Kernicterus

Information for Health Care Professionals

Kernicterus is a completely preventable debilitating developmental disability. Kernicterus is a neurologic syndrome that is caused by extreme and untreated hyperbilirubinemia during the neonatal period. Hyperbilirubinemia results from high levels of bilirubin, one of the breakdown products of the red blood cells. High levels of bilirubin are toxic to the developing newborn's central nervous system. In full term infants, acute symptoms of severe hyperbilirubinemia include extreme jaundice and neurologic abnormalities in level of consciousness, tone and movement, and brain stem function, particularly in relation to feeding and crying. The major features of kernicterus are cerebral palsy , sensorineural hearing loss , gaze paresis, and dental dysplasia. Mental retardation is also associated with some cases of kernicterus. Phototherapy and exchange transfusions effectively prevent kernicterus if treatment is timely. 

Risk factors for hyperbilirubinemia in the term newborn

Jaundice within the first 24 hours after birth
A sibling who was jaundiced as a neonate
Unrecognized hemolysis such as ABO blood type incompatibility or Rh incompatibility
Non-optimal sucking/nursing 
Deficiency in glucose-6-phosphate dehydrogenase (G6PD), a genetic disorder
Infection 
Cephalohematomas/bruising
East-Asian or Mediterranean descent

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Warning signs of kernicterus

The early physical signs of kernicterus include the following:

• Yellowing or orange skin tones (beginning at the head and spreading to the toes)

• Lethargy

• Poor sucking/nursing

• Changes in body tone, usually hypotonia (or later hypertonia)

• Opisthotonos

• High-pitched cry 

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This page last updated Thursday, August 05, 2004

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National Center on Birth Defects and Developmental Disabilities
 

The National Center on Birth Defects and Developmental Disabilities (NCBDDD) promotes the health of babies, children, and adults, and enhances the potential for full, productive living.  Our work includes identifying the causes of birth defects and developmental disabilities, helping children to develop and reach their full potential, and promoting health and well-being among people of all ages with disabilities.