CHOLESTASIS

Cholestasis
Gangguan aliran empedu
Sel hati duodenum
Kelainan intra-ekstra hepatic
Fisiologis
Klinis
Biokimia
Histopatologis
Neonatal Cholestasis
Peningkatan kadar direk bilirubin
Setelah usia 14 hari
Infeksi
Genetik
Metabolik
Mekanik & Fungsional
SELALU PATOLOGIS
Neonatal Cholestasis
Intra-hepatic
Ekstra-hepatic
Hepatocyte
Bile duct
Metabolic viral Idiopatic
neonatal
hepatitis
Intra hepatic bile duct
hypoplasia or paucity
Bile duct injury or
obstruction
- EHBA
-Choledocal cyst
Evaluasi
1. Pemeriksaan fungsi hati
2. Warna tinja urine
3. Pengukuran bile acid
4. Serologis marker
5. Screening metabolik
6. USG, scintigrafi, biopsi hati
Intra-hepatic Cholestasis
Infeksi : TORCH
Intra-hepatic bile duct paucity
- Alagile syndrome
- Byler disease
Intra-hepatic Cholestasis
- Gangguan metabolisme CBH
Galactosa
Fructosa
Glycogen I, II, III, IV
CBH glycosilation
Intra-hepatic Cholestasis
- Gangguan metabolisme asam amino
Tyrosine
Urea cycle defect
* CPS, OTC, DEF
* Cirulinemi
* Argininemi
* N-AGS defisiensi
- Gangguan Metabolisme lemak
Wolman, Gauchers, Niemann Pick
Intra-hepatic Cholestasis
- Gangguan metabolisme metal
Wilson
NISD
Cooper overload
- Gangguan metabolisme asam empedu
- Gangguan metabolisme bilirubin
Criger Najar
Gilbert
Dublin Johnson Rotor Syndrome
- Lain
1 anti trypsin DEF
cystic fibrosis
Ekstra-hepatic
Biliary atresia : progressive obliterative
cholangiopathy
Sukar DD dg Neonatal Hepatitis
Disertai kelainan lain
BBL cukup
Acholic stools
PA : - Bile plugs, ductular proliferation
periportal fibrosis
- Lobular architecture intact