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Hyperbilirubinemia

Jaundice, also known as icterus (adjective:"Icteric"), is yellowing of the skin, sclera (the white of the eyes) and mucous membranes caused by increased levels of bilirubin in the human body. Usually the concentration of bilirubin in the blood must exceed 2–3mg/dL for the coloration to be easily visible. Jaundice comes from the French word jaune, meaning yellow. more...

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Causes of jaundice

When red blood cells die, the heme in their hemoglobin is converted to bilirubin in the spleen and in the kupffer cells in the liver. The bilirubin is processed by the liver, enters bile and is eventually excreted through feaces.

Consequently, there are three different classes of causes for jaundice. Pre-hepatic or hemolytic causes, where too many red blood cells are broken down, hepatic causes where the processing of bilirubin in the liver does not function correctly, and post-hepatic or extrahepatic causes, where the removal of bile is disturbed.

Pre-hepatic

Pre-hepatic (or hemolytic) jaundice is caused by anything which causes an increased rate of hemolysis (breakdown of red blood cells). In tropical countries, malaria can cause jaundice in this manner. Certain genetic diseases, such as glucose 6-phosphate dehydrogenase deficiency can lead to increase red cell lysis and therefore hemolytic jaundice. Defects in bilirubin metabolism also present as jaundice. Jaundice usually comes with high fevers.

Hepatic

Hepatic causes include acute hepatitis, hepatotoxicity and alcoholic liver disease. Less common causes include primary biliary cirrhosis, Gilbert's syndrome and metastatic carcinoma. Jaundice commonly seen in the newborn baby is another example of hepatic jaundice.

Post-hepatic

Post-hepatic (or obstructive) jaundice, also called cholestasis, is caused by an interruption to the drainage of bile in the biliary system. The most common causes are gallstones in the common bile duct and pancreatic cancer in the head of the pancreas. Other causes include strictures of the common bile duct, ductal carcinoma, pancreatitis and pancreatic pseudocysts. A rare cause of obstructive jaundice is Mirizzi's syndrome.

The presence of pale stools suggests an obstructive or post-hepatic cause as normal feces get their colour from bile pigments.

Neonatal jaundice

Neonatal jaundice is usually harmless: this condition is often seen in infants around the second day after birth, lasting till day 8 in normal births, or to around day 14 in premature births. Serum bilirubin normally drops to a low level without any intervention required: the jaundice is presumably a consequence of metabolic and physiological adjustments after birth. Infants with neonatal jaundice are often treated with bili lights, exposing them to high levels of colored light to break down the bilirubin. This works due to a photo oxidation process occurring on the bilirubin in the subcutaneous tissues of the neonate. Light energy creates isomerization of the bilirubin and consequently transformation into compounds that the new born can excrete via urine and stools. Blue light is typically used for this purpose. Green light is more effective at breaking down bilirubin, but is not commonly used because it makes the babies appear sickly, which is disturbing to observers.

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Neonatal jaundice
From Gale Encyclopedia of Medicine, 4/6/01 by Altha Roberts Edgren

Definition

Neonatal jaundice (or hyperbilirubinemia) is a higher-than-normal level of bilirubin in the blood. Bilirubin is a by-product of the breakdown of red blood cells. This condition can cause a yellow discoloration of the skin and the whites of the eyes called jaundice.

Description

Bilirubin, a by-product of the breakdown of hemoglobin (the oxygen-carrying substance in red blood cells), is produced when the body breaks down old red blood cells. Normally, the liver processes the bilirubin and excretes it in the stool. Hyperbilirubinemia means there is a high level of bilirubin in the blood. This condition is particularly common in newborn infants. Before birth, an infant gets rid of bilirubin through the mother's blood and liver systems. After birth, the baby's liver has to take over processing bilirubin on its own. Almost all newborns have higher than normal levels of bilirubin. In most cases, the baby's systems continue to develop and can soon process bilirubin. However, some infants may need medical treatment to prevent serious complications which can occur due to the accumulation of bilirubin.

