Nephrotic

Syndrome (NS)

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

In children under age 5 years

the disease usually takes the
form of idiopathic (primary) NS
of childhood (nil disease, lipoid
Nephrosis).

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

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

The initial response to corticosteroids is a guide to prognosis.

(1) Total effect
(2) Partial effect
(3) Non-effect

3. Pathologic type (P328)

Minimal change disease,
MCD: 80% of patients.

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

1.The construction of the

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

The underlying pathogenesis is unknown, but evidence

strongly supports the importance of immune mechanisms
(P328).

Pathophysiology

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

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

volumethe activation of
the renin
angiotensinaldosterone system tubular
sodium chloride reabsorp-

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

Clinical Manifestations
There is a male preponderance
of 2:1.
1.Main manifestations: Edema
(varying degrees) is the common
symptom.

Periorbital swelling and

perhaps oliguria are noticed
increasing edema
anasarca evident.

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

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

May occur granular and red

cell casts.
2.Total serum protein,
30g/L .
Albumin levels are low (

3.Serum cholesterol and

triglycerides:
Cholesterol 5.7mmol/L
(220mg/dl).
4. ESR 100mm/hr.

5.Serum proteins electrophoresis :

Albumin, 2-G,-G,
A/G inversion.

6.Serum complemen: Vary

with clinical type.
7.Renal function:

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

Site: Respiratory tract, skin,

urinary tract and acute primary peritonitis.

Causes: Immunity lower ,

severe edemamalcirculation,
protein malnutrition, and use
hormone and immunosuppressive agents.

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

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

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

2.Clinical type
Simple NS or Nephritic NS.

Treatment
1.General measures

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

Increase proteins properly:

2g/(kgday)
While undergoing the corticosteroid treatment: Give VitD
500~1000iu/day (or Rocaltrol)
and calcium.

1.3 Prevent infection

1.4 Diuretics
Not requires diuretics usually.
HCT 2~5mg/(kg day)
Antisterone 3~5mg/(kg
day)

Attention: Volume depletion,

disorder of electrolyte and
embolism.

Apparent edema:
Give low molecular dextran
10~15ml/(kgtime);
[+Dopamine 2~3ug/(kgmin)
and/or Regitine 10mg +Lasix
1~2mg/kg].

2.Corticosteroid therapy
Short-course therapy:
Prednisone 2mg/(kgday) or
2
60mg/m /day (Max.60mg/day)
in 3 or 4 divided doses for 4wk

maintenance treatment:

Prednisone 1.5mg/kg, single

dose for every-other day4wk.
Total course of therapy: 8
wk.

Middle-course & long-course

therapy:
Induction of remission:
Prednisone 1.5~2mg/(kg day)
(Max.60mg/day) for 4wk until
the urinary protein falls to
trace or negative levels

After maintenance
treatment:
Prednisone 2mg/kg , single
dose for every-other-day4wk
tapered gradually (2.5~5
mg/2wk)
discontinued.

Total course of treatment :

Middle: 6mo
Long: 9~12mo
Estimate of curative effect
(P331).

3. Treatment of relapse and

recurrence
3.1 Extend the course of corticosteroid
3.2 Immunosuppressive agents
(Cytotoxic agents):

 CTX (Cytoxan)
2mg/(kgday) for 8~12wk .
Total amount: 250mg/kg
Side effects: nausea,
vomiting,

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

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

necessary, give another 1~2

courses after 1~2wk
prednisone 2mg/kg, qod
tapered gradually.

(2) CTX
2
0.5~0.75mg/m + NS/GS iv
drip (1hr), give liquid 2,000ml
2
/(m .d) .
Every one mo for 6~8 times.

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

(4) Anticoagulants
Heparin
Persantin 5mg/(kgday for
6mo.

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

Prognosis
Most cases of minimal
change disease eventually
remit permanently.