NEFROLOGI ANAK

PENYAKIT GLOMERULUS

POLI KLINIK :
1. NEPHROTIC SYNDROM (NS)
2. ACUT GLOMERULO NEPHRITIS (AGN)
3. MIXED NEPHRITIC-NEPHROTIC (MN)
4. ACUT RENAL FAILURE (ARF)
5. CHRONIC RENAL FAILURE (INSUFI CIENCY)
6. RECURRENT OR PERSISTENT HEMATURIA
7. A SYMTOMATIC PROTEINURIA
 NEPHROTIC SYNDROME
DEFINISI :
KEADAAN KLINIS DITANDAI DGN
PROTEINURIA, HIPOALBUMINEMIA, UDEMA
&HIPERCHOLESTEROLEMIA.

KADANG DISERTAI : HEMATURIA, HIPERTENSI

DAN PENURUNAN GFR.

KLASIFIKASI :
1. PRIMARY NS - PENY. PRIMER DI GML.
2. SECONDARY NS-PENY. DI GLM,
AKIBAT KELAINAN DILUAR GLM.
3. CONGENITAL NS.

SECONDARY NS :
PENYEBAB TERBANYAK :
1. S L E
2. ANAPHYLACTOID PURPURA (SHONLEIN -
HENOCH SYNDROME)
3. SICKLE CELL DISEASE
4. SYPHILIS
5. MALARIA (QUARTANA)
6. BEE-STING
7. DRUGS & TOXIN : (GOLD, CAPTO PRIL,
HEROIN, POISON)
PRIMARY NS :
HYSTOPATHOLOGIC CATEGORIES, BY
INTERNATIONAL STUDY OF KIDNEY DISEASE
IN CHILDREN (ISKDC)

CATEGORY NO OF PATIENT %
1. MINIMAL CHANGE 391 75,1
2. MEMBRANO PROLIFERATION 39 7,5
3. FOCAL AND SEGMENTAL
SCLEROSIS 41 7,9
4. PROLIFERATIVE GN 14 2,7
5. DIFFUSE MESANGIAL HIPER
CELLULARITHY 10 1,9
6. FOCAL AND GLOBOID
SCLEROSIS 9 1,7
7. MEMBRANEUS
GLM NEPHROPATHY 7 1,3
8. CHRONIC GN 4 0,8
9. UNCLASSIFIED 6
--------
521
1. MESANGIAL CELL
2.1ENDOTHELIAL CELL
3. VISCERAL EPITHELIAL CELL
4. PARIETAL EPITHELIAL CELL
5. MESANGIAL MATRIX
6. GLOMERULAR BASAL LAMINA
7. BOWMANS BASAL LAMINA
8. PODOCYT

PADA PERMULAAN PENY.

A. DIBIOPSY : 8O % MCN
B. DIOBATI DGN CORTICO STEROID 80 %
MPGN
- PERLU DOSIS TINGGI
PRIMARY NS :
1. MINIMAL LESION
2. PROLIFERATIP
2.1. PURE PROLIFERATIVE
2.2. MEMBRANEUS PROLIFERATIVE

3. SCLEROSING LESION
3.1. FOCAL SCLEROSIS
3.2. MEMBRANEUS SCLEROSIS

HASIL BIOPSI GINJAL DPERIKSA DGN:

1. LIGHT MICROSCOPE
2. ELECTRON MICROSCOPE
3. IMMUNO FLUORESCENCE MICROSCOPE
PATOGENESIS :
LESI GLOMERULUS PERMIABILITAS PROTEIN
MOLEKUL KECIL KELUAR DALAM URINE
(ALBUMINURIA) PROTEIN URIA BERAT
HYPOALBUMINEMIA ONC.PRES. ) UDEM

PROTEIN MOLEKUL KECIL YG KELUAR BERSAMA

ALBUMIN :
- IMMUNO GLOBULIN G (Ig G)
- TRANSFERRIN
MEKANISME TIMBULNYA UDEM
1. COLLOID ONCOTIC PRESSURE (SERUM ALB)
2. EXCRETION OF SODIUM DLM URIN (O.K TUB
REAB. ) RETENSI SODIUM SHIFT FLUID
TO THE EXTRAVAS CULAR

PROTEIN SERUM : - ALBUMIN

- GLOBULIN ( , , 1, 2 )

SERUM PROTEIN ELECTROPHORESIS (SPE)

