Nephrotic Syndrome in Children

Syarifuddin Rauf,

Bagian Ilmu Kesehatan Anak RSU Wahidin

Sudirohusodo,Fakultas Kedokteran UNHAS

NEPHROTIC SYNDROME
1. Generalized oedema
2. Heavy proteinuria(>50mg/kgbb)
3. Hypoproteinemia(<2,5gr/dl)
4. Hyperlipidaemia(>250mg/dl)
(Hypercholestrolemia)
Tabel. Sebaran penyakit ginjal anak yang dirawat inap di BIKA FK
UNHAS RS Wahidin Sudirohusodo (2000 2004)

Jenis penyakit ginjal Jumlah %

Sindrom Nefrotik 90 39,0
Glomerulonefritis akut 71 30,7
Infeksi saluran kemih 32 13,9
Gagal ginjal akut 4 1,7
Gagl ginjal kronik 2 0,9
Tumor ginjal 20 8,7
Kelainan kongenital sal. Kemih 2 0,9
Batu saluran kemih 4 1,7
Nefritis Schoenlein Henoch 4 1,7
Nefritis Lupus 1 0,4
Proteinuria persistent 1 0,4
Jumlah 231 100
INCIDENCE
Wilawirya (1992): 6 cases/100.000 population < 14 yr old/yr

Sex ratio : : : = 1,5 2 : 1

Children:Adult = 15 : 1

Age incidence : - Highest Inc. = 2-5 years

- Less common : > 5 years

Department of Child Health, Hasanuddin University /

2
General Hospital Wahidin Sudirohusodo : 1-2 cases/month
ETIOLOGY

Unknown (idiopatik=primer)

Acquired(sekunder): Diabetic Mellitus

Genetic factors : - Congenital NS (mutation

on chromosom 19)

- HLA antigens : HLA-DR7

Predisposition : Allergy
3
CLASSIFICATION

Primary Glomerular diseases

1. Minimal Change NS (MCNS) 70-80%

2. Focal Segmental Glomerulosclerosis (FSGS)

3. Mesangiocapillary Glomerulonephritis

4. Membranous Nephropathy

Secondary diseases

1. Henoch-Schoenlein Purpura

2. Systemic Lupus Erythematosus

4
PATOMECHANISM

Soluble antigen- antibody complex

Electrochemic theory

5
CLINICAL MANIFESTATIONS

Congenital NS (Finlandia type)

Placenta enlargement
Massif oedema
Genetic mutation on chromosome 19
Steroid sensitive NS
Responsif to cortikosteroid
Minimal change NS (MCNS) : 70-80%
Steroid resistant
No/minimal response to cortikosteroid
Focal glomerulosclerosis (FSGS) 8
SYMPTOMS & SIGNS
1. Oedema :

Pitting oedema

Generalized : starting in periorbital regions

face abdomen (ascites) extremities

Pleural effusions

Massive anasarca scrotal or vulval oedema

2. No hypertension or hematuria

3. Normal renal function

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MANAGEMENT

1. Hypoalbuminaemia (< 2 gr%)

Salt-poor human albumin (plasbumin) 1 gr/kgBW
2. Febrile / feels unwell / abdominal pain :
Antibiotics
3. Diuretic : Indications : severe oedema that causes
dyspnoe
4. Specific treatment : corticosteroid
Protocol : International Study of Kidney Disease
in Children (ISKDC) 8
PROTOCOL THERAPY OF NS
ISKDC:
1. ISKDC

CD = 4 weeks

AD/ID = 4 weeks Tap. off

Stop
1 2 3 4 5 6 7 8
remission remission

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PROTOCOL THERAPY OF NS
ISKDC:
2. Arbeitsgemeinschaft fur Paediatrische Nephrologie (APN)

CD = 6 weeks

AD/ID = 6 weeks Tap. off

1 year
1 2 3 4 5 6 7 8 9 10 11 12

remission remission

9
PROTOCOL THERAPY OF RELAPS NS

CD

AD/ID Tap. off

Stop
1 2 3 4

remission remission

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 Any
Question
s?
 KESIMPULAN
1. SN pada anak bersifat idiopatik dan umumnya sensitif
kortikosteroid.
2. Pengobatan SN pada anak sebaiknya dimulai dengan
prednison/ prednisolon yang setelah mencapai remisi,
prednison / prednisolon dilanjutkan sampai mencapai
dosis threshold selama 6 12 bulan.
3. Bila terjadi relaps, apakah itu bersifat resistent atau
dependen steroid maka dapat diberikan obat-obat
immunosupresif lain, yang diberikan secara bersama-
sama dengan steroid atau sebagai single tratment.
4. Selain pemberian obat maka pada penatalaksanaan SN
harus diperhatikan terapi penunjang..
 Sindrom nefrotik relaps frekuen atau
dependen steroid

Prednison FD Remisi
Prednison AD + CPA

Diturunkan sampai dosis treshoid 0,1-0,5

mg/kgBB AD
6-12 bulan

Relaps pada Relaps pada

Prednison > 0,5 mg/kgBB AD prednison > 1 mg/kgBB AD
atau
Efek samping steroid meningkat
Levamisol 2,5 mg/kgBB AD
(4-12 bulan)
CPA 2-3 mg/kgBB
8-12 minggu

Relaps prednison standar

Relaps pada prednison

> 0,5 mg/kgBB AD
Gambar. Diagram pengobatan
sindrom nefrotik relaps frekuen atau
Siklosporin 5 mg/kgBB/hari
dependen steroid selama 1 tahun
 MCNS(100%)

R/ 8 mgg I Initial responder (93%) Initial nonresponder (7%)

6 bln (sesudah R/ 8
mgg) Late responder Late non-
(5%) responder
(2%)
Non relapser (36%) Infrequent Frequent
relapser (18%) relapser (39%)

Subsequent
nonresponder (5%)

Minimal Change NS(MCNS) after 8 weeks

treatment of corticosteroid