Causes & symptoms

In newborn infants, the liver and intestinal systems are immature and cannot excrete bilirubin as fast as the body produces it. This type of hyperbilirubinemia can cause jaundice to develop within a few days after birth. About one-half of all newborns develop jaundice, while premature infants are much more likely to develop it. Hyperbilirubinemia is also more common in some populations, such as Native American and Asian. All infants with jaundice should be evaluated by a health care provider to rule out more serious problems.

Hyperbilirubinemia and jaundice can also be the result of other diseases or conditions. Hepatitis, cirrhosis of the liver, and mononucleosis are diseases that can affect the liver. Gallstones, a blocked bile duct, or the use of drugs or alcohol can also cause jaundice.

Extremely high levels of bilirubin in infants may cause kernicterus, a form of brain damage. Signs of severe hyperbilirubinemia include listlessness, high-pitched crying, apnea (periods of not breathing), arching of the back, and seizures. If severe hyperbilirubinemia is not treated, it can cause mental retardation, hearing loss, behavior disorders, cerebral palsy, or death.

Diagnosis

The initial diagnosis of hyperbilirubinemia is based on the appearance of jaundice at physical examination. The child is often placed by an open window so he/she may be checked in natural light. Blood samples may be taken to determine the bilirubin level in the blood.

Treatment

Most cases of newborn jaundice resolve without medical treatment within two to three weeks, but should be checked by the health care provider. It is important that the infant is feeding regularly and having normal bowel movements. If bilirubin levels are extremely high, the infant may be treated with phototherapy -- exposure of the baby's skin to fluorescent light. The bilirubin in the baby's skin absorbs the light and is changed to a substance that can be excreted in the urine. This treatment can be done in the hospital and is often done at home with special lights which parents can rent for the treatment. Treatment may be needed for several days before bilirubin levels in the blood return to normal. The baby's eyes are shielded to prevent the optic nerves from absorbing too much light. Another type of treatment uses a special fiberoptic blanket. There is no need to shield the baby's eyes with this treatment, and it can be done at home. In rare cases, where bilirubin levels are extremely high, the baby may need to receive a blood transfusion.

Prognosis

Most infants with hyperbilirubinemia and associated jaundice recover without medical treatment. Phototherapy is very effective in reducing bilirubin levels in the majority of infants who need it. There are usually no long-term effects on the child from the hyperbilirubinemia or the phototherapy. It is very rare that a baby may need a blood transfusion for treatment of this condition.

Prevention

There is no way to predict which infants will be affected by hyperbilirubinemia. Newborns should be breastfed or given formula frequently, and feedings should begin as soon as possible after delivery to increase activity of the baby's digestive system.

Key Terms

Bilirubin
A yellowish-brown substance in the blood that forms as old red blood cells are broken down.
Hemoglobin
A protein, an oxygen-carrying pigment of the erythrocyte (red blood cell) formed in the bone marrow.
Jaundice
A yellow discoloration of the skin and whites of the eyes.
Kernicterus
A serious condition where high bilirubin levels cause brain damage in infants.

Further Reading

For Your Information

    Books

  • "Hyperbilirubinemia." In The Merck Manual of Diagnosis and Therapy, edited by Robert Berkow. Rahway, NJ: Merck Research Laboratories, 1992.

    Other

  • D'Alessandro, Hellen Anne. Biliary Atresia. The Virtual Hospital, University of Iowa at http://www.vh.org/Providers/Textbooks/ElectricGiNucs/Text/BiliaryAtresia.html.
  • Jaundice in Newborn (Hyperbilirubinemia). http://www.healthwise.com/ivillage/kbase/kbindex.asap.
  • Jaundice/Hyperbilirubinemia. http://www2.medsch.wisc.edu/childrenshosp/Parents_of_Preemies/jaundice.html.
  • Kernicterus in Cedars-Sinai Medical Center Newborn Intensive Care Unit Teaching Files. http://external.csmc.edu/neonatology/syllabus/kernicterus.html.
  • Neonatal Jaundice. http://www.gi.vghtc.gov.tw/Teaching/Biliary/Jaundice/s13.htm.

Gale Encyclopedia of Medicine. Gale Research, 1999.

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