Jaundice is a condition in which a person's skin and the whites
of the eyes are discolored yellow due to an increased level of
bile pigments in the blood resulting from liver disease.
Jaundice is sometimes called icterus, from a Greek word for the
In order to understand jaundice, it is useful to know about the
role of the liver in producing bile. The most important function
of the liver is the processing of chemical waste products like
cholesterol and excreting them into the intestines as bile. The
liver is the premier chemical factory in the body most incoming
and outgoing chemicals pass through it. It is the first stop for
all nutrients, toxins, and drugs absorbed by the digestive
tract. The liver also collects chemicals from the blood for
Many of these outward-bound chemicals are excreted into the
bile. One particular substance, bilirubin, is yellow. Bilirubin
is a product of the breakdown of hemoglobin, which is the
protein inside red blood cells. If bilirubin cannot leave the
body, it accumulates and discolors other tissues. The normal
total level of bilirubin in blood serum is between 0.2 mg/dL and
1.2 mg/dL. When it rises to 3 mg/dL or higher, the person's skin
and the whites of the eyes become noticeably yellow.
Bile is formed in the liver. It then passes into the network of
hepatic bile ducts, which join to form a single tube. A branch
of this tube carries bile to the gallbladder, where it is
stored, concentrated, and released on a signal from the stomach.
Food entering the stomach is the signal that stimulates the
gallbladder to release the bile. The tube, which is now called
the common bile duct, continues to the intestines. Before the
common bile duct reaches the intestines, it is joined by another
duct from the pancreas. The bile and the pancreatic juice enter
the intestine through a valve called the ampulla of Vater. After
entering the intestine, the bile and pancreatic secretions
together help in the process of digestion.
Causes and Symptoms
There are many different causes for jaundice, but they can be
divided into three categories based on where they start—before,
in, or after the liver (pre-hepatic, hepatic and post-hepatic).
When bilirubin begins its life cycle, it cannot be dissolved in
water. The liver changes it so that it is soluble in water.
These two types of bilirubin are called unconjugated (insoluble)
and conjugated (soluble). Blood tests can easily distinguish
between these two types of bilirubin.
Hemoglobin and bilirubin formation
Bilirubin begins as hemoglobin in
the blood-forming organs, primarily the bone marrow. If the
production of red blood cells (RBCs) falls below normal, the
extra hemoglobin finds its way into the bilirubin cycle and adds
to the pool.
Once hemoglobin is in the red cells of the blood, it circulates
for the life span of those cells. The hemoglobin that is
released when the cells die is turned into bilirubin. If for any
reason the RBCs die at a faster rate than usual, bilirubin can
accumulate in the blood and cause jaundice.
Many disorders speed up the death
of red blood cells. The process of red blood cell destruction is
called hemolysis, and the diseases that cause it are called
hemolytic disorders. If red blood cells are destroyed faster
than they can be produced, the patient develops anemia.
Hemolysis can occur in a number of diseases, disorders,
conditions, and medical procedures:
Normal Jaundice in Newborns
malaria parasite develops inside red blood cells. When it is
mature it breaks the cell apart and swims off in the blood.
This process happens to most of the parasites
simultaneously, causing the intermittent symptoms of the
disease. When enough cells burst at once, jaundice may
result from the large amount of bilirubin formed from the
hemoglobin in the dead cells. The pigment may reach the
urine in sufficient quantities to cause "blackwater fever,"
an often lethal form of malaria.
Side effects of certain drugs. Some
common drugs can cause hemolysis as a rare but sudden side
effect. These medications include some antibiotic and
anti-tuberculosis medicines; drugs that regulate the
heartbeat; and levodopa, a drug used to treat Parkinson's
Certain drugs in combination. with a
hereditary enzyme deficiency known as glucose-6-phosphate
dehydrogenase (G6PD). G6PD is a deficiency that affects over
200 million people in the world. Some of the drugs listed
above are more likely to cause hemolysis in people with
G6PD. Other drugs cause hemolysis only in people with this
disorder. Most important among these drugs are anti-malarial
medications such as quinine, and vitamins C and K.
Poisons. Snake and spider venom,
certain bacterial toxins, copper, and some organic
industrial chemicals directly attack the membranes of red
Artificial heart valves. The
inflexible moving parts of heart valves damage RBCs as they
flutter back and forth. This damage is one reason to
recommend pig valves and valves made of other organic
Hereditary RBC disorders. There are a
number of hereditary defects that affect the blood cells.
There are many genetic mutations that affect the hemoglobin
itself, the best-known of which is sickle cell disease. Such
hereditary disorders as spherocytosis weaken the outer
membrane of the red cell. There are also inherited defects
that involve the internal chemistry of RBCs.
Enlargement of the spleen. The spleen
is an organ that is located near the upper end of the
stomach and filters the blood. It is supposed to filter out
and destroy only worn-out RBCs. If it has become enlarged,
it filters out normal cells as well. Malaria, other
infections, cancers and leukemias, some of the hereditary
anemias mentioned above, obstruction of blood flow from the
spleen—all these and many more diseases can enlarge the
spleen to the point where it removes too many red blood
Diseases of the small blood vessels.
