Jaundice

Jaundice Overview

Jaundice is a yellow discoloration of the skin, mucous membranes, and the whites of the eyes caused by increased amounts of bilirubin in the blood. Jaundice is a sign of an underlying disease process.

Bilirubin is a by-product of the daily natural breakdown and destruction of red blood cells in the body. The hemoglobin molecule that is released into the blood by this process is split, with the heme portion undergoing a chemical conversion to bilirubin. Normally, the liver metabolizes and excretes the bilirubin in the form of bile. However, if there is a disruption in this normal metabolism and/or production of bilirubin, jaundice may result.

Jaundice Causes

Jaundice may be caused by several different disease processes. It is helpful to understand the different causes of jaundice by identifying the problems that disrupt the normal bilirubin metabolism and/or excretion.

Pre-hepatic (before bile is made in the liver)

Jaundice in these cases is caused by rapid increase in the breakdown and destruction of the red blood cells (hemolysis), overwhelming the liver's ability to adequately remove the increased levels of bilirubin from the blood.

Examples of conditions with increased breakdown of red blood cells include:
malaria,

sickle cell crisis,

spherocytosis,

thalassemia,

glucose-6-phosphate dehydrogenase deficiency (G6PD),

drugs or other toxins, and

autoimmune disorders.

Hepatic (the problem arises within the liver)

Jaundice in these cases is caused by the liver's inability to properly metabolize and excrete bilirubin. Examples include:
hepatitis (commonly viral or alcohol related),

cirrhosis,

drugs or other toxins,

Crigler-Najjar syndrome,

Gilbert's syndrome, and

cancer.

Post-hepatic (after bile has been made in the liver)

Jaundice in these cases, also termed obstructive jaundice, is caused by conditions which interrupt the normal drainage of conjugated bilirubin in the form of bile from the liver into the intestines.

Causes of obstructive jaundice include:
gallstones in the bile ducts,

cancer (pancreatic and gallbladder/bile duct carcinoma),

strictures of the bile ducts,

cholangitis,

congenital malformations,

pancreatitis,

parasites,

pregnancy, and

newborn jaundice.

Jaundice in newborn babies can be caused by several different conditions, although it is often a normal physiological consequence of the newborn's immature liver. Even though it is usually harmless under these circumstances, newborns with excessively elevated levels of bilirubin from other medical conditions (pathologic jaundice) may suffer devastating brain damage (kernicterus) if the underlying problem is not addressed. Newborn jaundice is the most common condition requiring medical evaluation in newborns.

The following are some common causes of newborn jaundice:

Physiological jaundice

This form of jaundice is usually evident on the second or third day of life. It is the most common cause of newborn jaundice and is usually a transient and harmless condition. Jaundice is caused by the inability of the newborn's immature liver to process bilirubin from the accelerated breakdown of red blood cells that occurs at this age. As the newborn's liver matures, the jaundice eventually disappears.

Maternal-fetal blood group incompatibility (Rh, ABO)

This form of jaundice occurs when there is incompatibility between the blood types of the mother and the fetus. This leads to increased bilirubin levels from the breakdown of the fetus' red blood cells (hemolysis).

Breast milk jaundice

This form of jaundice occurs in breastfed newborns and usually appears at the end of the first week of life. Certain chemicals in breast milk are thought to be responsible. It is usually a harmless condition that resolves spontaneously. Mothers typically do not have to discontinue breastfeeding.

Breastfeeding jaundice

This form of jaundice occurs when the breastfed newborn does not receive adequate breast milk intake. This may occur because of delayed or insufficient milk production by the mother or because of poor feeding by the newborn. This inadequate intake results in dehydration and fewer bowel movements for the newborn, with subsequent decreased bilirubin excretion from the body.

Cephalohematoma (a collection of blood under the scalp)

Sometimes during the birthing process, the newborn may sustain a bruise or injury to the head, resulting in a blood collection/blood clot under the scalp. As this blood is naturally broken down, sudden elevated levels of bilirubin may overwhelm the processing capability of the newborn's immature liver, resulting in jaundice.

