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Bilirubin encephalopathy

Bilirubin-induced neurologic dysfunction (BIND); Kernicterus

Bilirubin encephalopathy is a rare neurological condition that occurs in some newborns with severe jaundice.

Causes

Bilirubin encephalopathy (BE) is caused by very high levels of bilirubin. Bilirubin is a yellow pigment that is created as the body gets rid of old red blood cells. High levels of bilirubin in the body can cause the skin to look yellow (jaundice).

If the level of bilirubin is very high or a baby is very ill, the substance will move out of the blood and collect in the brain tissue if it is not bound to albumin (protein) in the blood. This can lead to problems such as brain damage and hearing loss. The term "kernicterus" refers to the yellow staining caused by bilirubin. This is seen in parts of the brain on autopsy.

This condition most often develops in the first week of life, but may be seen up until the third week. Some newborns with Rh hemolytic disease are at high risk for severe jaundice that can lead to this condition. Rarely, BE can develop in seemingly healthy babies.

Symptoms

The symptoms depend on the stage of BE. Not all babies with kernicterus on autopsy have had definite symptoms.

Early stage:

  • Extreme jaundice
  • Absent startle reflex
  • Poor feeding or sucking
  • Extreme sleepiness (lethargy) and low muscle tone (hypotonia)

Middle stage:

  • High-pitched cry
  • Irritability
  • May have arched back with neck hyperextended backwards, high muscle tone (hypertonia)
  • Poor feeding

Late stage:

  • Stupor or coma
  • No feeding
  • Shrill cry
  • Muscle rigidity, markedly arched back with neck hyperextended backwards
  • Seizures

Exams and Tests

A blood test will show a high bilirubin level (greater than 20 to 25 mg/dL). However, there is not a direct link between bilirubin level and degree of injury.

Normal value ranges may vary slightly among different laboratories. Talk to your doctor about the meaning of your specific test results.

Treatment

Treatment depends on how old the baby is (in hours) and whether the baby has any risk factors (such as prematurity). It may include:

  • Light therapy (phototherapy)
  • Exchange transfusions (removing the child's blood and replacing it with fresh donor blood or plasma)

Outlook (Prognosis)

BE is a serious condition. Many infants with late-stage nervous system complications die.

Possible Complications

Complications may include:

  • Permanent brain damage
  • Hearing loss
  • Death

When to Contact a Medical Professional

Get medical help right away if your baby has signs of this condition.

Prevention

Treating jaundice or conditions that may lead to it can help prevent this problem. Infants with the first signs of jaundice have bilirubin level measured within 24 hours. If the level is high, the infant should be screened for diseases that involve the destruction of red blood cells (hemolysis).

All newborns have a follow-up appointment within 2 to 3 days after leaving the hospital. This is very important for late preterm or early term babies (born more than 2 to 3 weeks before their due date).

References

Hamati AI. Neurological complications of systemic disease: children. In: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, eds. Bradley's Neurology in Clinical Practice. 7th ed. Philadelphia, PA: Elsevier; 2016:chap 59.

Hansen TWR. Pathophysiology of kernicterus. In: Polin RA, Abman SH, Rowitch, DH, Benitz WE, Fox WW, eds. Fetal and Neonatal Physiology. 5th ed. Philadelphia, PA: Elsevier; 2017:chap 164.

Kaplan M, Wong RJ, Sibley E, Stevenson DK. Neonatal jaundice and liver disease. In: Martin RJ, Fanaroff AA, Walsh MC, eds. Fanaroff and Martin's Neonatal-Perinatal Medicine. 10th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 100.

Marcdante KJ, Kliegman RM. Anemia and hyperbilirubinemia. In: Marcdante KJ, Kliegman RM, eds. Nelson Essentials of Pediatrics. 8th ed. Elsevier; 2019:chap 62.

