Nefrologi Anak

Prof. Dr. dr.Syarifuddin Rauf, SpA(K)

Bagian Ilmu Kesehatan Anak FK - UNHAS

RS Dr. Wahidin Sudirohusodo Makassar

Nephrotic Syndrome in Children

Syarifuddin Rauf
Department of Childhealth, Medical Faculty Hasanuddin University

Pediatric
Nephrology

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

1,7

Gagl ginjal kronik

0,9

Tumor ginjal

20

8,7

Kelainan kongenital sal. Kemih

0,9

Batu saluran kemih

1,7

Nefritis Schoenlein Henoch

1,7

Nefritis Lupus

0,4

Proteinuria persistent

0,4

231

100

Jumlah

NEPHROTIC SYNDROME
1. Generalized oedema
2. Heavy
proteinuria(>50mg/kgbb)
3. Hypoproteinemia(<2,5gr/dl)
4. Hyperlipidaemia(>250mg/dl)
(Hypercholestrolemia)

1. Edema Masif
2. Proteinuri Masif
3. Hipoproteinemi (< 2,5 g/dl)
4. Hiperkolesterolemi
(>250 mg/dl)

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 /
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
3

PATOMECHANISM
Soluble antigen- antibody complex
Electrochemic theory

PATHOPHYSIOLOGY
INCITING FACTOR (URIStreptococcus)

IgG + Antigen complexes

Deposite in glom. basement membrane (gbm)

Activate complement

Immune complexes in gbm (Antigen,IgG+C3)

Renal symptoms

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)

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

Abdominal striae and pitting edema

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)

PROTOCOL THERAPY OF NS
1. ISKDC:
ISKDC
CD = 4 weeks
Tap. off

AD/ID = 4 weeks

1- 2 thn
1

5
remission

8
remission

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

CD = 6 weeks
AD/ID = 6 weeks

4 5

6 7

remission

9 10 11 12

Tap. off

1 year

remission

PROTOCOL THERAPY OF RELAPS NS

CD
Tap. off

AD/ID

CD until remission
( 1 - 4 minggu )

1
remission

1 year

4
remission

10

Any
Questions
?

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 obatobat 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 0,10,5 mg/kgBB AD
6-12 bulan

Relaps pada
Prednison > 0,5 mg/kgBB AD

Levamisol 2,5 mg/kgBB

AD
(4-12 bulan)

Relaps pada
prednison > 1 mg/kgBB AD
atau
Efek samping steroid meningkat

CPA 2-3 mg/kgBB

8-12 minggu
Relaps prednison
standar

Gambar. Diagram pengobatan

sindrom nefrotik relaps frekuen atau
dependen steroid

Relaps pada prednison

> 0,5 mg/kgBB AD
Siklosporin 5
mg/kgBB/hari
selama 1 tahun

MCNS(100%)

Initial responder (93%)

R/ 8 mgg I

6 bln (sesudah R/ 8
mgg)
Non relapser (36%)

Initial nonresponder (7%)

Late responder
(5%)
Infrequent
relapser (18%)

Frequent
relapser (39%)

Subsequent
nonresponder (5%)

Minimal Change NS(MCNS) after 8 weeks

treatment of corticosteroid

Late nonresponder (2%)

ACUTE POST STREPTOCOCCAL

GLOMERULONEPHRITIS

GLOMERULONEFRITIS AKUT
PASCA STREPTOKOKKUS (GNAPS)

BEBERAPA ISTILAH

Glomerulonefritis Akut (GNA) : Suatu bentuk

proses proliferasi dan inflamasi glomeruli
yang didahului oleh suatu proses infeksi dan
terjadi secara akut.( contoh : GNAPS )

Sindrom Nefritik Akut (SNA ) :

Suatu kumpulan gejala-gejala klinik berupa
proteinuria, hematuria azotemia, redblood
cast
( torak eritrosit ), oligouria dan hipertensi
(PHAROH)

ISTILAH GNA dan SNA

GNA lebih bersifat histologik

( proliferasi dan inflamasi sel-sel
glomerulus)
* SNA lebih bersifat klinik ( PHAROH)

Salah satu bentuk SNA/GNA adalah

GNAPS

Proliferasi
dan inflamasi glomeruli
GNAPS

Sekunder oleh mekanisme imunologik

Antigen: streptokokus B hemolitikus grup A

1. Angka kejadian
Lebih sering umur 6-7 thn, jarang < 3 thn
Laki laki > perempuan (2:1)
10- 12 % kasus infeksi strept. hemolitikus
grup A
Kaplan: 50% kasus asimtomatik pd epidemi
GNAPS didahului ISPA atau piodermi

2. Etiologi
Streptokokus hemolitikus grup A (tipe M)
NEFRITOGENIK

Faringitis (serotipe tersering 12, lalu 1,3,4,6,25)

Piodermi (serotipe tersering 49, lalu 2,53,55,
56,57,58,60

MANIFESTASI KLINIK
a. Periode latent: 1 3 minggu
b. Edema
c. Hematuri
d. Hipertensi
e. Oligouria
f. Gejala-gejala lain: lelah, malaise, letargi
& anoreksia
g. Kelainan laboratorium

