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ICTERUS

OR
JAUNDICE
Atan Baas Sinuhaji

Department of ChildHealth
School 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 %

JAUNDICE ≠ CAROTENEMIA

YELLOWNESS OF THE SKIN/PALMS


FREE

UNCONJUGATED FAT

ALBUMIN

BILIRUBIN

FREE

CONJUGATED WATER

ALBUMIN
(DELTA)
Hb

RES

TRANSPORT ALBUMIN

LIVER UPTAKE LIGANDIN

GLUCORONYL
TRANSFERASE

CONJUGATION GLUCURONIC
ACID

SECRETION

GUT

INFANTS ADULTS
B. Glucuro-
Bacteria
nidase

DECONJUGATION Bilinogen

STOOLS Urine Stools

Urobilin Stercobilin
BILIRUBIN METABOLISM
UNCONJUGATED HYPERBILIRUBINEMIA

1. INCREASED PRODUCTION G6 PD def


Infection
- Hemolysis
Antagonism

- Hematoma

- Drugs: Vit. K
Conc. : Premature

2. DEFECT OF
TRANSPORT ALBUMIN Capacity : Acidosis

Competitive : Sulfa,
Free Fatty Acid

3. DECREASED LIGANDIN : GILBERTS SYNDR.


UPTAKE (Y – Z PROTEIN
= GLUTHATHIONE – S – TRANSFERASE)
Conc. : Crigler Najjar Synd.

- GLUCORONYL
Block : Chloramphenicol
TRANSFERASE

4. DEFECT OF
CONJUGATION Activity : Infection, dehydration

- GLUCURONIC ACID

5. ENTEROHEPATIC CIRCULATION : - OBSTRUCTION


- ANTIBIOTICS
- BREAST MILK JAUNDICE
HUMAN MILK

Jaundice
Breast milk Breast Feeding

Abnormality Intake
CONSEQUENCES OF UNCONJUGATED
HYPERBILIRUBINEMIA

1. KERN ICTERUS = BILIRUBIN ENCEPHALOPATHY

2. CHOLESTASIS

3. UNDERLYNG - HEMOLYTIC
- CHOLESTASIS
CONJUGATED HYPERBILIRUBINEMIA

CHOLESTASIS
NON CHOLESTASIS

HEPATOCYTE

- ROTOR SYNDROME DUCTS =


- DUBIN JOHNSON SYND. OBSTRUCTIVE

INTRA EXTRA
HEPATIC HEPATIC
Hepatocyte

canaliculi

terminal bileduct

Intrahepatic intralobular bileduct

interlobular bileduct

septal bileduct

left right
hepatic hepatic
duct duct

Common hepatic duct


Extrahepatic
Cystic duct

Choledochal duct

Pancreatic duct

duodenum
BILIARY TRACT
CHOLESTASIS

STAGNATION/INTERFERENCE OF BILE FLOW

CONSEQUENCES

DEFECT OF ACCUMULATION RETENTION


CANALICULAR – IN
BILE SECRETION THE BLOOD
Bile Salt  bile acid  cholesterol

Bilirubin  Hb

electrolytes

BILE
phospholipid

protein

cholesterol
HEPATOCYTE CHOLESTASIS

INTERFERENCES OF:

1. CHOLEPOEIESIS
2. SECRETION
3. CANALICULAR CONTRACTION
OBSTRUCTIVE CHOLESTASIS
= DUCTS

1. Ducts  EHBA (Extrahepatic Biliary Atresia)


2. Inpissited bile
3. 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. ACCUMULATION INFECTION


OF CHEMICAL
AGENT

SECRETION DAMAGE OF ISCHEMIC OF


“CHOLANGITIS”
HEPATOCYTE DUCTS WALL

SUPERSATURATION “HEPATITIS”