PADA NS :
 2 PD NS , TERMASUK LIPO PROTEIN
SERUM ALB. Ca PLASMA (Ca TERIKAT PADA
ALBUMIN)
3. CONGENITAL NS
- AUTOSOMAL RESESSIVE
- RESISTEN PD PENGOBATAN
PENGOBATAN NS
1. ISTIRAHAT SAMPAI UDEMA
2. DIET : - ROBORANTIA
- TINGGI PROTEIN : PROTEIN LOSS
(ESBACH)
- DITAMBAH MINIMAL DAILY REQUIREMENT
3. PREDNISON REGIMEN ISKDC :
60 MG/M2/DAY : 28 HARI
REDUCTION DOSAGE
40 MG/M2/DAY (3 HARI DL 1 MINGGU : 28 HR

BILA RELAPS : PREDNISON DIULANGI LAGI

BILA STEROID RESISTEN : CYCLOPHOS
PHAMIDE DITAMBAH PREDNISON

PROGNOSIS :- UMUMNYA BAIK.

- LEBIH BAIK DARI PADA TIPE LAIN
- BILA TERJADI RENAL FAILURE >
IDIOPHATIC MPGN
 APROXIMATION OF SURFACE AREA (M2) TO
WEIGH (KG)

WEIGH RANGE APROXIMATE SURFACE AREA

1 - 5 KG M2 = (0.05 X KG) + 0.05

6 - 10 KG M2 = (0.04 X KG) + 0.10

11 - 20 KG M2 = (0.03 X KG) + 0.20

21 - 40 KG M2 = (0.02) X KG) + 0.40

CONTOH : BB 7 KG AREA (M2) =

0.04 X 7 + 0.10 = 0.38 M2
EFEK DARI HILANGNYA PROTEIN PD NS :

1. HIPOALBUMINEMIA
2. HIPERCHOLESTEROLEMIA
3. ABNORMAL FIBRINOLYSIS
4. HYPOGAMMA GLOBULINNEMIA
5. IRON RESISTENT HYPOCHROME ANEMIA
6. DESQUAMASI LUKA LAMA SEMBUH
7. PEROBAHAN VIT. D
8. PEROBAHAN METABOLISME CORTISOL
ISTILAH & PENGERTIAN HASIL PENGOBATAN

1. REMISI TOTAL : UDEMA (-) PROTEIN URIA (-)

2. REMISI PARSIAL : UDEMA (-) PROTEINURIA (+)

3. PROTEINURIA (-), PROTEIN 4 MG/M2/H

ATAU DGN PEM. KWALITATIP / SEMI-
KWALITATIP (DIPSTICK) (-) ATAU X TRACE
SELAMA 3 HARI BERTURUT SELAMA 1
MINGGU.
Background
Bright initially described acute glomerulonephritis (GN) in 1927. Acute poststreptococcal
glomerulonephritis (PSGN) is the archetype of acute GN. Acute nephritic syndrome is the most
serious and potentially devastating form of various renal syndromes. Acute GN is
characterized by the abrupt onset of hematuria and proteinuria, often accompanied by
azotemia (ie, decreased glomerular filtration rate [GFR]) and renal salt and water retention.
Characteristics of acute GN are seen in the image below.

Ultrastructure (electron microscopy): Photograph showing proliferation of

endothelial cells and mesangial cells and leukocyte infiltrate associated with
presence of large, subepithelial, electron-dense deposits (ie, "hump") (see
arrow). Photograph courtesy of Madeleine Moussa, MD, FRCPC, Department of
Pathology, London Health Sciences Centre, London, Ontario, Canada.
 ACUT GLOMERULO NEPHRITIS

DEFINISI :
GLOMERULONEPHRITIS AKUT ADALAH
PERADANGAN PRIMER GLOMERULUS DIIKUTI
GGN SISTEMIK (RINGAN BERAT)

KLINIK DAN LABORATORIUM :

1. OLIGURIA : 240 ML/M2/24 JAM
BATAS MINIMAL PENGELUARAN
HASIL METABOLISME
BILA LEBIH SEDIKIT OLIGURIA
RENAL FAILURE
2. EDEMA : SEDIKIT (JARANG SEPERTI PD NS)
PLASMA COLLOID ONCOTIC PRESSURE
TDK MENURUN
PLASMA PROTEIN LEVEL BIASA
NORMAL

3. HIPERTENSI (SERING)
DISANGKA : RENIN MENINGKAT ATAU
PERIPHERAL VASOCONSTRICTION
PENTING RETENSI AIR DAN Na
4. CIRCULATORY CONGESTION (BISA ADA) :
- PULMONARY UDEMA
- CARDIAC OVERLOAD
- HEPATOMEGALI,
- DISTENSI V. JUGULARIS,
- GALLOP RHYTHME

