Nephrotic Syndrome in Children for developing.ppt

Management of Minimal Change Nephrotic Syndrome

1. Nephrotic
Syndrome (NS)

2. Definition
NS 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 Attack
Second only to acute nephri-
tis.
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
(P
331
)

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 (P
328
)
Minimal change disease,
MCD: 80% of patients.

8. Pathogenesis
The 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 the
glomerular basement memb-
rane has changed.
2.The loss of the negative
charges on the GB
M.

10. ◆The underlying pathoge-
nesis is unknown, but evidence
strongly supports the impor- t
ance of immune mechanisms
(P
328
).

11. Pathophysiology







1.Proteinuria: Fundamental
and highly important change of
pathophysiology.

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

13. Hypoproteinemia plasma
oncotic pres
sure is diminished, result in a
shift of fluid from the vascular
to the interstitial compartmen
t and plasma

14. volume↓→the activation of

the renin–
angiotensin–aldo-
sterone system→ tubular
sodium chloride reabsorp-
tion↑.

15. 4. Hyperlipidemia (Hyper-
cholesterolemia)
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, an
orexia, fatigue,
abdominal pain, diarrhea.

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

20. May occur granular and red
cell casts.
2.Total serum protein↓,
＜30g/L .
Albumin levels are low (＜2
0g/L).

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

22. 5.Serum proteins electro-
phoresis :
Albumin↓, α
2
-G↑,γ-G↓,
A/G inversion.

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

24. Complications
1.Infections
Infections is a major compli-
cation in children with NS. It
frequently 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 thrombosis
4.Hypovolemic shook
5.Acute renal failure (prerenal)

29. Diagnosis
1.Diagnostic standard (P
330
):
●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: L
ow salt diet (<2gNa/ day) or sa
lt-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. 1.3 Prevent infection
1.4 Diuretics
Not requires diuretics usually.
＊HCT 2~5mg/(kg · day)
＊Antisterone 3~5mg/(kg · da
y)
＊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
60mg/m
2
/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:
Induction of remission:
①
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 (P
331
).

41. 3. Treatment of relapse and rec
urrence
3.1 Extend the course of corti-
costeroid
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, hemo-
rrhagic cystitis and the damage
of sexual glands.

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/m
2
+ NS/GS iv
drip (1hr), give liquid 2,000ml
/(m
2
.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 en-
zyme inhibitions (ACEI) :
Captopril, Enalapril and
Benazepril.

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