Name : Prof.Dr.dr.

Syarifuddin Rauf SpA(K)

Position :
Senior staff of Pediatric Department

Lecturer:

1. Urogenitalia System ( Pediatric Nephrology )

2. Bioethics,Humaniora and HAM system

3. Phylosophy ( Combined degree )

 Nefrologi Anak
(Pediatric nephrology)
Prof. Dr. dr.Syarifuddin Rauf,
SpA(K)

Department of Child Health, Medical Faculty

Hasanuddin University, Makassar
Pediatric
Nephrology
TOPIK NEFROLOGI ANAK :

1. ISK
2. KELAINAN KONGENITAL SALURAN KEMIH

3. SINDROM NEFROTIK
4. GLOMERULONEFRITIS AKUT PASCA
STREPTOKOKUS

5. GANGGUAN GINJAL AKUT ( ACUTE KIDNEY

INJURY= AKI )
6. GANGGUAN GINJAL KRONIK ( CHRONIC KIDNEY
DISEASE )
7. TUMOR WILMS
 Edema dan proteinuria telah dikenal
bahkan sejak 200 tahun yang lalu

1827 1905 1929

Richard Bright Friedrich von Henry Christian
• BRIGHT’S Muller • Frasa ‘sindrom
DISEASE • Nefritis nefrotik’
• Edema • Nefrosis
proteinuria 
ginjal
Distribusi Frekuensi penyakit ginjal
periode Januari 2010-Desember 2012 , DIKA FK UNHAS

Distribusi Frekuensi penyakit ginjal

periode Januari 2010-Desember 2012

Sindrom nefrotik
SINDR NEFROTIK
TUMOR GNAPS,14,2%
GINJAL4,4%
20,4% SNA
KK2,7% Penyakit Ginjal kronik
SLE,3,06%
Cedera Ginjal Akut
SHS,2,7% Hipertensi
SNA 13,6 %
BSK,2,7% ISK
BSK
ISK PGK
5,78% SHS
12,2% HIPERTENSI AKI
SLE
5,4%
11,5% kel.kongenital
tumor
GNAPS
GGK + CAPD
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
 Distribusi Frekuensi penyakit ginjal anak
periode Januari 2010-Desember 2012

SINDR NEFROTIK
GNAPS,14,2%
TUMOR 20,4%
GINJAL4,4% Sindrom nefrotik
KK2,7% SNA
SLE,3,06%
Penyakit Ginjal kronik
SHS,2,7% Cedera Ginjal Akut
SNA 13,6 % Hipertensi
BSK,2,7% ISK
BSK
ISK
SHS
12,2% SLE
HIPERTENSI AKI
5,4% kel.kongenital
11,5% tumor
GNAPS
GGK + CAPD
NEPHROTIC SYNDROME
1. Generalized oedema
2. Heavy proteinuria(>50mg/kgbb)
3. Hypoproteinemia(<2,5gr/dl)
4. Hyperlipidaemia(>250mg/dl)
(Hypercholestrolemia)
NEPHROTIC SYNDROME
1. Edema masif
2. Proteinuri berat (>50mg/kgbb)
3. Hypoproteinemia(<2,5gr/dl)
4. Hyperlipidaemia(>250mg/dl)
(Hypercholestrolemia)
edema pretibiall
edema
Abdominal striae and pitting edema
 SINDROM NEFRITIK(AKUT)
1.PROTEINURI
2.HEMATURI
3.HIPERTENSI
4.OLIGOURI
5.AZOTEMIA
6.TORAK ERITROSIT
7.EDEMA

SINDROM NEFROTIK
1.EDEMA MASIF(ANASARKA)
2.PROTEINURI
3.HIPOPROTEINEMI
4.HIPERKOLESTROLEMI
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

9
 Minimal
Histopatogis

Non Minimal

Sensitif Steroid
Klasifikasi Respon terhadap
Sindrom nefrotik pengobatan

Resisten steroid
Kongenital

Idiopatik

Etiologi Sekunder
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, kegiatan berlebih

3
 SOLUBLE ANTIGEN-AB COMPLEX

INCITING FACTOR (ANTIGEN)


IgG + Antigen complexes ( SAAC )

Deposite in glom. basement membrane (gbm)

Activate complement

Immune complexes in gbm (Antigen,IgG+C3)

Renal symptoms
PATOMECHANISM

Soluble antigen- antibody complex

Electrochemic theory

5
CLINICAL MANIFESTATIONS

Congenital NS (Finlandia type)