5. HEMATURIA : GROSS HEMATURIA, DARK RED

URIA, BROWNISH URINE, TEA COLORED
URINE, COCA COLA URINE, CUCIAN AIR
DAGING.
6. PROTEIN URIA : 30 - 100 MG/DL-1000 MG/DL
ATAU < NEPHROTIC LEVEL
- SEDIMEN URINE : RBC +++, GRANULAR CAST
RBC CAST

7. ANEMIA : BIASANYA NORMOCHROMIC

HB 9 - 11 G % OK HEMODILUSI

8. AZOTEMIA :
AKIBAT GFR - BUN
- SERUM CREATININ
- SERUM PHOSPHATE +/-
- SERUM URIC ACID + / -
- PLASMA Ca ++
- SERUM PHOSP.
9. - HELECTROLYTE & ACID BASE TERGANGGU :
- IPERKALEMIA,
- HIPONATREMIA,
- ACIDOSIS
DIPERKUAT OK HIPERKALEMIA.
RADANG PD KAPILER GLOMERULUS, ASAL
KUMAN INFEKSI DARI TENGGOROKAN, KULIT, DLL
YAITU :- KUMAN STREPTOCOCCUS HEMOLYTICUS GRP A
- SBG KECIL KUMAN TSB BERUPA ANTIGEN &
TUBUH KITA MEMBENTUK ANTI BODY
TERHDP KUMAN TSB AG + AB = AG - AB COMPLEX
BERUPA MOLEKUL, BEREDAR KE SIRKULASI
SISTEMIK, SERING TERSANGKUT / TRAPPING PD
ENDOTHELIAL GROMERULUS MENIMBULKAN
REAKSI RADANG + PROLIFERASI SEL CAPILLER
BERTAMBAH SEMPIT CAPILLER BISA PECAH
HEMATURIA DARAH MENUMPUK DI TUBULUS
(ERITROCYTE CAST (+). BILA LEKOSIT PECAH
BERCAMPUR ERITROSIT PECAH GRANULAR
CAST.

PATHOLOGIC FINDINGS :
TERGANTUNG DARI SPESIFIC DISEASE,
UMUMNYA SAMA :
- ADANYA INFILTRASI PMN LEKOSIT
- PROLIFERASI SATU ATAU LEBIH DARI SEL
GLOMERULUS : ENDOTHEL, MESANGIAL,
EPITHEL
- GLOMERULAR MEMBESAR
- MESANGIAL EDEMA ATAU MESANGIAL
MATRIX (FINEFIBRILARY TYPE)

PENGOBATAN DENGAN :
- ISTIRAHAT PADA FASE AKUT 1-2 MINGGU
DIBUTUHKAN SEMBUH DGN SPONTAN
- HARUS DIRUMAH SAKIT AWASI :
1. ACUTE RENAL INSUFFISIENCY
FLUID
ELECTROLYTE ABNORMAL
ACID BASE
2. ACUTE HYPERTENSION (SALT & WATER
RETENSION HYPERTENSIVE ENCEPHALO
PATHY

3. OLIGURIA / ANURIA CIRCULATING

CONGESTIVE PULMONARY OEDEMA

PERHATIAN :
WATER RETENTION : HIPERTENSI, PULMONARY
OEDEMA / DC
POTASSIUM RETENTION : GGN IRAMA JANTUNG
GALLOP RHYTHME
SODIUM RETENTION : HYPERTENSI
P Ca : - KEJANG,
- TETANY (CARPOPEDAL SPASM)
UREMIA TOXIN : COMA UREMICUM
BONE MARROW DEPRESION :
- AKTIVITAS FRAGMEN
DARAH TERHENTI,
- BLEEDING

THERAPY AGN TDK ADA YANG SPESIFIK

- ANTIBIOTIKA : PENICILLIN (OSPEN)

- SELEBIHNYA TERGANTUNG KONDISI, DAN HASIL
LABORATORIUM.
 ACUT RENAL FAILURE

DEFINISI :
KEADAAN KLINIS DIMANA FUNGSI GINJAL
GAGAL MEMPERTAHANKAN HOMEOSTASIS
(CAIRAN TUBUH ELEKTROLIT,HASIL AKHIR
METABOLISME PROTEIN).