URIN:
Hematuri, warna kemerah-merahan atau
seperti air daging
Proteinuri : kualitatif dan kuantitatif
> 6 bulan

proteinuri persisten

biopsi ginjal

DARAH:
Titer ASTO meningkat
Menurunnya kadar C3
LED meninggi
Hipoproteinemi ringan
Pemeriksaan bakteriologik

Diagnosis GNAPS
Bila memenuhi 4 gejala berikut
Hematuri makroskopik atau mikroskopik
Edema
Hipertensi
ASTO meningkat
C3 menurun

PENGOBATAN

ANTIBIOTIK :
Bila dijumpai tanda-tanda infeksi
PENGOBATAN SIMTOMATIK :
- Diuretik : + oligouri
+ edema pulmonum
- Antihipertensi

KOMPLIKASI YANG TERJADI PADA

GNAPS DAN SN:
1.HIPERTENSI ENSEFALOPATI
2. EDEMA PARU
3. SYOK HIPOALBUMINEMI
4. GAGAL GINJAL

PROGNOSIS

BAIK
BAIK
: Self limiting disease
JELEK : Bila ada komplikasi yang tidak

dapat diatasi :
- gagal ginjal akut
- edema pulmonum
- ensefalopati hipertensi

URINARY
URINARY TRACT
TRACT INFECTION
INFECTION
(UTI)
(UTI)

DEFINITION
DEFINITION
Infection from renal parenchyme
orificium urethrae externa
Significant bacteriuria
With or without symptoms

Significant
bacteriuria
Pathogenic bacteria
Colony count : > 100.000/ml urine
> 1x lab. examinations

Relapsing UTI :
Recurrent UTI
Same microorganism
Reinfection UTI :
Recurrent UTI
Different microrganism
3

ETIOLOGY
Bacteria :
E. Coli
Klebsiella
Proteus
Pseudomonas
Other microorganisms :
Protozoa
Virus

CLASSIFICATION

Clinically : 1. Symptomatic UTI

2. Asymptomatic UTI
Complication :
A. Simple UTI
B. Complicated UTI
Localization : 1. Upper UTI
2. Lower UTI

PATHOGENESIS

1. Hematogenic
2. Percontinuitatum
3. Lymphogenic

DIAGNOSIS

Clinically :
1. Upper UTI (Pyelonephritis) :
Fever, back/flank pain & with or
without lower UTI symptoms
2. Lower UTI (Cystitis) :
Suprapubic punction, dysuria,
frequent voiding etc.

PATHOGENESIS
Neonates

Baby & Child

(>1 month)

Colonization on GIT
Certain focus

Hematogen
(Septicemia)

Percontinuitatum
(Ascending)

: Periurethra/Perineum
: Subpreputium

Bacteria enter to
Urinary tract

Symptomatic UTI

Asymptomatic UTI

LAB.
LAB. EXAMINATIONS
URINE :
1.

Urinalysis :

Leukocyte > 5-10/HPF

Erythrocyte : +/-

2.

Urine culture :
a.

Mid : stream urine :

C.C. : > 100.000/ml urine

b.

Catheterization :
C.C. : > 10.000/ml urine

c.

Suprapubic punction :
C.C. : > 1000/ml urine

BLOOD :
Leucocytosis
Increased BSR (> 30 mm/hour)
Increased CRP (> 30 ug/ml)

MANAGEMENT
1.

Eradicate acute infection

2.

Detection, prevention, & treatment

recurrent infection

3.

Detection & surgical correction

abnormality of anatomical structure

Eradicate acute infection

Oral antibiotics : Amoksisilin,Sulfonamid,sefalosporin

Parenteral antibiotics: ceftriakson, sefotaksim,

seftazidim, gentamisin
Amikasin

CONGENITAL ANATOMY
OF URINARY TRACT

KIDNEY :
1.

AGENESIS : BILATERAL RENAL AGENESIS

= Potters Syndrome
Oligohydramnion
Pulmonary hypoplasia
Low-set ears

2.

RENAL HYPOPLASIA :

The kidney is small

Normal nephron

Potter syndrome

Renal agenesis (Potters

sequence)

Renal Renal
dysplasia
dysplasia
R
E

3.

Horseshoe kidney :
Fusion of the renal parenchyma
Joined at the lower pole

4.

Polycystic kidney :
a.

Infantile Polycystic Kidney (IPCK)

b.

Adults Polycystic Kidney (APCK)

Horseshoe kidny

HORSHOE KIDNEYIDNEYHO

Polycystic
Kidneys
Polycystic
kidneys

BLADDER (VESICA URINARIA)

Agnesia
Bladder neck obstruction

URETHRA
Agnesia / atresia urethra
Congenital posterior urethral valves

PUV

Posterior Urethral Valves(PUV)

Prune Belly Syndrome

Prune Belly Syndrome

URETER
Duplication of ureter
Ureterocele
Ectopic ureter

Ureteric
Duplication
Ureteric
duplication

PUJ obstruction

Pelvic Ureteral Junction Obstruction

VESICO URETERAL REFLUX

Reflux of urine from the bladder into ureter
Damage the upper urinary tract by bacterial
Infection
Causes :

Congenital anomalous development

of the ureterovesical junction

Bladder outlet obstruction

IPCK
Autosomal Recessive Polycystic Kidney
Enlargement of distal tubulus & colligents
ductus
Glomerulus & proximal tubulus normal
Liver enlargement

Thank You