Placenta membesar
Massif oedema
Mutasi genetik pada chromosome 19
Steroid sensitive NS
Responsive to corticosteroid
Minimal change NS (MCNS) : 70-80%
Steroid resistant NS
No/minimal response to corticosteroid
Focal glomerulosclerosis (FSGS) 8
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

1- 2 thn
1 2 3 4 5 6 7 8
remission remission

CD :continuous day
AD : alternating day
ID : intermittent day
9
PROTOCOL THERAPY OF RELAPS NS

CD(2mg/kgbb)

AD/ID(2/3 dosis CD) Tap. off

CD until remission
( 1 - 4 minggu )
1 year
1 2 3 4

remission remission

10
• Proteinuria (-)/trace • Proteinuria ≥ +2 • Relaps terjadi < 2 x
(proteinuria < 4 (proteinuria > 40 dalam 6 bulan
mg/m2/jam) 3 hari mg/m2/jam) 3 hari pertama setelah
berturut-turut dalam 1 berturut-turut dalam 1 respons awal atau < 4
minggu minggu kali pertahun
pengamatan

Relaps
Remisi Relaps jarang

• Relaps ≥ 2 x dalam 6 • Relaps ≥ 2 x berurutan • Tidak terjadi remisi

bulan pertama setelah pada saat dosis steroid pada pengobatan
respons awal atau ≥ diturunkan prednison dosis penuh
4 kali dalam periode 1 (alternating) atau (full dose) 2
tahun. dalam 14 hari setelah mg/kgbb/hari selama
pengobatan dihentikan 4 minggu/8 miggu

Relaps Dependen Resisten

frekuen steroid steroid
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
 Klasifikasi
Respon
terhadap Histopatologi
steroid

Sensitive Minimal

Non
Resisten minimal 
>> FSGS
Barratt TM. Steroid responsive nephrotic syndrome. In: Barratt TM, editor. Pediatric nephrology. 4th
edition. Baltimore: Lippincot Wiliams & Wilkins;1999. p. 732.
 Any
Question
s?
 Batasan SNRS

Resisten steroid: tidak terjadi remisi

pada pengobatan prednison dosis
penuh (full dose) 2 mg/kgbb/hari
selama 4, 4-6, 8 minggu.

KDIGO Glomerulonefritis working group, 2012:

• SNRS steroid primer = non responder awal:
tidak
remisi total setelah 8 mg terapi steroid
• SNRS sekunder = non responder kasep:
proteinuria persisten dalam terapi steroid 4 mg,
setelah satu atau lebih episode remisi
 lesi minimal/minimal change disease
proliferasi difus
mesangial/diffuse mesangial
Focal segmental glomerulus ( 75 % )

mesangioproliferatif
glomerulonefritis (MPGN)
membranous glomerulonephritis (MGN).
Sebagian besar penderita SNRS dengan
biopsi ginjal pertama menunjukkan lesi
minimal, setelah pengamatan selanjutnya
menunjukkan perubahan morfologi
 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
 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
 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..
ACUTE POST STREPTOCOCCAL
GLOMERULONEPHRITIS

GLOMERULONEFRITIS AKUT
PASCA STREPTOKOKKUS
(GNAPS)
 GNAPS

Proliferasi dan inflamasi glomeruli

Sekunder oleh mekanisme imunologik

Antigen: streptokokus B hemolitikus grup A

 SINDROM NEFRITIK(AKUT)
1.PROTEINURI
2.HEMATURI
3.HIPERTENSI
4.OLIGOURI
5.AZOTEMIA
6.TORAK ERITROSIT
7.EDEMA

SINDROM NEFROTIK
1.EDEMA MASIF(ANASARKA)
2.PROTEINURI
3.HIPOPROTEINEMI
4.HIPERKOLESTROLEMI
 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

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 makroskopik warna kemerah-merahan

seperti koka kola, hematuri mikroskopik

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 TRACT INFECTION
(UTI)
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

2
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 pain dysuria,

frequent voiding etc.

