Lect 6-Sindrome Nefrotik
Lect 6-Sindrome Nefrotik
*Hyperlipidemia:
serum cholesterol : > 5.7mmol/L
*Edema
Klasifikasi
Syndrom nefrotik idiopatik (paling umum)
penyebab utama tidak diketahui dengan pasti, diduga adanya
kelainan fungsi sel T. jenis ini menyerang sekitar 90% pada anak
Secondary NS
NS ini diakibatkan adanya gangguan sistemik lain seperti reaksi
anafilaksis, lupus dll
Congenital NS (jarang terjadi)
*cacat bawaan pada 3 bulan pertama umur bayi dan biasanya harus
dilakukan transplantasi
Clinical syndrome
Type of proteinuria:
Selective proteinuria: where proteins of low molecular weight such as
2. Hypercoagulability (Thrombosis).
Hypercoagulability of the blood leading to venous or arterial thrombosis:
Hypercoagulability in Nephrotic syndrome caused by:
Higher concentration of I,II, V,VII,VIII,X and fibrinogen
Lower level of anticoagulant substance: antithrombin III
decrease fibrinolysis.
Higher blood viscosity
Increased platelet aggregation
3. ARF: pre-renal and renal
4. cardiovascular disease :-Hyperlipidemia, may be a risk
factor for cardiovascular disease.
5. Hypovolemic shock
6. Others: growth retardation, malnutrition,
adrenal cortical insufficiency
Treatments
Support care
Perawatan : menurunkan asupan protein(0.8-1.0g/kg/d); asupan garam
(<3g/d)
Terapi dengan obat-obatan diuretik
mengatasi proteinuria: ACEI and ARB
Albumin : indicated if
• hipovolumea
• edema
.
Farmakoterapi NS
Antiinflamasi dan depresan imun
Corticosteroid therapy
Cyclophosphamide
Cyclosporin A
Tacrolimus
Microphenolate
Lini Pertama untuk NS
prednison/prednisolone selama 12 minggu lebih disarankan untuk terapi
awal
Dosis prednisolone berdasarkan luas permukaan tubuh.
60 mg/m2/day for 4 weeks (maximum 80 mg)
40 mg/m2/on alternate days for 4 weeks (maximum 60mg)
Reduce dose by 5-10mg/m2 each week for another 4 weeks then stop
Evaluasi pengobatan
• Most children with nephrotic syndrome will respond to steroid treatment within
2-4 weeks.
• A remission is defined as 3 or more days
• Treatment is continued for a total of 12 weeks.
• If proteinuria persists beyond the first 4 weeks of steroid treatment, then
children should be referred for renal biopsy.
Treatments of relaps NS
For infrequent relapses of SSNS:
treated with a single-daily dose of prednisone 60mg/m2 or 2mg/kg (maximum of
60mg/d) until the child has been in complete remission for at least 3 days.
after achieving complete remission, children be given prednisone as a single dose
on alternate days (40mg/m2 per dose or 1.5mg/kg per dose: maximum 40mg on
alternate days) for at least 4 weeks.
Treatment for Steroid -Resistant NS
Thrombosis