05 Jaundice

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gastroenterohepatologi

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  • 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/Lterlihat pada sklera, kulit, dan dark urine

    easiest to see under natural light

    easiest to see in sclera

    Jaundice dlm darah = hiperbilirubinemia. 8085% 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 reaksiTidak Terkonjugasiterkonjugasi

    Kelarutan dalam air 0+

    Afinitas thd lemak+0

    Ekskresi renal0+

    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, tumorConjugated/Direct Bil, High colored urine, Pre Hepatic (Acholuric) - HemolyticUnconjugated/Indirect Bil, pale urineHepatocellular Jaundice - Viral Liver damage - unconjugatedSwelling, 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 proteinsHaemolysis (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

  • Conjugated hyperbilirubinemia

    Defect of hepatic exretionExtrahepatic bile duct obstructionExtrahepatic bile duct atresiaHepatic jaundiceIntrahepatic reversible cholestatisfamilial recurrent cholestatis ( Summerskill -Walshe syndrome)Recurrent cholestatis of pregnancyIntrahepatic progressive cholestatisInfancy progressive cholestatis (Byler syndrome)Arteriohepatic dysplasia (Alagille syndrome)Primary biliary cirrhosisPrimary sclerosing cholangitis (PSC)Inherited defective excretio of conjugated organic anions and coproporphyrinDubin johnson syndromeRotor syndrome

  • Beberapa penyebab jaundice yg sering dgn investigasinya

    PenyebabInvestigasiAcute hepatitis HBsAg, anti HBc IgM, HBeAg, anti-Hbe Anti-HAV IGM, Anti delta antibody Anti HCV Anti HEV ? IgM-EBV, IGM-CMV, Leptospiral antibodyPancreatic / Biliary Disease Ultrasonography Endoscopic retrograde cholangiography- c Pancreatography Percutaneuos transhepatic cholangiography CT scanMalignancy 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 antitrypsinHaemolysis Reticulocyte count Haptoglobin Direct and indirect Coombs Test G-6-P-D levelCardiorespiratory Failure Chest X Ray ECGGilberts 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

  • Algoritma penanganan Jaundice Uncojungated hyperbilirubinemia oleh karena over production

  • Uncungated hyperbilirubinemia akibat defect atau inhibisi hepatic UDP-GT

  • Conjugated Hyperbilirubenemia Non Cholestasis Jaundice

    52.unknown
  • Conjugated hyperbilirubinemia cholestasis jaundice

  • Extrahepatic cholestasis

  • Conjugated hyperbilirubinemia akibat reversible atau progressive intra hepatic cholestasis

  • Consequences of Cholestasis

    Malabsorption of fatEssential fatty acid deficiencyFat soluble vitamin deficiency (A,D,E.K)PruritisSecondary biliary cirrhosis*Cholangitis*

    * especially seen with extrahepatic cholestasis