05 jaundice
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JAUNDICE
Dr. ERWIN SYAH
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 urineeasiest 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 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)
SIFAT-SIFAT KOMPARATIF ANTARA BILIRUBIN TERKONJUGASI DAN TIDAK
TERKONJUGASI-
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
PLASMA
SINUSOIDAL MEMBRANE
CYTOSOL
ENDOPLASMIC RETICULUM
BR – albumin
BR + albumin
Carrier proteins Flip / flop
BR
Protein bound ( ligandin )
Membrane-membrane transfer
Conjugation ( UGT1)
Mono and diglucuronides
Transporters MOAT
Bile
CANALICULAR MEMBRANE
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 erythropoiesisEnhanced turnover of hepatic heme enzymes Impairment of hepatic uptake
Decreased delivery to the liverDrugs
Gilbert syndrome,type I Impairment of hepatic conjugationNeonatal
Maternal milkLucey-Driscoll syndrome
Crigler – Najjar syndrome, type I Crigler – Najjar syndrome, type II (arias syndrome)Gilbert syndrome, type II
Drugs
Conjugated hyperbilirubinemia
Defect of hepatic exretionExtrahepatic bile duct obstruction
Extrahepatic bile duct atresiaHepatic jaundiceIntrahepatic 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 cirrhosisPrimary sclerosing cholangitis (PSC)Inherited defective excretio of conjugated organic
anions and coproporphyrinDubin – 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 Disease
- Ultrasonography- 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 Failure
- Chest X Ray- 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
Congestive heart failure Portal-Systemic
shunt
Drugs : Rifamycin
Bunamiodyl Probenecid flavaspidic
acid
Serum bilirubin after fasting (400 cal/3 days )
serum bilirubin after
phenobarbital Bil. kinetics : R4h>10% CBR<0.8
ml/min/kg K21 (uptake)
Normal AST/ALT, AP, -GT, BA
Decreased delivery to the liver
Competition for hepatic uptake
GILBERT syndrome
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
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