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Kernicterus

Kernicterus, also known as bilirubin-induced neurologic dysfunction (BIND) and bilirubin encephalopathy (BE), is a neonatal disorder caused by levels of bilirubin greater than 25 mg/dl in the blood. It occurs in approximately 1.5 per 100,000 live births in the United States. The rate has decreased from 5.1 per 100,000 live births since the American Academy of Pediatricians published a guideline on hyperbilirubinemia, or high levels of bilirubin, in 1994.

Bilirubin is a blood-breakdown byproduct that is yellow in color. Newborn babies with hyperbilirubinemia will be jaundiced, meaning their skin will have a yellow pigmentation. Normal newborns will sometimes have a mild to moderate case of hyperbilirubinemia; however, it is more common in infants born with intracranial, subdural, or subgaleal hemorrhages.

If a child has hypoxic-ischemic encephalopathy-related multi-organ dysfunction, which decreases the body’s ability to effectively process blood breakdown byproducts, or if levels of bilirubin are too high, the infant’s liver will not be able to process the excess byproduct, so it will collect on the infant’s brain. High levels of bilirubin are toxic to this vital organ, and may cause brain damage.  Hyperbilirubinemia may also be caused by diseases that inhibit the breakdown of red blood cells, so infants with jaundice should also be screened for congenital disorders.

Risk factors for kernicterus include bruising, cephalohematoma, vacuum delivery, gestational age of less than 38 weeks, visible jaundice within 24 hours of birth, and a history of a sibling with infant jaundice.

There are three stages of kernicterus. The first stage will usually occur within 24 hours of birth and is characterized by extreme jaundice, the inability to latch on while breastfeeding, an upward gaze, extreme sleepiness or lethargy, the absence of normal infant reflexes, and low muscle tone, also known as hypotonia. In the second stage, an infant may exhibit a shrill or high-pitched cry, arch its back while hyperextending its neck, have a bulging soft spot, make bicycling motions or repetitive hand or arm movements, exhibit high muscle tone, also known as hypertonia, appear jittery, have a fixed gaze, and may experience seizures. The third stage, which may lead to life-long injury, is characterized by high-frequency hearing loss, developmental delays and intellectual disability, high muscle tone and rigidity, disorganized movement, abnormal muscle contractions, writhing, speech disorders, and seizures. Hearing loss is common since the nerves in the ear are very sensitive to injury from elevated bilirubin.

Treatments of kernicterus are the same as for hyperbilirubinemia. The first treatment is usually light therapy since exposure to light causes bilirubin to break down into smaller, non-harmful byproducts. Other treatments also include blood exchange transfusions for severe cases. Kernicterus is a serious condition and may result in acute brain damage, partial or complete hearing loss, and even death.

If your child suffers from kernicterus, please contact us immediately at 877-262-9767 to discuss your unique situation.

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