Atan Baas Sinuhaji Sub Division of Pediatrics Gastroentero-Hepatology Department of Childhealth,School of Medicine University of Sumatera Utara /

Transcription

1 Atan Baas Sinuhaji Sub Division of Pediatrics Gastroentero-Hepatology Department of Childhealth,School of Medicine University of Sumatera Utara / Adam Malik Hospital Medan

2 JAUNDICE YELLOW APPEARANCE OF THE SKIN & MUCOUS MEMBRANES BILIRUBIN BODY FLUIDS TISSUE CHILDREN & ADULTS : > 2-3 mg % NEONATES : > 5 mg % JAUNDICE CAROTENEMIA YELLOWNESS OF THE SKIN/PALMS

3 FREE UNCONJUGATED FAT ALBUMIN BILIRUBIN FREE CONJUGATED WATER ALBUMIN (DELTA)

4 -BILIRUBIN HALF LIFE ± 22 DAYS PROLONGING JAUNDICE BIOCHEMICAL LIVER TEST ARE IMPROVING

5 Hb RES TRANSPORT ALBUMIN LIVER UPTAKE LIGANDIN GLUCORONYL TRANSFERASE CONJUGATION GLUCURONIC ACID SECRETION GUT INFANTS B. Glucuronidase ADULTS Bacteria DECONJUGATION Bilinogen STOOLS Urine Stools BILIRUBIN METABOLISM Urobilin Stercobilin

6 UNCONJUGATED HYPERBILIRUBINEMIA 1. INCREASED PRODUCTION - Hemolysis G 6 PD def Infection Antagonism - Hematoma 2. DEFECT OF TRANSPORT - Drugs: Vit. K ALBUMIN Conc. : Premature Capacity : Acidosis Competitive : Sulfa, Free Fatty Acid 3. DECREASED UPTAKE LIGANDIN : GILBERTS SYNDR. (Y Z PROTEIN = GLUTHATHIONE S TRANSFERASE)

7 Conc. : Crigler Najjar Synd. - GLUCORONYL TRANSFERASE Block : Chloramphenicol 4. DEFECT OF CONJUGATION Activity : Infection, dehydration - GLUCURONIC ACID 5. ENTEROHEPATIC CIRCULATION : - OBSTRUCTION - ANTIBIOTICS - BREAST MILK JAUNDICE

8 HUMAN MILK Jaundice Breast milk Breast Feeding Abnormality Intake

9 CONSEQUENCES OF UNCONJUGATED HYPERBILIRUBINEMIA 1. KERN ICTERUS = BILIRUBIN ENCEPHALOPATHY 2. CHOLESTASIS 3. UNDERLYNG - HEMOLYTIC - CHOLESTASIS

10 CONJUGATED HYPERBILIRUBINEMIA NON CHOLESTASIS CHOLESTASIS HEPATOCYTE - ROTOR SYNDROME - DUBIN JOHNSON SYND. DUCTS = OBSTRUCTIVE INTRA HEPATIC EXTRA HEPATIC

11 Hepatocyte canaliculi terminal bileduct Intrahepatic intralobular bileduct interlobular bileduct septal bileduct left hepatic duct right hepatic duct Extrahepatic Common hepatic duct Cystic duct Choledochal duct Pancreatic duct BILIARY TRACT duodenum

12 CHOLESTASIS STAGNATION/INTERFERENCE OF BILE FLOW CONSEQUENCES DEFECT OF CANALICULAR BILE SECRETION ACCUMULATION RETENTION IN THE BLOOD

13 Bile Salt bile acid cholesterol Bilirubin Hb electrolytes BILE phospholipid protein cholesterol

14 HEPATOCYTE CHOLESTASIS INTERFERENCES OF: 1. CHOLEPOEIESIS 2. SECRETION 3. CANALICULAR CONTRACTION

15 OBSTRUCTIVE CHOLESTASIS = DUCTS 1. Ducts EHBA (Extrahepatic Biliary Atresia) 2. Inpissated bile 3. Intrabilier pressure 4. Interferences of bile delivery

16 INFECTION NON INFECTION INFLAMMATION EMBRYOGENESIS CHOLANGIOPATHIA INFANTILE OBSTR. BILE DUCT ABNORMALITY 1. ATRESIA EHBA 2. HYPOPLASIA 3. PAUCITY 4. CYSTS 5. FIBROSIS

