Nephrotic syndrome

Syndrome nefrotik adalah kumpulan

gejala yang disebabkan oleh kelainan
glomerular dengan gejala edema,
proteinuria, hipoalbuminemia,
hiperkolesterolemia karena kondisi
rusaknya membran kapiler glomerolur
yang signifikan serta menyebabkan
peningkatan permeabilitas membran
glomerolus terhadap protein
 Kriteria NS

Tanda dan gelaja

*Massive proteinuria:
qualitative proteinuria: 3+ or 4+,
quantitative proteinuria : > 40 mg/m2/hr in children,
and > 3,5 g/day (adult)

*Hypo-proteinemia : total plasma proteins < 5.5g/dl and

serum albumin : < 2.5g/dl.

*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

Nephrotic Syndrome Nephritic Syndrome

 Massive proteinuria  Hematuria
 Hypoalbuminemia  Oliguria
 Edema  Azotemia
 Hyperlipidemia/-uria  Hypertension
 Minimal change disease

Minimal change disease Normal glomerular structure

 Histological patterns

Minimal change disease Normal glomerulus

 Pathogenesis of Proteinuria

 Meningkatnya permeabilitas dari glomerolus menyebabkan protein dapat

keluar dari glomerolus
 Degree of protineuria:-
 Mild : less than 0.5g/m2/day

 Moderate: 0.5 – 3 g/m2/day

 Severe: more than 3 g/m2/day

 Type of proteinuria:
 Selective proteinuria: where proteins of low molecular weight such
as albumin, are excreted more readily than protein of HMW
 Non selective : LMW+HMW are lost in urine
 Pathogenesis of Hyperlipidemia

 Hipoalbumin respon menyebabkan hati dengan reflex mensisntesis

LDL, VLDL dan lipoprotein lain. Lipoprotein yang memiliki BM tinggi
tidak tereliminasi pada urin sehingga terjadi hiperlipidemia

 Pengeluaran lipoprotein BM rendah seperti HDL

 Reaksi enzymatis yang menyebabkan abnormalitas syntesis lipid dan

kerusakan degradasinya.
 Pathogenesis of edema

 Reduksi koloid plasma menurunkan tekanan osmotik sehingga

menyebabkan intavaskular volume
 Intravascular volume↓ antidiuretic hormone (ADH ) and
aldosterone(ALD)  water and sodium retention Edema
 Intravascular volume↓ glomerular filtration rate
(GFR)↓ water and sodium retention  Edema
 Clinical Manifestation

The male preponderance of 2:1

1.Main manifestations:
Edema (varying degrees) is the common symptom
Local edema: edema in face , around
eyes( Periorbital swelling), in lower extremities
Generalized edema, edema in penis and scrotum.
2. Non-specific symptoms:
Fatigue and lethargy, loss of appetite, nausea and
vomiting ,abdominal pain , diarrhea, body weight
increase, urine output decrease, pleural effusion
(respiratory distress)
 Komplikasi NS
1. Infections: Infections is a major complication in children with NS.

Nephrotic patients are liable to infection because :

 loss of immunoglobins in urine.
 the edema fluid act as a culture medium.
 use immunosuppressive agents.
 malnutrition
Complication…..

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

Based on : proteinuria, serum protein, and resolution of symptoms,

there are several types of responses:
 Pengobatan komplit : tidak terjadi proteinuria, perbaikan serum
protein dan perbaikan simptom
 kambuh type I : masih terjadi proteeinuria meskipun kadar serum
protein normal dan gejala membaik
 kambuh type II : tidak terjadi perbaikan baik protein urin protein
serum maupun gejala-gejala yang dialami pasien
 Evaluation of treatment responses

 No response : no treatment response

 Relapse : After remission is achieved, a urinary protein level of
40 mg/m2/hr or more, or that of 100mg/dL or more determined
early in the morning using the test paper method persists for 3
days

• 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

 A calcineurin inhibitor (CNI) is recommended as initial therapy for children with

SRNS, CNI therapy be continued for a minimum of 6 months and then stopped if
a partial or complete remission of proteinuria is not achieved.
 Low-dose corticosteroid therapy be combined with CNI therapy treatment with
ACE-I or ARBs for children with SRNS.
 In children who fail to achieve remission with CNI therapy: mycophenolate
mofetil, high-dose corticosteroids, or a combination of these agents be
considered
 cyclophosphamide should be given to children with SRNS
Treatment
Tatalaksana untuk NS komplikasi
 Infeksi

 Thrombosis

 ARF（HD; corticosteroids, diuresis)

 dyslipidemia