Kernicterus: Lessons for the Future From a Current Tragedy

Abstract

Kernicterus, a preventable injury to the brain due to severe neonatal jaundice, has re-emerged in the United States as a health and societal concern. In its usually recognized form, kernicterus causes devastating disabilities, including athetoid cerebral palsy and speech and hearing impairments. In response to disturbing reports of kernicterus in term and near-term infants discharged as “healthy” from their place of birth, A. K. Brown and colleagues inaugurated a Pilot Kernicterus Registry in May, 1992 at the 10th Annual Kernicterus Symposium. Drs. Lois Johnson and Audrey Brown maintained a confidential manual database of acute and chronic bilirubin encephalopathies for voluntary reporting of cases of kernicterus (considered by many to be an “underground” disease). Usually referring to the icteric (yellow) staining of the basal ganglia and lesions of the extrapyramidal nervous system, kernicterus is detected at autopsy. Now, the Pilot Kernicterus Registry has offered formal clinical definitions of bilirubin-induced neurologic dysfunction (BIND) and kernicterus. (1) Audrey K. Brown, the “grande dame of bilirubin,” helped us to understand bilirubin conjugation and to respect the dangers of excessive total serum bilirubin (TSB) load. She was a leader in the campaign to prevent kernicterus. Her historical review (see accompanying special article) chronicles the achievements of the researchers of kernicterus. Her research reminds us of the universality of neonatal bilirubinemia. Sadly, she also chronicled both the fall and rise of kernicterus. Dr. Brown and other “ancestral” researchers imparted a legacy to maintain vigilance for the perpetual and ominous specter of kernicterus that can threaten humanity now and for generations to come. The current re-emergence of kernicterus in babies discharged as healthy from United States hospitals represents a crisis of pediatrician credibility, a societal demand for patient safety, and a disease that needs to be controlled by public health policy. The actual incidence of kernicterus …