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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.
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 nephri-tis. 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 cortico-
steroids 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 permea- bility to plasma proteins. ▲Foot processes of the visceral epithelium of the GBM.
1.The construction of the
glomerular basement memb-
rane has changed.
2.The loss of the negative
charges on the GB
M.
◆The underlying pathoge-
nesis is unknown, but evidence
strongly supports the impor- t
ance of immune mechanisms
(P328).
Pathophysiology
1.Proteinuria: Fundamental
and highly important change of
pathophysiology.
2.Hypoproteinemia (mainly
albumin)
3.Edema: Nephrotic edema
(pitting edema)
Hypoproteinemia plasma
oncotic pres
sure is diminished, result in a
shift of fluid from the vascular
to the interstitial compartmen
t and plasma
volume↓→the activation of
the renin–
angiotensin–aldo-
sterone system→ tubular
sodium chloride reabsorp-
tion↑.
4. Hyperlipidemia (Hyper-
cholesterolemia)
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, an
orexia, fatigue,
abdominal pain, diarrhea.
Laboratory Exam 1.Urinary protein: 2+~ 4+
24hr total urinary protein
> 0.1g/kg.
( The most are selective p
roteinuria. )
May occur granular and red
cell casts.
2.Total serum protein↓,
< 30g/L .
Albumin levels are low (< 2
0g/L).
3.Serum cholesterol and
triglycerides:
Cholesterol > 5.7mmol/L
(220mg/dl).
4. ESR↑> 100mm/hr.
5.Serum proteins electro-
phoresis :
Albumin↓, α2-G↑,γ-G↓,
A/G inversion.
6.Serum complemen: Vary w
ith clinical type.
7.Renal function:
Complications
1.Infections
Infections is a major compli-
cation in children with NS. It
frequently trigger relapses.
Site: Respiratory tract, skin,
urinary tract and acute pri-
mary peritonitis.
Causes: Immunity lower ,
severe edema→malcirculation,
protein malnutrition, and use
hormone and immunosuppre-
ssive 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
1.1 Rest
1.2 Diet
Hypertension and edema: L
ow salt diet (<2gNa/ day) or sa
lt-free diet.
Severe edema: Restricting
fluid intake.
Increase proteins properly:
2g/(kg·day)
While undergoing the corti-
costeroid 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 · da
y)
* Triamterene
Attention: Volume
depletion, disorder of
electrolyte and embolism.
Apparent edema:
Give low molecular dextran
10~15ml/(kg·time);
[+Dopamine 2~3ug/(kg·min)
and/or Regitine 10mg +Lasix
1~2mg/kg].
2.Corticosteroid therapy
Short-course therapy:
Prednisone 2mg/(kg·day) or
60mg/m2/day (Max.60mg/day)
in 3 or 4 divided doses for 4wk
→maintenance treatment:
Prednisone 1.5mg/kg, single
dose for every-other day×4wk.
▲Total course of therapy: 8
wk.
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
②After maintenance
treatment:
Prednisone 2mg/kg , single
dose for every-other-day×4wk
tapered gradually (2.5~5
mg/2wk) discontinued.
▲Total course of treatment :
★Middle: 6mo
★Long: 9~12mo
Estimate of curative effect (P
331).
3. Treatment of relapse and rec
urrence
3.1 Extend the course of corti-
costeroid
3.2 Immunosuppressive agents
(Cytotoxic agents):
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.
② CB (Chlorambucil)
0.2mg/kg for 8wk .
Total amount : 10mg/kg
③ 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
0.5~0.75mg/m2 + NS/GS iv
drip (1hr), give liquid 2,000ml
/(m2.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/(kg·day) for
6mo.
5.Alleviar proteinuria
Angiotensin converting en-
zyme inhibitions (ACEI) :
Captopril, Enalapril and
Benazepril.
Prognosis Most cases of minimal
change disease eventually
remit permanently.