><li>Hypercoagulability and thrombosis </li></ul><ul><li>Acute renal failure </li></ul><ul><li>Renal tubular function disorder </li></ul>
  38. 48. <ul><li>Infection </li></ul><ul><li>Manifestations </li></ul><ul><ul><li>URI, spontaneous peritonitis , tuberculosis, cellulitis, urinary tract infection </li></ul></ul><ul><li>Cause </li></ul><ul><ul><li>Immunoglobulin and complement factor↓ </li></ul></ul><ul><ul><li>protein malnutrition, edema, </li></ul></ul><ul><ul><li>immunosuppressive therapy </li></ul></ul><ul><li>Management and Prophylaxis </li></ul><ul><ul><li>high index of suspicion, prompt evaluation </li></ul></ul><ul><ul><li>early initiation of therapy </li></ul></ul><ul><ul><li>polyvalent neumococcal vaccine </li></ul></ul>
  39. 49. <ul><li>Manifestations </li></ul><ul><ul><li>Hyponatremia, hypokalemia, Hypocalcemia </li></ul></ul><ul><ul><li>Hypovolemic shock </li></ul></ul><ul><li>Cause </li></ul><ul><ul><li>salt intake restriction </li></ul></ul><ul><ul><li>diuretic treatment </li></ul></ul><ul><ul><li>vomit, diarrhea, intestinal reabsorbtion </li></ul></ul><ul><ul><li>Loss of calcium binding protein </li></ul></ul><ul><li>Prophylaxis </li></ul><ul><ul><li>Avoiding aggressive diuretic therapy </li></ul></ul><ul><ul><li>Inappropriate salt intake restriction </li></ul></ul>E lectrolytes disorder and hypovolemia
  40. 50. <ul><li>Hypercoagulability and thrombosis </li></ul><ul><li>Manifestations </li></ul><ul><ul><li>thrombosis within kidney, extremities, brain and lung </li></ul></ul><ul><li>Cause </li></ul><ul><ul><li>coagulation factorsⅡ, Ⅴ, Ⅶ, Ⅷ, Ⅹ↑, platelet aggregation↑, antithrombin Ⅲ↓ </li></ul></ul><ul><ul><li>Hyperlipidemia, diuretic and steroid therapy </li></ul></ul><ul><li>prophylaxis </li></ul><ul><ul><li>Avoiding puncture of deep veins </li></ul></ul><ul><ul><li>Prophylactic anticoagulation drugs </li></ul></ul>
  41. 53. <ul><li>Manifestations </li></ul><ul><ul><li>Oliguria or anuria , hypertension </li></ul></ul><ul><ul><li>Elevated serum Cr and BUN levels </li></ul></ul><ul><li>Cause </li></ul><ul><ul><li>Intravascular blood volume↓ </li></ul></ul><ul><ul><li>Obstruction, crescent formation </li></ul></ul><ul><ul><li>Acute interstitial nephritis, drugs </li></ul></ul><ul><li>Prophylaxis </li></ul><ul><ul><li>Avoiding use of renal toxic drugs </li></ul></ul><ul><ul><li>Avoiding aggressive diuretic therapy </li></ul></ul>Acute renal failure
  42. 54. <ul><li>Renal tubular function disorder </li></ul><ul><li>Manifestations </li></ul><ul><ul><li>polyuria, nocturia, Glucosuria, </li></ul></ul><ul><ul><li>aminoaciduria, Fanconi syndrome </li></ul></ul><ul><li>Cause </li></ul><ul><ul><li>Progress of the glomerular disease </li></ul></ul><ul><ul><li>Persistent massive proteinuria </li></ul></ul><ul><li>prophylaxis </li></ul><ul><ul><li>avoiding excessive albumin transfusion </li></ul></ul>
  43. 55. Prognosis <ul><li>Relapse and resolve </li></ul><ul><li>Prognosis is depend on pathologic patterns </li></ul>
  44. 56. Thank You
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