2. JAUNDICE
The word jaundice comes from the French word
jaune, which means āyellowā
ā¢ Jaundice, or icterus, is used to describe the
yellow discoloration of the skin, eyes, and
mucous membranes most often resulting from
the retention of bilirubin
ā¢ Jaundice is most commonly classified based
on the site of the disorder: prehepatic,
hepatic, and posthepatic jaundice.
3. Jaundice cont
ā¢ Prehepatic and posthepatic jaundice, as the
names imply, are caused by abnormalities
outside of the liver, either before, as in
āprehepatic,ā or after, as in āposthepatic.ā
ā¢ In these conditions, liver function is normal
ā¢ This is not the case with hepatic jaundice,
where the jaundice is due to a problem with
the liver itselfāan intrinsic liver defect or
disease.
4. CAUSES OF JAUNDICE
1.Prehepatic Jaundice
ā¢ Problem causing the jaundice occurs prior to liver
metabolism.
ā¢ most commonly caused by an increased amount
of bilirubin being presented to the liver such as
that seen in acute and chronic hemolytic
anemias.
ā¢ This type of jaundice may also be referred to as
unconjugated hyperbilirubinemia because the
fraction of bilirubin increased in people with
prehepatic jaundice is the unconjugated fraction.
5. 2. Hepatic Jaundice
ā¢ Occurs when the primary problem causing the
jaundice resides in the liver (intrinsic liver
defect or disease).
ā¢ This intrinsic liver defect or disease can be due
to disorders of bilirubin metabolism and
transport defects (Crigler-Najjar syndrome,
Dubin-Johnson syndrome, Gilbert disease, and
neonatal physiologic jaundice of the newborn)
or due to diseases resulting in hepatocellular
injury or destruction.
6. 2. Hepatic Jaundice cont.
ā¢ Gilbert disease, Crigler-Najjar syndrome, and
physiologic jaundice of the newborn are
hepatic causes of jaundice that result in
elevations in unconjugated bilirubin.
ā¢ Conditions such as Dubin-Johnson and Rotor
syndrome are hepatic causes of jaundice that
result in elevations in conjugated bilirubin.
7. 2. Hepatic Jaundice cont.
a) Gilbert Syndrome
ā¢ It is a benign hereditary disorder characterized by
intermittent unconjugated hyperbilirubinemia in the
absence of hemolysis and underlying liver disease
due to a defective conjugation system.
ā¢ usually manifests during adolescence or early
adulthood.
ā¢ The molecular basis of Gilbert syndrome is related to
the glucuronyltransferase superfamily, which is
responsible for encoding enzymes that catalyze the
conjugation of bilirubin.
8. 2. Hepatic Jaundice cont.
b) Crigler-Najjar syndrome
ā¢ Is a syndrome of chronic nonhemolytic
unconjugated hyperbilirubinemia.
ā¢ is an inherited disorder of bilirubin
metabolism resulting from a molecular defect
within the gene involved with bilirubin
conjugation.
ā¢ Crigler-Najjar syndrome may be divided into
two types:
9. ā¢ type 1, where there is a complete absence of
enzymatic bilirubin conjugation, and type II,
where there is a mutation causing a severe
deficiency of the enzyme responsible for
bilirubin conjugation.
ā¢ Crigler-Najjar syndrome is rare and is a more
serious disorder than Gilbert syndrome
10. 2. Hepatic Jaundice cont.
C) Physiologic jaundice of the newborn
ā¢ is a result of a deficiency in the enzyme
glucuronyl transferase, one of the last liver
functions to be activated in prenatal life since
bilirubin processing is handled by the mother
of the fetus.
ā¢ This deficiency results in rapid buildup of
unconjugated bilirubin, which can be life
threatening
11. ā¢ When this type of bilirubin builds up in the
neonate, it cannot be processed and it is
deposited in the nuclei of brain and nerve
cells, causing kernicterus.
ā¢ Kernicterus often results in cell damage and
death in the newborn, and this condition will
continue until glucuronyl transferase is
produced.
ā¢ usually treated with UV radiation to destroy
the bilirubin some infants may require an
exchange transfusion.
12. 2. Hepatic Jaundice cont.
d) Dubin-Johnson syndrome
ā¢ Rare inherited disorder caused by a deficiency
of the canalicular transporter protein
ā¢ The liverās ability to uptake and conjugate
bilirubin is functional but the removal of
conjugated bilirubin from the liver cell and the
excretion into the bile are defective.
13. ā¢ This results in accumulation of conjugated
and, to some extent, unconjugated bilirubin in
the blood, leading to hyperbilirubinemia and
bilirubinuria.
ā¢ A distinguishing feature of Dubin-Johnson
syndrome is the appearance of dark-stained
granules (thought to be pigmented lysosomes)
on a liver biopsy sample.
ā¢ People with Dubin- Johnson have a normal life
expectancy, so no treatment is necessary
14. 2. Hepatic Jaundice cont.
e) Rotor syndrome
ā¢ defect causing Rotor syndrome is not Known.
ā¢ It is hypothesized to be due to a reduction in
the concentration or activity of intracellular
binding proteins such as ligandin.
ā¢ liver biopsy does not show dark pigmented
granules.
ā¢ Excellent prognosis, and therefore treatment
is not warranted.
ā¢ clinically similar to Dubin-Johnsonsyndrome
15. 3. Posthepatic jaundice
ā¢ Results from biliary obstructive disease,
usually from physical obstructions (gallstones
or tumors), that prevent the flow of
conjugated bilirubin into the bile canaliculi.
ā¢ Since the liver itself is functioning, bilirubin is
effectively conjugated but it is unable to be
properly excreted from the liver.
ā¢ Since bile is not being brought to the
intestines, stool loses its pigment and
becomes clay-colored.
16. Effects of jaundice
Eyes, Skin, Mucous Membranes
ā¢ causes a yellow tint in the whites of the eyes, skin
and mucous membranes
Dark Urine
ā¢ It typically occurs because of bile duct blockage due
to gallstones, cysts, tumors,
Change in Stool Color
ā¢ may turn pale or a clay color. This change in stool
color is also linked to a blocked bile duct. This
symptom is often accompanied by vomiting or
nausea and a fever