ICTERUS OR JAUNDICE

Atan Baas Sinuhaji

Department of ChildHealthSchool of Medicine,University Of Sumatera Utara Medan

JAUNDICE

YELLOW APPEARANCE OF THE SKIN & MUCOUS MEMBRANES

BILIRUBIN

BODY FLUIDS TISSUE

CHILDREN & ADULTS : > 2-3 mg %NEONATES : > 5 mg %

YELLOWNESS OF THE SKIN/PALMS

JAUNDICE ≠ CAROTENEMIA

BILIRUBIN

UNCONJUGATED

CONJUGATED

FREE

ALBUMIN

FAT

FREE

ALBUMIN(DELTA)

WATER

Hb

RES

TRANSPORT ALBUMIN

LIVER UPTAKE

CONJUGATION

SECRETION

GLUCORONYL TRANSFERASE

GLUCURONICACID

ADULTS

GUT

INFANTS

BILIRUBIN METABOLISM

B. Glucuro-nidase

Bilinogen

Bacteria

Urine Stools

Urobilin Stercobilin

LIGANDIN

DECONJUGATION

STOOLS

UNCONJUGATED HYPERBILIRUBINEMIA

1. INCREASED PRODUCTION

- Hemolysis

- Hematoma

- Drugs: Vit. K

G6 PD def

Infection

Antagonism

2. DEFECT OF TRANSPORT ALBUMIN

Conc. : Premature

Capacity : Acidosis

Competitive : Sulfa, Free Fatty Acid

3. DECREASED UPTAKE

LIGANDIN : GILBERTS SYNDR.(Y – Z PROTEIN = GLUTHATHIONE – S – TRANSFERASE)

4. DEFECT OF CONJUGATION

- GLUCORONYLTRANSFERASE

Conc. : Crigler Najjar Synd.

Block : Chloramphenicol

Activity : Infection, dehydration

- GLUCURONIC ACID

5. ENTEROHEPATIC CIRCULATION : - OBSTRUCTION- ANTIBIOTICS- BREAST MILK JAUNDICE

Jaundice

HUMAN MILK

Breast milkBreast milk Breast FeedingBreast Feeding

AbnormalityAbnormality IntakeIntake

CONSEQUENCES OF UNCONJUGATEDHYPERBILIRUBINEMIA

1. KERN ICTERUS = BILIRUBIN ENCEPHALOPATHY

2. CHOLESTASIS

3. UNDERLYNG - HEMOLYTIC- CHOLESTASIS

CONJUGATED HYPERBILIRUBINEMIA

CHOLESTASISNON CHOLESTASIS

- ROTOR SYNDROME- DUBIN JOHNSON SYND.

DUCTS =OBSTRUCTIVE

HEPATOCYTE

INTRAHEPATIC

EXTRAHEPATIC

Hepatocyte

canaliculi

terminal bileduct

intralobular bileduct

interlobular bileduct

septal bileduct

left hepatic

duct

right hepatic

duct

Common hepatic duct

Choledochal duct

duodenum

Pancreatic duct

Cystic duct

Intrahepatic

Extrahepatic

BILIARY TRACT

CHOLESTASIS

STAGNATION/INTERFERENCE OF BILE FLOW

CONSEQUENCES

DEFECT OF CANALICULAR – BILE SECRETION

ACCUMULATION RETENTION IN

THE BLOOD

BILE

Bile Salt bile acid cholesterol

Bilirubin Hb

electrolytes

phospholipid

protein

cholesterol

HEPATOCYTE CHOLESTASIS

1. CHOLEPOEIESIS2. SECRETION3. CANALICULAR CONTRACTION

INTERFERENCES OF:INTERFERENCES OF:

OBSTRUCTIVE CHOLESTASIS= DUCTS

1. Ducts EHBA (Extrahepatic Biliary Atresia)2. Inpissited bile3. Intrabilier pressure 4. Interferences of bile delivery

INFECTION NON INFECTION

INFLAMMATION

EMBRYOGENESIS CHOLANGIOPATHIA INFANTILE OBSTR.

BILE DUCT ABNORMALITY

1. ATRESIA EHBA

2. HYPOPLASIA

3. PAUCITY

4. CYSTS

5. FIBROSIS

OBSTRUCTION

PROXIMAL PRESS. ACCUMULATIONOF CHEMICAL

AGENT

INFECTION

SECRETION DAMAGE OF HEPATOCYTE

ISCHEMIC OF DUCTS WALL

“CHOLANGITIS”

SUPERSATURATION “HEPATITIS”

OBSTRUCTION

CONSEQUENCES OF OBSTRUCTIVE CHOLESTASIS

CHOLESTASIS

HEPATOCYTE DUCTS

“HEPATITIS” “CHOLANGITIS”

+

“HEPATITIS”

