Health Library

Bilirubin encephalopathy
     
Print-Friendly
Bookmarks

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, Felker MV. Neurological complications of systemic disease: children. In: Jankovic J, Mazziotta JC, Pomeroy SL, Newman NJ, eds. Bradley and Daroff's Neurology in Clinical Practice. 8th ed. Philadelphia, PA: Elsevier; 2022:chap 59.

Hansen TWR. Pathophysiology of kernicterus. In: Polin RA, Abman SH, Rowitch, DH, Benitz WE, eds. Fetal and Neonatal Physiology. 6th ed. Philadelphia, PA: Elsevier; 2022:chap 163.

Kaplan M, Wong RJ, Burgis JC, Sibley E, Stevenson DK. Neonatal jaundice and liver disease. In: Martin RJ, Fanaroff AA, Walsh MC, eds. Fanaroff and Martin's Neonatal-Perinatal Medicine. 11th ed. Philadelphia, PA: Elsevier; 2020:chap 91.

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

BACK TO TOPText only

 
  • Newborn jaundice

    Animation

  • Kernicterus

    Kernicterus

    illustration

  • Newborn jaundice

    Animation

  • Kernicterus

    Kernicterus

    illustration

A Closer Look

 

    Talking to your MD

     

      Tests for Bilirubin encephalopathy

       
         

        Review Date: 5/24/2021

        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.
        adam.com