    • Newborn jaundice

      Animation

    •  

      Newborn jaundice - Animation

      Are your newborn baby's skin or eyes yellow? Is she extremely tired and doesn't want to eat? Your baby may have jaundice. Newborn jaundice happens when your baby has high levels of bilirubin in her blood. This yellow pigment is created in the body during the normal recycling of old red blood cells. The liver helps break bilirubin down so it can be removed from the body in the stool. Before a baby is born, the placenta removes the bilirubin from your baby so it can be processed by your liver. Right after birth, the baby's own liver takes over the job, but it can take time. Most babies have some jaundice. It usually appears between the second and third day after birth. Often babies get a screening test in the first 24 hours of life to predict if they are likely to develop jaundice. Your baby's doctor will also watch for signs of jaundice at the hospital, and during follow-up visits after your baby goes home. If your baby seems to have jaundice, the doctor will test the bilirubin levels in her blood. So, how do you treat newborn jaundice? Jaundice usually goes away on its own, so treatment is usually not necessary. If your baby's bilirubin level is too high or rising too quickly, however, she may need treatment. You'll need to keep the baby well hydrated with breast milk or formula. Feeding up to 12 times a day will encourage frequent bowel movements, which help to remove the bilirubin. If your baby needs treatment in the hospital, she may be placed under special blue lights that help break down bilirubin in the baby's skin. This treatment is called phototherapy. If your baby's bilirubin level isn't rising too quickly, you can also do phototherapy at home with a fiberoptic blanket that contains tiny bright lights. For most babies, it takes about a week or two for jaundice to go away. Very high levels of bilirubin, however, can damage a baby's brain. The good news is that this condition, called kernicterus, is almost always diagnosed long before bilirubin levels become high enough to cause damage, and phototherapy treatment will usually make it go away.

    • Kernicterus

      Kernicterus - illustration

      Kernicterus is a condition where very high bilirubin levels in the blood are deposited in the brain tissue causing irreversible damage to the brain. In the newborn, early diagnosis and treatment of jaundice or conditions that lead to jaundice may help prevent kernicterus.

      Kernicterus

      illustration

    • Newborn jaundice

      Animation

    •  

      Newborn jaundice - Animation

      Are your newborn baby's skin or eyes yellow? Is she extremely tired and doesn't want to eat? Your baby may have jaundice. Newborn jaundice happens when your baby has high levels of bilirubin in her blood. This yellow pigment is created in the body during the normal recycling of old red blood cells. The liver helps break bilirubin down so it can be removed from the body in the stool. Before a baby is born, the placenta removes the bilirubin from your baby so it can be processed by your liver. Right after birth, the baby's own liver takes over the job, but it can take time. Most babies have some jaundice. It usually appears between the second and third day after birth. Often babies get a screening test in the first 24 hours of life to predict if they are likely to develop jaundice. Your baby's doctor will also watch for signs of jaundice at the hospital, and during follow-up visits after your baby goes home. If your baby seems to have jaundice, the doctor will test the bilirubin levels in her blood. So, how do you treat newborn jaundice? Jaundice usually goes away on its own, so treatment is usually not necessary. If your baby's bilirubin level is too high or rising too quickly, however, she may need treatment. You'll need to keep the baby well hydrated with breast milk or formula. Feeding up to 12 times a day will encourage frequent bowel movements, which help to remove the bilirubin. If your baby needs treatment in the hospital, she may be placed under special blue lights that help break down bilirubin in the baby's skin. This treatment is called phototherapy. If your baby's bilirubin level isn't rising too quickly, you can also do phototherapy at home with a fiberoptic blanket that contains tiny bright lights. For most babies, it takes about a week or two for jaundice to go away. Very high levels of bilirubin, however, can damage a baby's brain. The good news is that this condition, called kernicterus, is almost always diagnosed long before bilirubin levels become high enough to cause damage, and phototherapy treatment will usually make it go away.

    • Kernicterus

      Kernicterus - illustration

      Kernicterus is a condition where very high bilirubin levels in the blood are deposited in the brain tissue causing irreversible damage to the brain. In the newborn, early diagnosis and treatment of jaundice or conditions that lead to jaundice may help prevent kernicterus.

      Kernicterus

      illustration

    Review Date: 5/17/2019

    Reviewed By: Neil K. Kaneshiro, MD, MHA, Clinical Professor of Pediatrics, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

    The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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