Kernicterus

What is Kernicterus?

Kernicterus is a rare but serious disease that causes brain damage in infants due to increased levels of bilirubin within their blood system. This disease typically results from jaundice that has gone untreated. Jaundice is a yellowing of the skin also caused by increased levels of bilirubin. Kernicterus will only develop if bilirubin toxicity is not remedied. Bilirubin is a result of the breakdown of red blood cells and will normally travel to the liver where it is removed naturally. Jaundice occurs in nearly 70 percent of term newborns and is, for the most part, easy to treat and not considered a serious condition.

Kernicterus is a condition that only applies to patients with either autopsy-proven kernicterus or those with permanent bilirubin-induced brain injury. Patients with transient or reversible clinical evidence of toxicity during the neonatal period are referred to as having “early bilirubin toxicity.”      

Risks

While jaundice is usually a harmless condition if it is ignored it can develop into kernicterus, sometimes causing irreversible damage. When certain levels of bilirubin penetrate the brain’s gray matter the effects can range from unapparent to severe brain damage and even death. Symptoms of kernicterus in infants include:

• Lethargy
• Distinct high-pitched cries and abnormal tones
• Arching of the back

There is also a risk of misdiagnosis associated with kernicterus as it mimics autism-spectrum disorders which can result in mistreatment or no treatment at all. 
Some studies have noted a challenge among the medical community to establish a national set of guidelines for the management of patients with hyperbilirubinemia (a strong indication of kernicterus). Guidelines such as these may be able to assist doctors with the resurgence of kernicterus that has occurred over the past decade according to an article in Pediatrics.   

Treatment

It was once commonplace for physicians to focus on a patient’s total serum bilirubin concentration, or TSB, as a means of determining the presence of kernicterus. However, in recent years studies have shown that relying solely on these levels as an indicator is inconclusive. Research has shown TSB to be a poor risk indicator and should not be cited as a physician’s reasoning for not intervening when otherwise necessary. Instead, it is suggested that free bilirubin concentration be considered over TSB as a critical serum factor involved in brain uptake of bilirubin and subsequent neurotoxicity. 

Kernicterus is a completely preventable disease and is usually treated in the same respects as jaundice through phototherapy or a Biliblanket which help to breakdown and remove bilirubin from the infant’s system. In more severe cases, physicians will perform exchange transfusions which involve blood or plasma transfusions, replacing the contaminated blood with a donor’s.

Sources:
 
Zhu, Jiajun, et al. "Total Serum Bilirubin Levels during the First Two Days of Life and Subsequent Neonatal Morbidity in Very Low Birth Weight Infants: a Retrospective Review." European Journal of Pediatrics 171.4 (2012): 669-674. MEDLINE with Full Text. Web. 25 May 2012.
 
Ahlfors, Charles E, et al. "Toward Understanding Kernicterus: A Challenge to Improve the Management of Jaundiced newborns." Pediatrics 117.2 (2006): 474. MEDLINE with Full Text. Web. 25 May 2012.
 

 

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