 PATHOGENESIS

Neonates Baby & Child

(>1 month)

Colonization on GIT

♀: Periurethra/Perineum
Hematogen Percontinuitatum
Certain focus
(Septicemia) (“Ascending”) ♂: Subpreputium

Bacteria enter to
Urinary tract

Symptomatic UTI Asymptomatic UTI

 LAB. EXAMINATIONS

URINE :
1. Urinalysis : Leukocyte > 5-10/HPF
Erythrocyte : +/-
2. Urine culture :
a. Midstream 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)

 FAKTOR PREDISPOSISI ISK
ANAK PEREMPUAN
ANAK LAKI TIDAK DISIRKUMSISI
OBSTIPASI KRONIK
INSTRUMENTASI URETRA
KATETER BULI-BULI JANGKA PANJANG
INFESTASI CACING KREMI
MEMBERSIHKAN FESES : BAWAH  ATAS
MANDI BUSA

63
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
TOPIK :
1.SINDROM NEFROTIK
2.GNAPS
3.ISK

4.KELAINAN KONGENITAL
SALURAN KEMIH ( KKSK )

5.ACUTE RENAL FAILURE =

ACUTE KIDNEY INJURY
(GAGAL GINJAL AKUT )

6.GAGAL GINJAL KRONIK

(PENYAKIT GINJAL KRONIK )
KIDNEY :
1. AGENESIS : BILATERAL RENAL AGENESIS

= Potter’s Syndrome

Oligohydramnion

Pulmonary hypoplasia

Low-set ears

2. RENAL HYPOPLASIA : The kidney is small

Normal nephron
Renal agenesis (Potter’s
Potter syndrome

sequence)
 Renaldysplasia
Renal
R dysplasia
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
 PolycysticKidneys
Polycystic kidneys
IPCK

Autosomal Recessive Polycystic Kidney

Enlargement of distal tubulus & colligents

ductus

Glomerulus & proximal tubulus normal

Liver enlargement
BLADDER (VESICA URINARIA)
Agnesia

Bladder neck obstruction

URETHRA

Agnesia / atresia urethra

Congenital posterior urethral valves

Posterior Urethral Valves(PUV)
PUV
URETER

Duplication of ureter

Pelvic ureteral junction obstruction

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

Prune Belly
Prune Syndrome
Belly Syndrome
THANK YOU
GANGGUAN GINJAL AKUT = ACUTE KIDNEY INJURY

( GAGAL GINJAL AKUT = ACUTE RENAL FAILURE )

 GGA (ARF)  GgGA (AKI)
PERUBAHAN 
ADQI: 2001 KONSEP
2007

Kenaikan Cr serum minimal  prognosis buruk

Definisi harus mampu mencakup deteksi dini

“injury” lebih mencerminkan patobiologi kelainan ginjal

dibanding istilah “failure” atau “gagal”

“kidney” dlm bhs Inggris lebih mudah dimengerti

dibandingkan “renal”
Sudden loss or decreased of kidneys

function

Imbalance of water & electrolyte blood

Increased of waste products (Ureum &

Creatinin)
ETIOLOGY:

A. Prerenal: Decrased blood flow

Diarrhea dehydration

Hemorrhage

Burns

Septic shock
B. Renal:

Acute Tubular Necrosis

Acute Nephritic Syndrome

Nephrotic syndrome

Acute Pyelonephritis
C. Postrenal: Urinary tract obstruction

Tumors

Kidneystones

Nephrocalcinosis
 Etiology of AKI in Newborn:
1. Prerenal:

Perinatal hemorrhage (birth trauma, placenta

abruption)

Neonatal hemorrhage (severe intraventricular

hemorrhage, adrenal hemorrhage)

Perinatal asphyxia

Hyaline membrane disease

2. Renal :

Acute Tubular Necrosis (perinatal asphyxia)

Maternal-fetal transfer:

Antibodies

Infections: syphilis, Cytomegalovirus

3. Postrenal : Congenital malformations of urinary

collecting system
CLINICAL MANIFESTATIONS:

1. Decreased urine volume (Oligouria  Anuria)

2. Generalized swelling
3. Changes in mental status:
Unconscious
Delirium/confusion
Coma
4. Seizures
5. Nausea, vomiting
6. Anemia
7. Kusmaul respiration
DIAGNOSIS

Clinical Manifestations

Lab. Examination:

Serum Ureum increased

Serum Creatinin increased

Creatinin Clearance ↓

Serrum Potasium ↑
TREATMENT
1. The goal of the treatment is to identify & treat

reversible causes like prerenal causes

(diarrhea dehydration) & post renal causes

(e.g. obstructive uropathy by surgeon

procedure)

2. Medical drugs:

Antibiotics: To prevent infection

Diuretics: to treat oligouria or anuria

Diazepam: To handle convulsion

3. Emergency condition:

Hyperkalemia:

Ca Glukonas

Potassium exchange resin

(Kayexalate) Oral/rectal

Metabolic acidosis: Bicarbonate Natricus

 Simple assessment of GFR in children
• Schwartz formula:

• GFR= body height (cm) x K

Pcr (mg/dL)

K= 0.45 for babies < 1 year old

K= 0.55 for boys and girls 1-13 year old
K= 0.57 for girls > 13 year old
K= 0.70 for boys > 13 year old

• This equation gives the GFR in ml/min./1.73 m2 BSA The

problem is, the Schwartz formula does not work so good in
children with very high plasma creatinine levels.
Normal range of GFR in children
Age GFR (ml/min/1.73 m2)
Birth 20.8 ± 1.9
1 week 46.6 ± 5.2
3-5 week 60.1 ± 4.6
6-9 week 67.5 ± 6.5
3-6 month 73.8 ± 7.2
6-12 month 93.7 ± 14.0
1-2 year 99.1 ± 18.7
2-5 year 126.5 ± 24.0
5-15 year 116.7 ± 20.2
(Alatas H, 2002)
11
 Acute kidney injury

Kriteria RIFLE
Kellum JA, Bellomo R, Ronco C. The concept of acute kidney injury and the RIFLE criteria. In: Ronco C, Bellomo R, Kellum JA,
97
eds. Acute Kidney Injury. Contrib Nephrol. Basel: Karger. 2007; 10-6
 Dialisis

Indikasi dialisis pada anak dengan AKI adalah :

1. Tahap Failure dari kriteria RIFLE
2. Hiperkalemia > 7,5 mEq/l
3. Bikarbonat serum < 12 mEq/l
4. Adanya gejala-gejala overhidrasi : edema paru,
dekompensasi jantung, dan hipertensi yang tidak dapat
diatasi dengan obat-obatan.
5. Perburukan keadaan umum dengan gejala uremia berat :
perdarahan, kesadaran menurun sampai koma.
 PROGNOSIS
• Angka kematian pada AKI tergantung kepada
penyebabnya, umur pasien dan tahapan AKI

• Prognosis pasien AKI juga ditentukan oleh lama

terjadinya gagal ginjal sampai intervensi terapi
diberikan.

• Meskipun AKI yang terjadi dikatakan ringan, angka

kematian mencapai 30-60%, dan jika pasien tersebut
memerlukan terapi dialisis, maka angka kematian
meningkat sampai 50-90%.
 CAUSES OF AKI IN INFANTS AND CHILDREN

•Vasomotor nephropathy
Pre • Ischemic : hypotension, hypoxia, cardiac failure,
sepsis, intestinal obstruction
Renal • Toxic : aminoglycosides, radiocontrast agents

• Glomerular : AGN, crescentic GN

• Vascular : HUS, renal A/V thrombosis, LN
Renal • Interstitial : pyelonephritis, interstitial
nephritis
• Tubular : ATN, toxin

• Structural : PUV, ureteric obstruction,

Post neurogenic bladder
Renal • Crystalluria : tumor lysis syndrome
• Calculi
17
• Pediatric-modified RIFLE (pRIFLE)(2007)

eCCl ~ Schwartz
= k x Ht / SCr

(Akcan-Arikan et al, 2007) 9

Renal function progresses to decrease

slowly

Imbalance of water & electrolyte

Increased of waste products (Blood

ureum & creatinin)

ETIOLOGY:

< 5 years old:

Hypoplasia / Dysplasia kidney

Congenital structure of urinary tract

Vesicoureteral reflux

Congenital Nephrotic Syndrome

5 – 15 years old :

Hereditary diseases: Alport’s syndrome,

sistinuri

Primary glomerulonephritis: Nephritic

Syndrome

Secondary glomerulonephritis : SLE,SHS

VASCULUS :
VASCULITIS
PEMBULUH DARAH
ITIS : RADANG
VASCULITIS : PERADANGAN PEMB,DARAH
(AORTA SAMPAI KAPILER)

CONTOH : - PENYAKIT KAWASAKI

- POLYARTERITIS NODOSA
- SCHOENLEIN HENOCH SYNDROME
- SYSTEMIC LUPUS ERYTHEMATOSUS

PENYEBAB : PROSES IMUNOLOGIK

SHS : Systemic disease

Vasculitis on :

Skin  purpura

Joint  arthritis

Digestive tract  abdominal pain

Kidney  glomerulonephritis
INCIDENCE

Age incidence :