17 CYST A tissue enclosed space that is abnormal in location or size CONTAIN STRUCTURE Air Liquid or solids (partially or wholly)

18 STRUCTURE NORMAL ABNORMAL DILATED WITH A DISCRETE WALL BILIARY TRACT

19 RATIO BILE DUCT / PORTAL TRIADE < 0,9 BILE DUCT PAUCITY SYNDROMIC NON SYNDROMIC ALAGILLE SYNDROME PRIMARY -α-1 antitrysin deficiency -Byler disease -etc SECONDARY -Graft vs host disease -etc

20 OBSTRUCTION PROXIMAL PRESS. ACCUMULATION OF CHEMICAL AGENT INFECTION SECRETION DAMAGE OF HEPATOCYTE ISCHEMIC OF DUCTS WALL CHOLANGITIS SUPERSATURATION HEPATITIS OBSTRUCTION CONSEQUENCES OF OBSTRUCTIVE CHOLESTASIS

21 CHOLESTASIS HEPATOCYTE DUCTS HEPATITIS CHOLANGITIS + HEPATITIS

22 DIAGNOSIS CHOLESTASIS - BILIRUBIN CONJ. > 2 mg % OR - BILIRUBIN CONJ. > 20% TOTAL BILIRUBIN WITH - SERUM ASAM EMPEDU > 10 µgr / L a 2 X N USBA (URINARY SULFATED BILE ACID) > 55 µmol/gr creatinine

23 HEPATIC ARTERY S I N U S O I D Central Vein Portal Vein PARACELLULAR SPACE OF DISSE entero hepatic circulation HEPATOCYTE BILE ACID CIRCULATION DUCT BOWEL

24 HEPATOCYTE BILE ACID ENTEROHEPATIC CIRC ± 95%

25 BILE ENTEROHEP. CIRC. CHOLESTASIS INPISSITED BILE FAT MALABSORPTION * STEATORRHOEA * PCM * DEF. VIT. A HEMERALOPIA D RICKETS E NEUROMUSC. DEG K INTRACRANIAL BLEEDING RETENTION CHOLESTEROL XANTHOMAS BILE ACID BILIARY CIRRHOSIS TRACE ELEMEN CUPRUM CONJ. BILIRUBIN ICTERUS

26 CHOLESTASIS NEONATES CHILDREN - EHBA - INTRAHEPATIC CHOLESTASIS - VIRAL HEPATITIS - MECHANICAL OBSTR. - INTRAHEPATIC CHOLESTASIS

27 INTRAHEPATIC CHOLESTASIS 1. IDIOPATHIC NEONATAL HEPATITIS ( IDIOPATHIC NEONATAL GIANT CELL HEPATITIS ) 2. INFECTION HEPATITIS IN A NEONATE 3. INTRAHEPATIC BILE DUCT PAUCITY

28 EHBA OPERATIVE CORRECTABLE UNCORRECTABLE PARTIAL ATRESIA TOTAL ATRESIA KASAI OPERATION

29 KASAI OPERATION To bypass the obstructed extrahepatic bile ducts and to restore the biliary flow Fails to clear jaundice and/or complications associated with biliary cirrhosis appear LIVER TRANSPLANTATION

30 KASAI OPERATION INCREASED AGE AT SURGERY HAD A PROGRESSIVE AND SUSTAINED DELETERIOUS EFFECT Serinet MO.Pediatrics 2009;123 :1280-6

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32 KASAI I

33 KASAI II (double Roux-en-Y)

34 SAWAGUCHI

35 SURUGA I

36 Sonde SURUGA II

37 KASAI I with an INTESTINAL VALVE

38 HEPATITIS = INFLAMMATION OF HEPATOCYTE ALT (ALANINE AMINOTRANSFERASE) ALT (ALANINE AMINOTRANSFERASE) = SGPT (SERUM GLUTAMATE PYRUVATE TRANSAMINASE) 2 x N

39 HEPATOTROPIC VIRAL NON HEPATOTROPIC INFECTION BACTERIA PARASITES HEPATITIS DRUGS DRUG INDUCED HEPATITIS NONINFECTION TOXIN METABOLIC INFARCT Ag-Ab

