1. Nephrotic
Syndrome (NS)

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

3. 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 Attack
Second only to acute nephritis.
Incidence age: At all ages,
but most commonly between
2~5 years of age.

5. Type
1.Clinical type
Simple NS ; Nephritic NS
2.Response to steroid therapy
(P331)

6. The initial response to corticosteroids 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. Pathogenesis
The primary disorder is an
increase in glomerular permeability to plasma proteins.
▲Foot processes of the visceral
epithelium of the GBM.

9. 1.The construction of the
glomerular basement membrane has changed.
2.The loss of the negative
charges on the
GBM.

10. ◆The underlying pathogenesis is unknown, but evidence
strongly supports the importance of immune mechanisms
(P328).

11. 1.Proteinuria: Fundamental

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–aldosterone system→ tubular
sodium chloride reabsorption↑.

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

16. Clinical Manifestations
There 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 Exam
1.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.Serum cholesterol and
triglycerides:
Cholesterol ＞ 5.7mmol/L
(220mg/dl).
4. ESR↑ ＞ 100mm/hr.

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

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

24. Complications
1.Infections
Infections is a major complication in children with NS. It
frequently trigger relapses.

25. Site: Respiratory tract, skin,
urinary tract and acute primary peritonitis.

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

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

28. 3.Thromboembolic phenomena
( Hypercoagulability )
Renal vein thrombosis
4.Hypovolemic shook
5.Acute renal failure (prerenal)

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

30. 2.Clinical type
Simple NS or Nephritic NS.
Treatment
1.General measures
1.1 Rest

31. 1.2 Diet
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 corticosteroid treatment: Give VitD
500~1000iu/day (or Rocaltrol)
and calcium.

33. 1.3 Prevent infection
1.4 Diuretics
Not 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 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. 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 until
the urinary protein falls to
trace or negative levels
②

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

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

41. 3. Treatment of relapse and
recurrence
3.1 Extend the course of corticosteroid
3.2 Immunosuppressive agents
(Cytotoxic agents):

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. ② CB (Chlorambucil)
0.2mg/kg for 8wk .
Total amount : 10mg/kg
③ VCR & 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 1~2
courses after 1~2wk
prednisone 2mg/kg, qod
tapered gradually.

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. (3) CsA
5~7mg/kg, in 3 divided doses
for 3~6mo.
★expense and nephrotoxicity.

48. (4) Anticoagulants
Heparin
Persantin 5mg/(kg·day ） for
6mo.

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

50. Prognosis
Most cases of minimal
change disease eventually
remit permanently.