Neonatal Jaundice and Kernicterus

* Abbreviations: AAP = : American Academy of Pediatrics • G-6-PD = : glucose-6-phosphate dehydrogenase The American Academy of Pediatrics (AAP) Subcommittee on Hyperbilirubinemia is currently revising the practice parameter (guidelines) on neonatal hyperbilirubinemia published in October 1994.1 Although this revision is in progress, the Subcommittee wishes to bring the issue of kernicterus to the attention of the pediatric community and provide additional information pending a more formal assessment of the literature and an analysis of the risks and benefits of new approaches to the jaundiced infant. The Joint Commission on Accreditation of Healthcare Organizations has already issued an alert on this subject.2 Kernicterus, or bilirubin encephalopathy, is a condition caused by bilirubin toxicity to the basal ganglia and various brainstem nuclei. In the acute phase, severely jaundiced infants become lethargic, hypotonic and suck poorly. If the hyperbilirubinemia is not treated, the infant becomes hypertonic and may develop a fever and a high-pitched cry. The hypertonia is manifested by backward arching of the neck (retrocollis) and trunk (opisthotonus). Surviving infants usually develop a severe form of athetoid cerebral palsy, hearing loss, dental dysplasia, paralysis of upward gaze and, less often, intellectual and other handicaps. Kernicterus is a condition that is unfamiliar to most pediatricians practicing today. In the 1940s and 1950s, kernicterus was a common complication of hyperbilirubinemia associated with Rh erythroblastosis fetalis and, occasionally, ABO hemolytic disease. With the introduction of exchange transfusion, kernicterus became much less common. The use of Rh immunoglobulin all but eliminated erythroblastosis fetalis and phototherapy drastically reduced the need for exchange transfusion. In the last several years, however, there have been reports of kernicterus associated with extremely high serum bilirubin levels.3–9Most of these infants did not have obvious hemolytic disease or another recognized cause of neonatal jaundice. Many appeared to be otherwise healthy, breastfeeding newborns although frequently they were not receiving adequate nutrition and hydration. …