Kernicterus (patient information)

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Synonyms and Keywords: Bilirubin encephalopathy

Overview
Kernicterus is a rare neurological condition that occurs in some newborns with severe jaundice.

What are the symptoms of Kernicterus?
The symptoms depend on the stage of kernicterus.


 * Early stage:
 * Extreme jaundice
 * Absent startle reflex
 * Poor feeding or sucking
 * Extreme sleepiness (lethargy)


 * Mid stage:
 * High-pitched cry
 * Arched back with neck hyperextended backwards
 * Bulging fontanel (soft spot)
 * Seizures


 * Late stage (full neurological syndrome):
 * High-frequency hearing loss
 * Mental retardation
 * Muscle rigidity
 * Speech difficulties
 * Seizures
 * Movement disorder

What causes Kernicterus?

 * Kernicterus is caused by very high levels of bilirubin. Bilirubin is a yellow pigment that is created in the body during the normal recycling of old red blood cells. High levels of bilirubin in the body can cause the skin to look yellow (which is called jaundice).


 * In some cases when there are extremely high levels of bilirubin in the body or the baby is extremely ill, the substance will move out of the blood and collect in the brain tissue. This can lead to serious neurological complications, including brain damage and hearing loss.

Rh hemolytic disease]] that may lead to hydrops fetalis are at high risk for severe jaundice that leads to this condition. However, kernicterus has been seen in apparently healthy babies.
 * Kernicterus usually develops in the first week of life, but may be seen up until the third week. Newborns with [[Hemolytic disease of the newborn (patient information)

When to seek urgent medical care?
Seek immediate medical help if your baby has signs of this condition.

Diagnosis
A blood test will show a high bilirubin level (greater than 20-25 mg/dL).

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

Treatment options

 * 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

Where to find medical care for Kernicterus?
Directions to Hospitals Treating Kernicterus

What to expect (Outlook/Prognosis)?
Kernicterus is a serious condition. The outcome is guarded. Many infants with late stage nervous system complications die.

Possible complications

 * Permanent brain damage
 * Hearing loss
 * Death

Prevention

 * Early diagnosis and treatment of jaundice or conditions that lead to jaundice may help prevent this complication.


 * The American Academy of Pediatrics recommends that infants with the first signs of jaundice have their 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).


 * The association also recommends that all newborns have a follow-up appointment within 2 to 3 days after leaving the hospital. This is particularly important for premature or near-term babies.

Source
http://www.nlm.nih.gov/medlineplus/ency/article/007309.htm