DIAGNOSIS CHOLESTASIS

- BILIRUBIN CONJ. > 2 mg %

- BILIRUBIN CONJ. > 20% TOTAL BILIRUBIN

- SERUM ASAM EMPEDU > 10 gr / L a 2 X N

USBA

(URINARY SULFATED BILE ACID) > 55 mol/gr creatinine

OROR

WITHWITH

S I N U S O I D

HEPATOCYTE

DUCT

PARACELLULAR

TRANCELLULAR

SPACE OF DISSE

Central Vein

BOWEL

entero hepatic circulation

BILE ACID CIRCULATION

Portal Vein

HEPATIC ARTERY

BILE ACID

HEPATOCYTE

ENTEROHEPATIC CIRC 95%

CHOLESTASIS

BILE

FAT MALABSORPTION

* STEATORRHOEA* PCM* DEF. VIT. A HEMERALOPIA D RICKETS E NEUROMUSC. DEG K INTRACRANIAL

BLEEDING

RETENTION

ENTEROHEP. CIRC.

CHOLESTEROL XANTHOMAS

BILE ACID BILIARY CIRRHOSIS

TRACE ELEMEN CUPRUM

CONJ. BILIRUBIN ICTERUS

INPISSITED BILE

CHOLESTASIS

NEONATES CHILDREN

- EHBA- INTRAHEPATIC CHOLESTASIS

- VIRAL HEPATITIS- MECHANICAL OBSTR. - INTRAHEPATICCHOLESTASIS

EHBA

OPERATIVE

CORRECTABLE UNCORRECTABLE

PARTIAL ATRESIA TOTAL ATRESIA

KASAI OPERATION

HEPATITIS

= INFLAMMATION OF HEPATOCYTE

ALT (ALANINE AMINOTRANSFERASE)= SGPT (SERUM GLUTAMATE PYRUVATE TRANSAMINASE)

2 x N

HEPATITIS

INFECTION

VIRAL

BACTERIA

PARASITES

HEPATOTROPIC

NON HEPATOTROPIC

NONINFECTION

•DRUGS DRUG INDUCED HEP.

•TOXIN

•METABOLIC

•INFARCT

•Ag-Ab

HEPATOTROPIC VIRAL

A HEP. INFEKSIOSA

B HEP. B

C HEP. C

D HEP. DELTA

E HEP. E

F ??

G HEP.G

STADIUM

PRODROMAL

ICTERUS= FEVER(-)

RECOVERY

VIRAL HEPATITIS

RECOVERY

PROGRESSIVE

ACUTE FULMINANT HEPATIC FAILURE

CHRONIC

CARRIER

CHRONIC HEP. (SGPT ≥ 6 MONTHS )

HEPATIC CIRRHOSIS

VIRAL HEPATITIS

SYMPTOMATIC ASYMPTOMATIC

ICTERIC ANICTERIC

‘FLU LIKE’

SUBCLINICAL

BIOCHEMISTRY

INAPPARENT INFECTION

SEROLOGY

eg. IgM ANTI HAV (+)

HEPATITIS. A

TREATMENT : 1.BED-REST2.WATER & ELECTROLYTES : PREVENTION OF DEHYDRATION 3.DIET : FAT ISN’T LIMITATED

HEPATIC CIRRHOSIS

- FIBROSIS(+)

- NODULE (+)

LIVER DYSFUNCTION

HEPATIC FAILURE= HEPATIC ENCEPHALOPATHY

PORTAL HYPERTENSION

HYPERSPLENISM

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)

PORTAL HYPERTENSION

= PORTA VENOUS PRESSURE 12 mmHg HIGHER THAN THE PRESSURE IN THE INFERIOR VENA CAVA

VARICESASCITES SPLENOMEGALY

COLLATERAL VEINS

SUP. MESENTERIC V. SPLENIC V.

PORTAL V.

RIGHT PORTAL V. LEFT PORTAL V.

HEPATIC VEIN

INFERIOR VENA CAVA

HEART

DIAGRAM OF PORTAL VENOUS

PANCREATICODUODENAL V.

CAPUT MEDUCAE

CORONARY V. ESOPH. VARICES

UMBILICAL V.

PORTAL HYPERTENSION

INTRAHEPATIC

EXTRAHEPATIC

(50-70%)

PRE HEP

THROMBOSIS V.UMBILICALIS

POST HEP.

BUDD CHIARY SYNDR.

INTRAHEPATIC

POST SINUSOIDAL

SINUSOIDAL

PRE SINUSOIDAL

TERMINAL

HEPATIC

VENULE

VENO OCCLUSIVE DISEASE

HEPATIC CIRRHOSIS

TERMINAL

PORTAL

VENULE

SCHISTOSOMIASIS

BLEEDING IN PORTAL HYPERTENSION

INTRAHEPATIC EXTRAHEPATIC FREQUENCY > >>>

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ASCITES >>> >

THE OTHER SIGNS

- JAUNDICE + -

- PALMAR ERYTHEMA + -

- VASCULAR SPIDER + -

- LIVER DYSFUNCTION + -