gis 20102011 slide jaundice

7/29/2019 Gis 20102011 Slide Jaundice

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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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gis 20102011 slide jaundice

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