Jaundice Symptoms

Jaundice is a sign of an underlying disease process. .

Common signs and symptoms seen in individuals with jaundice include:
yellow discoloration of the skin, mucous membranes, and the whites of the eyes,

light-colored stools,

dark-colored urine, and

itching of the skin.

The underlying disease process may result in additional signs and symptoms. These may include:
nausea and vomiting,

abdominal pain,

fever,

weakness,

loss of appetite,

headache,

confusion,

swelling of the legs and abdomen, and

newborn jaundice.

In newborns, as the bilirubin level rises, jaundice will typically progress from the head to the trunk, and then to the hands and feet. Additional signs and symptoms that may be seen in the newborn include:
poor feeding,

lethargy,

changes in muscle tone,

high-pitched crying, and

seizures.

What is neonatal jaundice (jaundice in newborn infants)?

Neonatal jaundice is jaundice that begins within the first few days after birth. (Jaundice that is present at the time of birth suggests a more serious cause of the jaundice.) In fact, bilirubin levels in the blood become elevated in almost all infants during the first few days following birth, and jaundice occurs in more than half. For all but a few infants, the elevation and jaundice represents a normal physiological phenomenon and does not cause problems.


The cause of normal, physiological jaundice is well understood. During life in the uterus, the red blood cells of the fetus contain a type of hemoglobin that is different than the hemoglobin that is present after birth. When an infant is born, the infant's body begins to rapidly destroy the red blood cells containing the fetal-type hemoglobin and replaces them with red blood cells containing the adult-type hemoglobin. This floods the liver with bilirubin derived from the fetal hemoglobin from the destroyed red blood cells. The liver in a newborn infant is not mature, and its ability to process and eliminate bilirubin is limited. As a result of both the influx of large amounts of bilirubin and the immaturity of the liver, bilirubin accumulates in the blood. Within two or three weeks, the destruction of red blood cells ends, the liver matures, and the bilirubin levels return to normal.

There is another uncommon syndrome associated with neonatal jaundice, referred to as breast-milk or breast feeding jaundice. In this syndrome, jaundice appears to be caused by or at least accentuated by breast feeding. Although the cause of this type of jaundice is unknown, it has been hypothesized that there is something in breast milk that reduces the ability of the liver to process and eliminate bilirubin. With breast-milk jaundice, the bilirubin levels rise and reach peak levels in approximately two weeks, remain elevated for a week or so, and then decline to normal over several weeks or months. This timing of the elevation in bilirubin and jaundice is different than normal physiological jaundice described previously and allows the two causes of jaundice to be differentiated. The real importance of the more prolonged jaundice associate with breast-milk jaundice is that it raises the possibility that there is a more serious cause for the jaundice that needs to be sought, for example, biliary atresia (destruction of the bile ducts). Breast-milk jaundice alone usually does not cause problems for the infant.

Physiologic jaundice and breast-milk jaundice usually do not cause problems for the infant; however, there is a concern that high or prolonged elevations in levels of unconjugated bilirubin (the type of bilirubin that is not attached to glucuronic acid and the main type of bilirubin that is present in physiologic and breast-milk jaundice) will cause neurologic damage to the infant. Therefore, when unconjugated bilirubin levels are high or prolonged, treatment usually is started to lower the levels of bilirubin. Treatment may be started earlier in infants who are born prematurely since their livers take longer to mature, and the risk of higher and more prolonged elevations of bilirubin is greater. Treatment involves phototherapy with artificial or natural sunlight and, if phototherapy is not successful, exchange transfusion in which the infant's blood is exchanged for normal blood from blood donors.

The benign nature of physiologic and breast-milk allergy need to be distinguished from hemolytic disease of the newborn, a much more serious, even life-threatening cause of jaundice in newborns that is due to blood group incompatibilities between mother and fetus, for example Rh incompatibility. The incompatibility results in an attack by the mother's antibodies on the babies red blood cells leading to hemolysis. Fortunately, because of modern management of pregnancy, this cause of jaundice is rare.

