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BILIRUBIN
BODY FLUIDS
TISSUE
FREE
FAT
UNCONJUGATED
ALBUMIN
BILIRUBIN
FREE
CONJUGATED
WATER
ALBUMIN
(DELTA)
Hb
RES
TRANSPORT
LIVER
ALBUMIN
UPTAKE
LIGANDIN
GLUCORONYL
TRANSFERASE
CONJUGATION
GLUCURONIC
ACID
SECRETION
GUT
ADULTS
INFANTS
B. Glucuronidase
DECONJUGATION
STOOLS
BILIRUBIN METABOLISM
Bacteria
Bilinogen
Urine
Stools
Urobilin
Stercobilin
UNCONJUGATED HYPERBILIRUBINEMIA
1. INCREASED PRODUCTION
G6 PD def
Infection
- Hemolysis
Antagonism
- Hematoma
- Drugs: Vit. K
2. DEFECT OF
TRANSPORT
ALBUMIN
Conc.
Capacity
: Premature
: Acidosis
Competitive : Sulfa,
Free Fatty Acid
3. DECREASED
UPTAKE
LIGANDIN
: GILBERTS SYNDR.
(Y Z PROTEIN
= GLUTHATHIONE S TRANSFERASE)
Conc.
- GLUCORONYL
TRANSFERASE
4. DEFECT OF
CONJUGATION
Block : Chloramphenicol
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
- DUBIN JOHNSON SYND.
DUCTS =
OBSTRUCTIVE
INTRA
HEPATIC
EXTRA
HEPATIC
Hepatocyte
canaliculi
terminal bileduct
Intrahepatic
intralobular bileduct
interlobular bileduct
septal bileduct
right
hepatic
duct
left
hepatic
duct
Extrahepatic
Cystic duct
Choledochal duct
Pancreatic duct
BILIARY TRACT
duodenum
CHOLESTASIS
STAGNATION/INTERFERENCE OF BILE FLOW
CONSEQUENCES
DEFECT OF
CANALICULAR
BILE SECRETION
ACCUMULATION
RETENTION
IN
THE BLOOD
protein
cholesterol
HEPATOCYTE CHOLESTASIS
INTERFERENCES OF:
1.
2.
3.
CHOLEPOEIESIS
SECRETION
CANALICULAR CONTRACTION
OBSTRUCTIVE CHOLESTASIS
= DUCTS
1.
2.
3.
4.
INFECTION
NON INFECTION
INFLAMMATION
EMBRYOGENESIS
CHOLANGIOPATHIA
INFANTILE OBSTR.
OBSTRUCTION
PROXIMAL PRESS.
SECRETION
DAMAGE OF
HEPATOCYTE
SUPERSATURATION
ACCUMULATION
OF CHEMICAL
AGENT
ISCHEMIC OF
DUCTS WALL
HEPATITIS
OBSTRUCTION
CONSEQUENCES OF OBSTRUCTIVE
CHOLESTASIS
INFECTION
CHOLANGITIS
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
Portal Vein
AN
TR
R
LA
U
LL
E
C
entero hepatic
circulation
Central Vein
PARACELLULAR
SPACE OF DISSE
HEPATOCYTE
DUCT
BILE ACID
CIRCULATION
BOWEL
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
- EHBA
- INTRAHEPATIC
CHOLESTASIS
CHILDREN
- VIRAL HEPATITIS
- MECHANICAL
OBSTR.
- INTRAHEPATIC
CHOLESTASIS
EHBA
OPERATIVE
CORRECTABLE
PARTIAL ATRESIA
UNCORRECTABLE
TOTAL ATRESIA
KASAI OPERATION
HEPATITIS
= INFLAMMATION OF HEPATOCYTE
HEPATOTROPIC
VIRAL
INFECTION
BACTERIA
NON
HEPATOTROPIC
PARASITES
HEPATITIS
DRUGS
NONINFECTION
TOXIN
METABOLIC
INFARCT
Ag-Ab
HEPATOTROPIC VIRAL
A
HEP. INFEKSIOSA
HEP. B
HEP. C
HEP. DELTA
HEP. E
??
HEP.G
PRODROMAL
STADIUM
ICTERUS= FEVER(-)
RECOVERY
RECOVERY
ACUTE
VIRAL
HEPATITIS
FULMINANT HEPATIC
FAILURE
CHRONIC HEP.
(SGPT >= 6 MONTHS )
PROGRESSIVE
CHRONIC
HEPATIC
CIRRHOSIS
CARRIER
VIRAL HEPATITIS
SYMPTOMATIC
ICTERIC
ANICTERIC
FLU LIKE
ASYMPTOMATIC
SUBCLINICAL
BIOCHEMISTRY
INAPPARENT
INFECTION
SEROLOGY
eg. IgM ANTI HAV (+)
HEPATITIS. A
TREATMENT :
1.BED-REST
2.WATER & ELECTROLYTES :
PREVENTION OF
DEHYDRATION
3.DIET : FAT ISNT LIMITATED
HEPATIC CIRRHOSIS
- FIBROSIS(+)
- NODULE (+)
LIVER
DYSFUNCTION
HYPERSPLENISM
PORTAL
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.
CORONARY V.
PANCREATICO
DUODENAL V.
RIGHT PORTAL V.
ESOPH. VARICES
LEFT PORTAL V.
HEPATIC VEIN
HEART
DIAGRAM
OF PORTAL
VENOUS
INTRAHEPATIC
PORTAL
HYPERTENSION
PRE HEP
THROMBOSIS
V.UMBILICALIS
EXTRAHEPATIC
(50-70%)
POST HEP.
BUDD CHIARY
SYNDR.
POST
SINUSOIDAL
TERMINAL
HEPATIC
VENO
OCCLUSIVE
DISEASE
VENULE
SINUSOIDAL
HEPATIC
CIRRHOSIS
INTRAHEPATIC
TERMINAL
PORTAL
PRE SINUSOIDAL
VENULE
SCHISTOSO
MIASIS
BLEEDING IN PORTAL
HYPERTENSION
INTRAHEPATIC
EXTRAHEPATIC
FREQUENCY
>
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SEVERITY
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LIFE EXPECTANCE
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ASCITES
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- JAUNDICE
- PALMAR ERYTHEMA
- VASCULAR SPIDER+
- LIVER DYSFUNCTION