40 HEPATOTROPIC VIRAL A HEP. INFEKSIOSA B HEP. B C HEP. C D HEP. DELTA E HEP. E F?? G HEP.G

41 PRODROMAL STADIUM ICTERUS= FEVER(-) RECOVERY

42 RECOVERY ACUTE FULMINANT HEPATIC FAILURE VIRAL HEPATITIS PROGRESSIVE CHRONIC CHRONIC HEP. (SGPT >= 6 MONTHS ) HEPATIC CIRRHOSIS CARRIER

43 VIRAL HEPATITIS SYMPTOMATIC ASYMPTOMATIC ICTERIC ANICTERIC SUBCLINICAL INAPPARENT INFECTION FLU LIKE BIOCHEMISTRY SEROLOGY eg. IgM ANTI HAV (+) HEPATITIS. A

44 TREATMENT : 1.BED-REST 2.WATER & ELECTROLYTES : PREVENTION OF DEHYDRATION 3.DIET : FAT IS NOT LIMITATED

45 HEPATIC CIRRHOSIS - FIBROSIS(+) - NODULE (+) LIVER DYSFUNCTION PORTAL HYPERTENSION HYPERSPLENISM HEPATIC FAILURE = HEPATIC ENCEPHALOPATHY

46 HYPERSPLENISME INCREASED SPLENIC FUNTION SEQUESTRATION OR DESTRUCTION OF CIRCULATING CELL 1. PERIPHERAL BLOOD CYTOPENIA 2. INCREASED BONE MARROW ACTIVITY 3. SPLENOMEGALY

47 HEPATIC CIRRHOSIS NODULAR MICRO MACRO MIXED < 3 mm > 3 mm 5 cm 47

48 HEPATIS CIRRHOSIS BIOCHEMICAL CLINICAL Active Inactive Compensated Decompensated 48

49 CAUSES OF BLEEDING IN CIRRHOSIS 1. VIT. K DEFICIENCY 2. DEFECT OF SYNTHESIS CLOTTING FACTORS 3. RUPTURE OF ESOPHAGEAL VARICES 4. GASTROPATHY 5. ABNORMAL TROMBOCYTES 6. COAGULATION INHIBITOR 7. DIC (DISSEMINATED INTRAVASCULAR COAGULATION)

50 MAJOR SEQUELAE OF CIRRHOSIS mnemonic HEPATIC 1.Hepatic encephalopathy 2.Esophageal varices 3.Portal hyperension 4.Ascites 5.Thrombosis of portal vein 6.Infection ( spontaneous bacterial peritonitis ) 7.Carcinoma ( hepatocellular )

51 PORTAL HYPERTENSION = PORTA VENOUS PRESSURE 12 mmhg HIGHER THAN THE PRESSURE IN THE INFERIOR VENA CAVA VARICES ASCITES SPLENOMEGALY COLLATERAL VEINS

52 SUP. MESENTERIC V. SPLENIC V. CAPUT MEDUCAE PORTAL V. UMBILICAL V. PANCREATICO DUODENAL V. CORONARY V. ESOPH. VARICES RIGHT PORTAL V. LEFT PORTAL V. HEPATIC VEIN INFERIOR VENA CAVA HEART DIAGRAM OF PORTAL VENOUS

53 INTRAHEPATIC PORTAL HYPERTENSION EXTRAHEPATIC (50-70%) PRE HEP THROMBOSIS V.UMBILICALIS POST HEP. BUDD CHIARY SYNDR.

54 POST SINUSOIDAL TERMINAL HEPATIC VENULE VENO OCCLUSIVE DISEASE SINUSOIDAL HEPATIC CIRRHOSIS INTRAHEPATIC PRE SINUSOIDAL TERMINAL PORTAL VENULE SCHISTOSO MIASIS

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57 BLEEDING IN PORTAL HYPERTENSION INTRAHEPATIC EXTRAHEPATIC FREQUENCY > >>> SEVERITY >>> > LIFE EXPECTANCE > >>> ASCITES >>> > THE OTHER SIGNS - JAUNDICE PALMAR ERYTHEMA VASCULAR SPIDER+ - - LIVER DYSFUNCTION + -