Nephrotic (1)

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Nephrotic (1)

  1. 1. Nephrotic Syndrome (NS)
  2. 2. Definition NS is an accumulation of symptoms and signs and is characterized by proteinuria, hypoproteinemia, edema, and hyperlipidemia.
  3. 3. In children under age 5 years the disease usually takes the form of idiopathic (primary) NS of childhood (nil disease, lipoid Nephrosis).
  4. 4. Conditions Of Attack Second only to acute nephritis. Incidence age: At all ages, but most commonly between 2~5 years of age.
  5. 5. Type 1.Clinical type Simple NS ; Nephritic NS 2.Response to steroid therapy (P331)
  6. 6. The initial response to corticosteroids is a guide to prognosis. (1) Total effect (2) Partial effect (3) Non-effect
  7. 7. 3. Pathologic type (P328) Minimal change disease, MCD: 80% of patients.
  8. 8. Pathogenesis The primary disorder is an increase in glomerular permeability to plasma proteins. ▲Foot processes of the visceral epithelium of the GBM.
  9. 9. 1.The construction of the glomerular basement membrane has changed. 2.The loss of the negative charges on the GBM.
  10. 10. ◆The underlying pathogenesis is unknown, but evidence strongly supports the importance of immune mechanisms (P328).
  11. 11. 1.Proteinuria: Fundamental
  12. 12. 2.Hypoproteinemia (mainly albumin) 3.Edema: Nephrotic edema (pitting edema)
  13. 13. Hypoproteinemia plasma oncotic pressure is diminished, result in a shift of fluid from the vascular to the interstitial compartment and plasma
  14. 14. volume↓→the activation of the renin– angiotensin–aldosterone system→ tubular sodium chloride reabsorption↑.
  15. 15. 4. Hyperlipidemia (Hypercholesterolemia) Ch↑, TG↑, LDL-ch↑, VLDL-ch↑.
  16. 16. Clinical Manifestations There is a male preponderance of 2:1. 1.Main manifestations: Edema (varying degrees) is the common symptom.
  17. 17. Periorbital swelling and perhaps oliguria are noticed →→increasing edema→→ anasarca evident.
  18. 18. 2.General symptoms: Pallid, anorexia, fatigue, abdominal pain, diarrhea.
  19. 19. Laboratory Exam 1.Urinary protein: 2 ~ 4 24hr total urinary protein > 0.1g/kg. ( The most are selective proteinuria. ) + +
  20. 20. May occur granular and red cell casts. 2.Total serum protein↓, < 30g/L . Albumin levels are low ( < 20g/L).
  21. 21. 3.Serum cholesterol and triglycerides: Cholesterol > 5.7mmol/L (220mg/dl). 4. ESR↑ > 100mm/hr.
  22. 22. 5.Serum proteins electrophoresis : Albumin↓, α2-G↑,γ-G↓, A/G inversion.
  23. 23. 6.Serum complemen: Vary with clinical type. 7.Renal function:
  24. 24. Complications 1.Infections Infections is a major complication in children with NS. It frequently trigger relapses.
  25. 25. Site: Respiratory tract, skin, urinary tract and acute primary peritonitis.
  26. 26. Causes: Immunity lower , severe edema→malcirculation, protein malnutrition, and use hormone and immunosuppressive agents.
  27. 27. 2.Electrolyte disturbances (1) Hyponatremia (2) Hypokalemia (3) Hypocalcemia
  28. 28. 3.Thromboembolic phenomena ( Hypercoagulability ) Renal vein thrombosis 4.Hypovolemic shook 5.Acute renal failure (prerenal)
  29. 29. Diagnosis 1.Diagnostic standard (P330): ●Four characteristics. ●Excluding other renal disease (second nephrosis).
  30. 30. 2.Clinical type Simple NS or Nephritic NS. Treatment 1.General measures 1.1 Rest
  31. 31. 1.2 Diet Hypertension and edema: Low salt diet (<2gNa/ day) or salt-free diet. Severe edema: Restricting fluid intake.
  32. 32. Increase proteins properly: 2g/(kg·day) While undergoing the corticosteroid treatment: Give VitD 500~1000iu/day (or Rocaltrol) and calcium.
  33. 33. 1.3 Prevent infection 1.4 Diuretics Not requires diuretics usually. * HCT 2~5mg/(kg · day) * Antisterone 3~5mg/(kg · day) * Triamterene
  34. 34. Attention: Volume depletion, disorder of electrolyte and embolism.
  35. 35. Apparent edema: Give low molecular dextran 10~15ml/(kg·time); [+Dopamine 2~3ug/(kg·min) and/or Regitine 10mg +Lasix 1~2mg/kg].
  36. 36. 2.Corticosteroid therapy Short-course therapy: Prednisone 2mg/(kg·day) or 2 60mg/m /day (Max.60mg/day) in 3 or 4 divided doses for 4wk →maintenance treatment:
  37. 37. Prednisone 1.5mg/kg, single dose for every-other day×4wk. ▲Total course of therapy: 8 wk.
  38. 38. Middle-course & long-course therapy: ① Induction of remission: Prednisone 1.5~2mg/(kg · day) (Max.60mg/day) for 4wk until the urinary protein falls to trace or negative levels ②
  39. 39. ②After maintenance treatment: Prednisone 2mg/kg , single dose for every-other-day×4wk tapered gradually (2.5~5 mg/2wk) discontinued.
  40. 40. ▲Total course of treatment : ★Middle: 6mo ★Long: 9~12mo Estimate of curative effect (P331).
  41. 41. 3. Treatment of relapse and recurrence 3.1 Extend the course of corticosteroid 3.2 Immunosuppressive agents (Cytotoxic agents):
  42. 42. ① CTX (Cytoxan) 2mg/(kg·day) for 8~12wk . Total amount: 250mg/kg Side effects: nausea, vomiting, WBC↓, trichomadesis, hemorrhagic cystitis and the damage
  43. 43. ② CB (Chlorambucil) 0.2mg/kg for 8wk . Total amount : 10mg/kg ③ VCR & Levamisole
  44. 44. 4.Impulsive therapy (1) Methylprednisolone (MP) 15~30mg/kg(<1g/day+10% GS 100~ 250ml, iv drip (within 1~2hr) , 3 times/one course. If
  45. 45. necessary, give another 1~2 courses after 1~2wk prednisone 2mg/kg, qod tapered gradually.
  46. 46. (2) CTX 0.5~0.75mg/m2 + NS/GS iv drip (1hr), give liquid 2,000ml 2 /(m .d) . Every one mo for 6~8 times.
  47. 47. (3) CsA 5~7mg/kg, in 3 divided doses for 3~6mo. ★expense and nephrotoxicity.
  48. 48. (4) Anticoagulants Heparin Persantin 5mg/(kg·day ) for 6mo.
  49. 49. 5.Alleviar proteinuria Angiotensin converting enzyme inhibitions (ACEI) : Captopril, Enalapril and Benazepril.
  50. 50. Prognosis Most cases of minimal change disease eventually remit permanently.

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