Exams and Tests

The health care pracitioner will need to take a detailed history of the patient's illness, and he or she will also be examined to see if there are any findings that indicate the cause of the patient's jaundice. However, additional testing is usually required to clearly determine the underlying cause of jaundice. The following tests and imaging studies may be obtained:

Blood tests

These may initially include a complete blood count (CBC), liver function tests (including a bilirubin level), lipase/amylase level to detect inflammation of the pancreas (pancreatitis), and an electrolytes panel. In women, a pregnancy test may be obtained. Additional blood tests may be required depending upon the initial results and the history provided to the practitioner.

Urinalysis: Urinalysis is an analysis of the urine and is a very useful test in the diagnosis of screening many diseases.

Imaging Studies
 
Ultrasound: This is a safe, painless imaging study that uses sound waves to examine the liver, gallbladder, and pancreas. It is very useful for detecting gallstones and dilated bile ducts. It can also detect abnormalities of the liver and the pancreas.

Computerized tomography (CT) scan: A CT scan is imaging study similar to an X-ray that provides more details of all the abdominal organs. Though not as good as ultrasound at detecting gallstones, it can identify various other abnormalities of the liver, pancreas, and other abdominal organs as well.

Cholescintigraphy (HIDA scan): A HIDA scan is an imaging study that uses a radioactive substance to evaluate the gallbladder and the bile ducts.

Magnetic resonance imaging (MRI): MRI is an imaging study that uses a magnetic field to examine the organs of the abdomen. It can be useful for detailed imaging of the bile ducts.

Endoscopic retrograde cholangiopancreatography (ERCP): ERCP is a procedure that involves the introduction of an endoscope (a tube with a camera at the end) through the mouth and into the small intestine. A dye is then injected into the bile ducts while X-rays are taken. It can be useful for identifying stones, tumors, or narrowing of the bile ducts.

Liver Biopsy
 
In this procedure, a needle is inserted into the liver after a local anesthetic has been administered. Often ultrasound will be used to guide placement of the needle. The small sample of liver tissue which is obtained is sent to a laboratory for examination by a pathologist (a physician who specializes in diagnosis of tissue samples). Among other things, a liver biopsy can be useful for diagnosing inflammation of the liver, cirrhosis, and cancer.
 
How is jaundice treated? 

With the exception of the treatments for specific causes of jaundice mentioned previously, the treatment of jaundice usually requires a diagnosis of the specific cause of the jaundice and treatment directed at the specific cause, e.g., removal of a gallstone blocking the bile duct.

Self-Care at Home

The objectives of home therapy include symptom relief and managing the medical condition causing the underlying jaundice. The various measures that may be undertaken include:
Maintain adequate hydration by drinking fluids, and rest as needed.

Take medications only as instructed and prescribed by a health care practitioner.

Avoid medications, herbs, or supplements which may cause detrimental side effects. Consult a health care practitioner for advice.

Avoid drinking alcohol until the patient has discussed it with their health care practitioner.

Certain dietary restrictions may be recommended by a health care practitioner.

In certain cases of newborn jaundice, the parents or caregivers can place the baby next to a well lit window a few times a day to decrease elevated bilirubin levels. In more severe cases, a health care practitioner may need to discharge the baby home from the hospital with home phototherapy.

Provide adequate milk intake for the baby in cases of breastfeeding jaundice.

If symptoms worsen or if any new symptoms arise, consult a health care practitioner.

Medical Treatment

Treatment varies based on the medical condition responsible for causing jaundice, and the associated symptoms and complications. Treatments may include the following:
supportive care,

IV fluids in cases of dehydration,

medications for nausea/vomiting and pain,

antibiotics,

antiviral medications,

blood transfusions,

steroids,

chemotherapy/radiation therapy, and

phototherapy (newborns).