JAUNDICE

Dr. Soegiarto Gani, SpPD

Bagian Ilmu Penyakit Dalam/ FK. USU/ RSUP. HAM

Jaundice ( ikterus ) :

-kuning pada mukosa dan kulit.

-bilirubin > 2 mg/dl atau > 34 mol/L
-terlihat pada sklera, kulit, dan dark urine
easiest to see under natural light
easiest to see in sclera

Jaundice  dlm darah = hiperbilirubinemia.

80–85% bilirubin dari pemecahan haemoglobin
dan sekitar 15-20% dari haem erythrocyte yang
matang (mature ) dan immature cell pada lien
dan sumsum tulang.
Metabolisme Haemoglobin  bilirubin
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)
 Jaundice

• Post Hepatic (Obstructive) – Stone, tumor

– Conjugated/Direct Bil, High colored urine,
• Pre Hepatic (Acholuric) - Hemolytic
– Unconjugated/Indirect Bil, pale urine
• Hepatocellular Jaundice - Viral
– Liver damage - unconjugated
– Swelling, canalicular obstruction - Conjugated
 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
Transportase dari bilirubin
Klasifikasi
Klasifikasi jaundice menurut :
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
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
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, -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
Algoritma penanganan Jaundice Uncojungated
hyperbilirubinemia oleh karena over production
Uncungated hyperbilirubinemia akibat defect atau
inhibisi hepatic UDP-GT
Conjugated Hyperbilirubenemia Non Cholestasis
Jaundice
Conjugated hyperbilirubinemia cholestasis jaundice
Extrahepatic cholestasis
Conjugated hyperbilirubinemia akibat reversible atau
progressive intra hepatic cholestasis
 Consequences of Cholestasis
• Malabsorption of fat
• Essential fatty acid deficiency
• Fat soluble vitamin deficiency (A,D,E.K)
• Pruritis
• Secondary biliary cirrhosis*
• Cholangitis*

* especially seen with extrahepatic cholestasis