JAUNDICE

Dr.LEONARDO B DAIRY, SpPD-KGEH

Divisi Gastroentero-Hepatologi
Bagian Ilmu Penyakit Dalam/ FK. USU/ RSUP. HAM

Jaundice ( ikterus ) :

- warna kuning pada mukosa, selaput lendir, dan

kulit.
- bila kadar bilirubin diatas 2 mg/dl atau > 34
µmol/L
- terlihat pada sklera, kulit, dan dark urine
Jaundice
dlm darah (hiperbilirubinemia ).
80–85% bilirubin dihasilkan oleh pemecahan
haemoglobin dan sekitar 15-20% dari haem
erythrocyte yang matang (mature ) dan
immature cell pada lien dan sumsum tulang.

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Metabolisme Haemoglobin
bilirubin

Metabolisme Bilirubin

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Bilirubin Formation Plasma Hepatocyte Bile

SIFAT-SIFAT KOMPARATIF ANTARA BILIRUBIN

TERKONJUGASI DAN TIDAK TERKONJUGASI

Sifat dan reaksi Tidak Terkonjugasi

terkonjugasi

Kelarutan dalam air 0 +

Afinitas thd lemak + 0
Ekskresi renal 0 +
Reaksi van den Bergh Indirek (total) Direk
(minus direk)
Ikatan dgn albumin serum +++ +
(reversibel)
Pembentukan kompleks 0 +†
bilirubin-albumin
(ireversibel)

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 Bilirubin uptake
BR – albumin
PLASMA
BR + albumin

Carrier proteins Flip / flop SINUSOIDAL

MEMBRANE
BR

Protein bound Membrane-membrane

( ligandin ) transfer
CYTOSOL
Conjugation ( UGT1) ENDOPLASMIC RETICULUM

Mono and diglucuronides

Transporters MOAT CANALICULAR MEMBRANE

Bile

Transpotase dari bilirubin

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 Klasifikasi

Klasifikasi jaundice.
1. Unconjugated hyperbilirubinemia
2. Conjugated hyperbilirubinemia
1. Unconjugated hyperbilirubinemia
Overproduction from breakdown of haemoglobin and
other heme proteins
Haemolysis (intra-and extravascular)
Ineffective erythropoiesis
Enhanced turnover of hepatic heme enzymes
Impairment of hepatic uptake
Decreased delivery to the liver
Drugs
Gilbert syndrome,type I
Impairment of hepatic conjugation
Neonatal
Maternal milk
Lucey-Driscoll syndrome
Crigler – Najjar syndrome, type I
Crigler – Najjar syndrome, type II (arias syndrome)
Gilbert syndrome, type II
Drugs

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2.Conjugated hyperbilirubinemia
Defect of hepatic exretion
Extrahepatic bile duct obstruction
Extrahepatic bile duct atresia
Hepatic jaundice
Intrahepatic reversible cholestatis
familial recurrent cholestatis ( Summerskill -
Walshe syndrome)
Recurrent cholestatis of pregnancy
Intrahepatic progressive cholestatis
Infancy progressive cholestatis (Byler syndrome)
Arteriohepatic dysplasia (Alagille syndrome)
Primary biliary cirrhosis
Primary sclerosing cholangitis (PSC)
Inherited defective excretio of conjugated organic
anions and coproporphyrin
Dubin – johnson syndrome
Rotor syndrome

Beberapa penyebab jaundice yg sering dgn investigasinya

Penyebab Investigasi
Acute hepatitis - HBsAg, anti – HBc IgM, HBeAg, anti-Hbe
- Anti-HAV IGM, Anti delta antibody
- Anti HCV
- Anti HEV ?
- IgM-EBV, IGM-CMV, Leptospiral antibody
Pancreatic / Biliary - Ultrasonography
Disease - Endoscopic retrograde cholangiography- c
Pancreatography
- Percutaneuos transhepatic cholangiography
- CT scan
Malignancy - Ultrasonography
- CT scanning
- Liver biopsy
- Alpha foetoprotein

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Cirrhosis - Hepatitis B/C serology
- Ultrasonography
- Liver biopsy
- Immunoglobulins
- Auto antibodies
- Iron studies
- serum, urine and liver copper;serum
ceruloplasmin
- alpha 1 antitrypsin
Haemolysis - Reticulocyte count
- Haptoglobin
- Direct and indirect Coombs’ Test
- G-6-P-D level
Cardiorespiratory - Chest X Ray
Failure - ECG
Gilbert’s Syndrome - Increase in unconjugated bilirubin following
2-3 days on a 400 calorie diet

Unconjungated Hyperbilirubinemia Akibat

Defektive Hepatic Uptake
Jaundice

Unconjugated hyperbilirubinemia

Normal AST/ALT, AP, γ-GT, BA

Congestive Drugs : ↑ Serum bilirubin after fasting

heart failure Rifamycin (400 cal/3 days )
Portal-Systemic Bunamiodyl ↓ serum bilirubin after
shunt Probenecid phenobarbital Bil. kinetics :
flavaspidic acid R4h>10% CBR<0.8 ml/min/kg
↓ K21 (uptake)

Decreased Competition for GILBERT

delivery to hepatic uptake syndrome
the liver

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 Algoritma penanganan Jaundice Uncojungated
hyperbilirubinemia karena over production

Uncungated hyperbilirubinemia akibat defect atau

inhibisi hepatic UDP-GT

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Conjugated Hyperbilirubenemia Non Cholestasis
Jaundice

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Conjugated hyperbilirubenemia cholestasis jaundice

Conjugated hyperbilirubinemia akibat reversible atau

progressive intra hepatic cholestasis

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