Hemolysis that occurs in diseased small blood vessels is
called microangiopathic hemolysis. It results from damage
caused by rough surfaces on the inside of the capillaries.
The RBCs squeeze through capillaries one at a time and can
easily be damaged by scraping against the vessel walls.
Immune reactions to RBCs. Several
types of cancer and immune system diseases produce
antibodies that react with RBCs and destroy them. In 75% of
cases, this reaction occurs all by itself, with no
underlying disease to account for it.
If a patient is given an incompatible blood type, hemolysis
Kidney failure and other serious diseases.
Several diseases are characterized by defective blood
coagulation that can destroy red blood cells.
Erythroblastosis fetalis is a disease of newborns marked by
the presence of too many immature red blood cells
(erythroblasts) in the baby's blood. When a baby's mother
has a different blood type, antibodies from the mother may
leak into the baby's circulation and destroy blood cells.
This reaction can produce severe hemolysis and jaundice in
the newborn. Rh factor incompatibility is the most common
High bilirubin levels in newborns.
Even in the absence of blood type incompatibility, the
newborn's bilirubin level may reach threatening levels.
Normal newborn jaundice is the result of two conditions
occurring at the same time—a pre-hepatic and a hepatic source of
excess bilirubin. First of all, the baby at birth immediately
begins converting hemoglobin from a fetal type to an adult type.
The fetal type of hemoglobin was able to extract oxygen from the
lower levels of oxygen in the mother's blood. At birth the
infant can extract oxygen directly from his or her own lungs and
does not need the fetal hemoglobin any more. So fetal hemoglobin
is removed from the system and replaced with adult hemoglobin.
The resulting bilirubin loads the system and places demands on
the liver to clear it. But the liver is not quite ready for the
task, so there is a period of a week or so when the liver has to
catch up. During that time the baby is jaundiced.
Liver diseases of all kinds threaten the organ's ability to keep
up with bilirubin processing. Starvation, circulating
infections, certain medications, hepatitis, and cirrhosis can
all cause hepatic jaundice, as can certain hereditary defects of
liver chemistry, including Gilbert's syndrome and Crigler-Najjar
Post Hepatic Jaundice
Post-hepatic forms of jaundice include the jaundices caused by
failure of soluble bilirubin to reach the intestines after it
has left the liver. These disorders are called obstructive
jaundices. The most common cause of obstructive jaundice is the
presence of gallstones in the ducts of the biliary system. Other
causes have to do with birth defects and infections that damage
the bile ducts; drugs; infections; cancers; and physical injury.
Some drugs and pregnancy on rare occasions simply cause the bile
in the ducts to stop flowing.
Symptoms and Complications Associated with
Certain chemicals in bile may cause itching when too much of
them ends up in the skin. In newborns, insoluble bilirubin may
get into the brain and do permanent damage. Long standing
jaundice may upset the balance of chemicals in the bile and
cause stones to form. Apart from these potential complications
and the discoloration of skin and eyes, jaundice by itself is
inoffensive. Other symptoms are determined by the disease
producing the jaundice.
Diagnosis of Jaundice
In many cases the diagnosis of jaundice is suggested by the
appearance of the patient's eyes and complexion. The doctor will
ask the patient to lie flat on the examining table in order to
feel (palpate) the liver and spleen for enlargement and to
evaluate any abdominal pain. The location and severity of
abdominal pain and the presence or absence of fever help the
doctor to distinguish between hepatic and obstructive jaundice.
Disorders of blood formation can be diagnosed by
more thorough examination of the blood or the bone marrow, where
blood is made. Occasionally a bone marrow biopsy is required,
but usually the blood itself will reveal the diagnosis. The
spleen can be evaluated by an ultrasound examination or a
nuclear scan if the physical examination has not yielded enough
disease is usually assessed from blood studies alone, but again
a biopsy may be necessary to clarify less obvious conditions. A
liver biopsy is performed at the bedside. The doctor uses a thin
needle to take a tiny core of tissue from the liver. The tissue
sample is sent to the laboratory for examination under a
Assessment of Jaundice in Newborns
are more likely to have problems with jaundice if:
are Asian or Native Americans.
have been bruised during the birth process.
have lost too much weight during the first few days.
are born at high altitude.
mother has diabetes.
had to be induced.
Disease in the biliary system can be identified by imaging
techniques, of which there are many. X rays are taken a day
after swallowing a contrast agent that is secreted into the
bile. This study gives functional as well as anatomical
information. There are several ways of injecting x ray dye
directly into the bile ducts. It can be done through a thin
needle pushed straight into the liver or through a scope passed
through the stomach that can inject dye into the Ampulla of
Vater. CT and MRI scans are very useful for imaging certain
conditions like cancers in and around the liver or gall stones
in the common bile duct.
Prevention of Jaundice
While jaundice cannot be totally prevented, early recognition
and treatment are important in preventing bilirubin levels from
rising to dangerous levels. If your baby's color is turning more
yellow, promptly call your baby's physician.
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