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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, dankulit.
- bila kadar bilirubin diatas 2 mg/dl atau > 34mol/L
- terlihat pada sklera, kulit, dan dark urine
Jaundice dlm darah (hiperbilirubinemia ).
8085% bilirubin dihasilkan oleh pemecahanhaemoglobin dan sekitar 15-20% dari haemerythrocyte yang matang (mature ) danimmature 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 dan reaksi Tidak Terkonjugasiterkonjugasi
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
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Bilirubin uptake
PLASMA
SINUSOIDALMEMBRANE
CYTOSOL
ENDOPLASMIC RETICULUM
BR albumin
BR + albumin
Carrier proteins Flip / flop
BR
Protein bound( ligandin )
Membrane-membranetransfer
Conjugation ( UGT1)
Mono and diglucuronides
Transporters MOAT
Bile
CANALICULAR MEMBRANE
Transpotase dari bilirubin
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Klasifikasi
Klasifikasi jaundice.
1. Unconjugated hyperbilirubinemia2. Conjugated hyperbilirubinemia
1. Unconjugated hyperbilirubinemia
Overproduction from breakdown of haemoglobin andother heme proteins
Haemolysis (intra-and extravascular)Ineffective erythropoiesisEnhanced turnover of hepatic heme enzymes
Impairment of hepatic uptakeDecreased delivery to the liverDrugsGilbert syndrome,type I
Impairment of hepatic conjugationNeonatalMaternal milkLucey-Driscoll syndromeCrigler Najjar syndrome, type ICrigler Najjar syndrome, type II (arias syndrome)Gilbert syndrome, type IIDrugs
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2.Conjugated hyperbilirubinemia
Defect of hepatic exretion
Extrahepatic bile duct obstructionExtrahepatic bile duct atresiaHepatic jaundiceIntrahepatic reversible cholestatis
familial recurrent cholestatis ( Summerskill -Walshe syndrome)
Recurrent cholestatis of pregnancyIntrahepatic progressive cholestatis
Infancy progressive cholestatis (Byler syndrome)Arteriohepatic dysplasia (Alagille syndrome)Primary biliary cirrhosis
Primary sclerosing cholangitis (PSC)Inherited defective excretio of conjugated organicanions and coproporphyrin
Dubin johnson syndromeRotor syndrome
Beberapa penyebab jaundice yg sering dgn investigasinya
Penyebab InvestigasiAcute 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 / BiliaryDisease
- Ultrasonography
- 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
CardiorespiratoryFailure
- Chest X Ray- ECG
Gilberts Syndrome - Increase in unconjugated bilirubin following
2-3 days on a 400 calorie diet
Unconjungated Hyperbilirubinemia Akibat
Defektive Hepatic UptakeJaundice
Unconjugated hyperbilirubinemia
Congestiveheart failure
Portal-Systemicshunt
Drugs :Rifamycin
BunamiodylProbenecid
flavaspidic acid
Serum bilirubin after fasting(400 cal/3 days )
serum bilirubin afterphenobarbital Bil. kinetics :
R4h>10% CBR
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gor ma penanganan aun ce nco unga ehyperbilirubinemia karena over production
Uncungated hyperbilirubinemia akibat defect atauinhibisi hepatic UDP-GT
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on ugate yper ru enem a on o estas sJaundice
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Conjugated hyperbilirubenemia cholestasis jaundice
Conjugate yper i iru inemia a i at reversi e atauprogressive intra hepatic cholestasis
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