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Nephrotic Syndrome (NS)

Nephrotic Syndrome (NS). 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.

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Nephrotic Syndrome (NS)

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  1. Nephrotic Syndrome (NS)

  2. DefinitionNS is an accumulation of symptoms and signs and is characterized by proteinuria, hypoproteinemia, edema, and hyperlipidemia.

  3. The vast majority patients (90% of cases) with NS of childhood are primary. In children under age 5 years the disease usually takes the form of idiopathic (primary) NS of childhood (nil disease, lipoid Nephrosis).

  4. Conditions Of AttackSecond only to acute nephri-tis. Incidence age: At all ages, but most commonly between 2~5 years of age.

  5. Type1.Clinical type Simple NS ; Nephritic NS2.Response to steroid therapy (P331)

  6. The initial response to cortico- steroids is a guide to prognosis. (1) Total effect (2)  Partial effect (3)  Non-effect

  7. 3.Pathologic type (P328) Minimal change disease, MCD: 80% of patients.

  8. PathogenesisThe primary disorder is an increase in glomerular permea- bility to plasma proteins.▲Foot processes of the visceral epithelium of the GBM.

  9. 1.The construction of theglomerular basement memb-rane has changed.2.The loss of the negative charges on the GBM.

  10. ◆The underlying pathoge-nesis is unknown, but evidence strongly supports the impor- tance of immune mechanisms(P328).

  11. Pathophysiology1.Proteinuria: Fundamental and highly important change of pathophysiology.

  12. 2.Hypoproteinemia (mainly albumin) 3.Edema: Nephrotic edema (pitting edema)

  13. Hypoproteinemia plasma oncotic pressure is diminished, result in a shift of fluid from the vascular to the interstitial compartment and plasma

  14. volume↓→the activation of the renin–angiotensin–aldo-sterone system→ tubularsodium chloride reabsorp-tion↑.

  15. 4.Hyperlipidemia (Hyper-cholesterolemia) Ch↑, TG↑, LDL-ch↑,VLDL-ch↑.

  16. Clinical ManifestationsThere is a male preponderance of 2:1.1.Main manifestations: Edema (varying degrees) is the common symptom.

  17. Periorbital swelling and perhaps oliguria are noticed→→increasing edema→→anasarca evident.

  18. 2.General symptoms: Pallid, anorexia, fatigue,abdominal pain, diarrhea.

  19. Laboratory Exam1.Urinary protein: 2+～4+ 24hr total urinary protein > 0.1g/kg.( The most are selective proteinuria. )

  20. May occur granular and red cell casts.2.Total serum protein↓,＜30g/L .Albumin levels are low (＜20g/L).

  21. 3.Serumcholesterol and triglycerides: Cholesterol ＞5.7mmol/L (220mg/dl).4.ESR↑＞100mm/hr.

  22. 5.Serum proteins electro-phoresis : Albumin↓, α2-G↑,γ-G↓, A/G inversion.

  23. 6.Serum complemen: Vary with clinical type.7.Renal function:

  24. Complications1.InfectionsInfections is a major compli-cation in children with NS. Itfrequently trigger relapses.

  25. Site:Respiratory tract, skin, urinary tract and acute pri-mary peritonitis.

  26. Causes: Immunity lower , severe edema→malcirculation, protein malnutrition, and use hormone and immunosuppre-ssive agents.

  27. 2.Electrolyte disturbances (1) Hyponatremia(2) Hypokalemia(3) Hypocalcemia

  28. 3.Thromboembolic phenomena ( Hypercoagulability ) Renal vein thrombosis4.Hypovolemic shook5.Acute renal failure (prerenal)

  29. Diagnosis1.Diagnostic standard (P330):●Four characteristics.●Excluding other renal disease (second nephrosis).

  30. 2.Clinical typeSimple NS or Nephritic NS.Treatment 1.General measures1.1Rest

  31. 1.2Diet Hypertension and edema: Low salt diet (<2gNa/ day) or salt-free diet. Severe edema: Restricting fluid intake.

  32. Increase proteins properly: 2g/(kg·day) While undergoing the corti-costeroid treatment: Give VitD 500~1000iu/day (or Rocaltrol)and calcium.

  33. 1.3Prevent infection1.4DiureticsNot requires diuretics usually.＊HCT 2~5mg/(kg · day)＊Antisterone 3~5mg/(kg · day)＊Triamterene

  34. Attention:Volume depletion, disorder of electrolyte and embolism.

  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. 2.Corticosteroid therapyShort-course therapy:Prednisone 2mg/(kg·day) or 60mg/m2/day (Max.60mg/day) in 3 or 4 divided doses for 4wk→maintenance treatment:

  37. Prednisone 1.5mg/kg, single dose for every-other day×4wk.▲Total course of therapy: 8 wk.

  38. Middle-course & long-course therapy:① Induction of remission: Prednisone 1.5~2mg/(kg · day)(Max.60mg/day) for 4wk untilthe urinary protein falls totrace or negative levels ②

  39. ②After maintenance treatment: Prednisone 2mg/kg , single dose for every-other-day×4wk tapered gradually (2.5~5mg/2wk) discontinued.

  40. ▲Total course of treatment : ★Middle: 6mo ★Long: 9~12mo Estimate of curative effect (P331).

  41. 3. Treatment of relapse and recurrence3.1Extend the course of corti- costeroid3.2Immunosuppressive agents(Cytotoxic agents):

  42. ①CTX (Cytoxan)2mg/(kg·day) for 8~12wk .Total amount: 250mg/kg Side effects: nausea, vomiting,WBC↓, trichomadesis, hemo-rrhagic cystitis and the damage of sexual glands.

  43. ②CB (Chlorambucil)0.2mg/kg for 8wk . Total amount : 10mg/kg③VCR & Levamisole

  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. necessary, give another 1~2courses after 1~2wkprednisone 2mg/kg, qodtapered gradually.

  46. (2)CTX 0.5~0.75mg/m2+ NS/GS iv drip (1hr), give liquid 2,000ml/(m2.d) . Every one mofor6~8times.

  47. (3)CsA 5~7mg/kg, in 3 divided doses for3~6mo. ★expenseand nephrotoxicity.

  48. (4)AnticoagulantsHeparin Persantin 5mg/(kg·day）for6mo.

  49. 5.Alleviar proteinuria Angiotensin converting en-zyme inhibitions (ACEI) : Captopril, Enalapril and Benazepril.

  50. PrognosisMost cases of minimal change disease eventually remit permanently.

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