All ages

75% of cases : 2-11 years

Sex incidence : ♀ : ♂ = 2 : 1

50% of cases : preceded by upper respiratory

infection group A streptococci

 CLINICAL MANIFESTATIONS

1. PURPURA :
Erythematous macular  palpable purpura
Ecchymotic
Associated with subcutaneous edema
(extremities, scalp, periorbital region,
hands, feet, scrotal area)
Lower legs, ankles, dorsal foot ( symmetric)
2. Gastrointestinal Symptoms:

Abdominal pain : 35-85% of cases

Gastrointestinal bleeding: melena,

hematemesis, bloody stool

Invagination, intestinal perforation

3. Joint Symptoms:

Arthritis

Arthralgia

Ankles & knees: most commonly

affected

The three signs: Classic triad of SHS

 PATHOPHYSIOLOGY

INCITING FACTOR (URI→Streptococcus)


IgA complexes (IgA1 & IgA2)

Deposite in glom. basement membrane (gbm)

Activate complement

Immune complexes in gbm (IgA,IgG+C3)

Renal symptoms
RENAL INVOLVEMENT (SHS NEPHRITIS)

Haematuria associated with proteinuria

Acute Nephritic Syndrome

Nephrotic Syndrome

Renal Failure
 SY
SYSTEMIC LUPUS ERYTHEMATOSUS
SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)

Autoimune disorder
Damage to: Joints
Heart
Lungs
Blood vessels
Kidneys
Skin
 GENERAL SYMPTOMS

Fever
Malar rash: A rash shaped like butterfly
Usually found on the bridge
of the nose & cheeks
Inflammation of the joints (arthritis)
Anorexia
Weight loss
Anemia
DIAGNOSIS

Clinical Manifestations

Lab. Examination:

Blood: Anti Nuclear Antibody (+)

LE cell (+)

Ds-DNA (+)

C3 ↓ & C 4 ↓
ETIOLOGY :

1. Unknown

2. Genetic factor :

HLA Antigen (HLA-DR2, HLA-DR3)

High incidence in monozygotic twin

High incidence in family

3. Nongenetic factor:

Longterm treatment of certain drugs

(>6 months) → hydralazine

Sex hormone: estrogen → SLE (♀>♂)

Viral infection
CLINICAL MANIFESTATIONS:
RENAL SYMTOMPS

1. PROTEINURIA:

(+)→(++) (30-100 mg/dl)

Haematuria (+)/(-)

2. ACUTE NEPHRITIC SYNDROME

3. NEPHROTIC SYNDROME
PATHOGENESIS

Autoimune process
↓
Antinuclear Antibody (ANA)
+ Ds-DNA (Antigen)
↓
Soluble Ag-Ab Complex
↓
MBG
↓
Deposition of: ANA + Ds-DNA + C3
↓
Renal symptoms
CLASIFICATION HISTOPATHOLOGIC

I. Normal histopathologic feature

II. Mesangial Glomerulonephritis

III. Focal Segmental Proliferative Glomerulonephritis

IV. Diffuse Proliferative Glomerulonephritis

V. Membranous Glomerulonephritis
TREATMENT

Class I & II : Symptomatic treatment

No specific treatment

Class III,IV&V: Symptomatic treatment

Specific treatment

Corticosteroid

Immunosuppressive
MANAGEMENT

1. Self Limiting Disease

2. Supportive & symptomatic Treatment:

Fluid intake

Antihypertensive  Hypertensive patient

Diuretic  Oligouria

Antibiotic  Infection

Analgesics  Arthralgia
3. Specific treatment:

Corticosteroid (Prednison 1-2 mg/kgBW)

Severe Abdominal pain

GI bleeding

Renal treatment

R/ Acute Nephritic syndrome

Nephrotic Syndrome

Renal Failure
Complication of SLE

Damage to glomerulus

Progressive loss of kidney function

KIDNEY :
1. AGENESIS : BILATERAL RENAL AGENESIS

= Potter’s Syndrome

Oligohydramnion

Pulmonary hypoplasia

Low-set ears

2. RENAL HYPOPLASIA : The kidney is small

Normal nephron
IPCK

Autosomal Recessive Polycystic Kidney

Enlargement of distal tubulus & colligents

ductus

Glomerulus & proximal tubulus normal

Liver enlargement
URETER

Duplication of ureter

Ureterocele

Ectopic ureter
BLADDER (VESICA URINARIA)
Agnesia

Bladder neck obstruction

URETHRA

Agnesia / atresia urethra

Congenital posterior urethral valves

BLADDER (VESICA URINARIA)
Agnesia

Bladder neck obstruction

URETHRA

Agnesia / atresia urethra

Congenital posterior urethral valves

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