- BIASANYA DISERTAI : OLIGURIA--> OLIGURIC

RENAL FAILURE
- BISA NON OLIGURI RENAL FAILURE
- 0,5 % KENAIKAN SERUM CREATININE / HARI
FUNGSI GLOMERULUS
GFR (GLOMERULAR FILTRATION RATE) BISA DI
UKUR:

A. COVNAHAN (1976)
GFR = 0,55 x TB (CM)
PLASMA CREATININ (MG)/100 ML)
= . ML/MIN/1,73 M2
GFR= 0,85 x HASIL RUMUS DIATAS
RUMUS INI TDK BERLAKU UNTUK : 0 - 1 THN
COCOK UNTUK : 1 - 14 THN
CONSTANTA BARRAT : BBLR (1 THN) : 0,33
CB (1THN) : 0,45
2 - 12 THN : 0,55
O 13 - 21 THN : 0,55
13 - 21 THN : 0,70
B. COCKCROF & GAULT (1976)
GFR : (140 - UMUR) x BB (KG)
------------------------------------ : SERING DIPAKAI
72 x SERUM CREATININE DI PENY.DALAM
CONTOH : ANAK 5 THN TB : 100 CM, BB 15 KG.
SERUM CREATININE 2 MG %

GFR = 0,55 x 100 = 27,5 ML / MENIT / 1,73 M2

2
GFR NORMAL 1 - 12 THN : 127 2 SD ; 1 SD = 19

89 - 165 ML / MIN./1.73M2

127 ML / MIN / 1.73 M2 FUNGSI GINJAL : 100 %

63 ML / MIN / 1.73 M2 FUNGSI GINJAL : 50 %
31 ML / MIN / 1.73 M2 FUNGSI GINJAL 25 %
15 ML / MIN / 1.73 M2 FUNGSI GINJAL 12.5 %
FUNGSI GINJAL 50 - 100 % : IMPAIR RENAL
FUNCTION
FUNGSI GINJAL 25 - 50 % : CHRONIC RENAL
INSUFFISIENCY
FUNGSI GINJAL 12.5 - 25 % : CHRONIC RENAL
FAILURE
PATOGENESIS ARF :
1. PRE RENAL FAILURE
BILA PERFUSI KEGINJAL (HIPOTENSI /
DEHIDRASI) FILTRASI GLOMERULUS
PRODUKSI URIN OLIGURIA ( 240
ML/M2/24 JAM) GGN KESEIMBANGAN AIR,

ELEKTROLIT DAN SISA METABOLISME

POST RENAL FAILURE
MISAL : A. DEHIDRASI
B. NEPHROTIC SYNDROME
C. CONGESTIVE HEART FAILURE
D. HYPOTENSI : 1. NEONATAL
ASPHYXIA
2. HEMORRHAGE
3. SEPTICSHOCK
2. RENAL FAILURE
- ORGANIC CAUSE OF RF
RENAL PARENCHYMAL INJURY
A. AGN
B. HUS
C. PURPURA FULMINANS
D. HYPERURICEMIA
E. ACUT TUBULAR / CORTICAL NECROSIS
F. ARTERIA / VENA THROMBOSIS
G. CONGENITAL MALFORMATION
H. MYOGLOBIN URIA / HEMOGLOBINURIA
I. NEPHROTIC DRUGS
3. POST RENAL FAILURE
- OBSTRUCTIVE CAUSE OF ARF -
OBSTRUCTIVE UROPATHIES :
A. UROLITHIASIS
B. HYDRONEPHROSIS
C. RENAL DYSPLASIA
D. KERACUNAN JENGKOL

BEDA ANTARA PRE ARF & ORGANIC ARF YANG

DIUKUR
KONSENTRASI HIGH ISOTONIC
URINE OSMOLALITY > 320 MOS < 320 MOS
URINE SODIUM < 30 MEg/L > 30 MEg/L
Na IN URINE <1 >1
K

U UREA NITROGEN > 20 < 10

P

U CREATININE > 20 < 15

P
PATOFISIOLOGI ARF EFEK

WATER RETENSION HYPONATREMIA

SODIUM RETENSION EXPANSI EXT. FL
POTASSIUM RETENSION HYPERKALEMIA
H + 10 N RETENSION ACIDOSIS
PHOSPHATE RETENSION HYPOCALCEMIA
UREA / UREMIC RETENSION UREMIA
BONE MARROW DEPRESSION ANEMIA/BLEEDING

KONSEKUENSI
CEREBAALUDEMA, KEJANG
HIPERTENSI, PUL. OEDEMA
ARRYTHMIA, CARDIAC ARREST HYPERKALEMIA
TETANY, CONVULSI BLEEDING