OBSTRUCTION

CONSEQUENCES OF OBSTRUCTIVE
CHOLESTASIS
CHOLESTASIS

HEPATOCYTE DUCTS

“HEPATITIS” “CHOLANGITIS”
+
“HEPATITIS”
DIAGNOSIS CHOLESTASIS

- BILIRUBIN CONJ. > 2 mg %

OR

- BILIRUBIN CONJ. > 20% TOTAL BILIRUBIN

WITH

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

USBA
(URINARY SULFATED BILE ACID) > 55 mol/gr
creatinine
HEPATIC ARTERY

SINUSOID Central Vein


PARACELLULAR

Portal Vein SPACE OF DISSE

entero hepatic HEPATOCYTE


circulation

DUCT

BILE ACID BOWEL


CIRCULATION
HEPATOCYTE

BILE ACID
ENTEROHEPATIC CIRC  95%
BILE  ENTEROHEP. CIRC. 

INPISSITED BILE
CHOLESTASIS

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
CHOLESTASIS

NEONATES CHILDREN

- EHBA - VIRAL HEPATITIS


- INTRAHEPATIC - MECHANICAL
CHOLESTASIS OBSTR.
- INTRAHEPATIC
CHOLESTASIS
EHBA

OPERATIVE

CORRECTABLE UNCORRECTABLE

PARTIAL ATRESIA TOTAL ATRESIA

KASAI OPERATION
HEPATITIS
= INFLAMMATION OF HEPATOCYTE

ALT (ALANINE AMINOTRANSFERASE)


= SGPT (SERUM GLUTAMATE PYRUVATE TRANSAMINASE)
2xN
HEPATOTROPIC
VIRAL NON
HEPATOTROPIC
BACTERIA
INFECTION
PARASITES

HEPATITIS

•DRUGS DRUG INDUCED HEP.


•TOXIN
NONINFECTION
•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
PRODROMAL

STADIUM ICTERUS= FEVER(-)

RECOVERY
RECOVERY

ACUTE FULMINANT HEPATIC


FAILURE

VIRAL CHRONIC HEP.


PROGRESSIVE (SGPT  ≥ 6 MONTHS )
HEPATITIS
CHRONIC

HEPATIC
CIRRHOSIS

CARRIER
VIRAL HEPATITIS

SYMPTOMATIC ASYMPTOMATIC

INAPPARENT
ICTERIC ANICTERIC SUBCLINICAL INFECTION

‘FLU LIKE’ BIOCHEMISTRY SEROLOGY

eg. IgM ANTI HAV (+)

HEPATITIS. A
TREATMENT :
1.BED-REST
2.WATER & ELECTROLYTES :
PREVENTION OF
DEHYDRATION
3.DIET : FAT ISN’T LIMITATED
HEPATIC CIRRHOSIS

- FIBROSIS(+)
- NODULE (+)

LIVER
PORTAL HYPERSPLENISM
DYSFUNCTION
HYPERTENSION

HEPATIC FAILURE
= HEPATIC ENCEPHALOPATHY
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

COLLATERAL VEINS
VARICES
ASCITES SPLENOMEGALY
SUP. MESENTERIC V. SPLENIC V.
PORTAL V.
CAPUT MEDUCAE

UMBILICAL V.

PANCREATICO CORONARY V. ESOPH. VARICES


DUODENAL V.

RIGHT PORTAL V. LEFT PORTAL V.

HEPATIC VEIN

INFERIOR VENA CAVA DIAGRAM


OF PORTAL
HEART VENOUS
INTRAHEPATIC

PORTAL PRE HEP

HYPERTENSION
THROMBOSIS
V.UMBILICALIS

EXTRAHEPATIC

(50-70%) POST HEP.

BUDD CHIARY
SYNDR.
POST TERMINAL VENO
SINUSOIDAL OCCLUSIVE
HEPATIC
DISEASE
VENULE

HEPATIC
SINUSOIDAL CIRRHOSIS

INTRAHEPATIC

TERMINAL
PORTAL SCHISTOSO
MIASIS
PRE SINUSOIDAL
VENULE
BLEEDING IN PORTAL
HYPERTENSION

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

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