Urgent Clinical Need for Accurate and Precise Bilirubin Measurements in the United States to Prevent Kernicterus

Kernicterus, a preventable brain injury resulting from severe neonatal jaundice, has reemerged in the US (1-3 ). Newborn jaundice, a usually benign condition that typi- cally resolves with supervision and appropriate nutri- tional intake, can progress to severe hyperbilirubinemia in 8 -10% of healthy newborn infants. Severe hyperbiliru- binemia may need treatment with phototherapy. Some newborns discharged as healthy have developed severe hyperbilirubinemia after discharge and succumbed to serious and often irreversible posticteric sequelae. Kernicterus, which was first described during the au- topsy of a dead infant, refers to the icteric (yellow) staining of the basal ganglia, specifically the globus pal- lidus. The voluntary Pilot Kernicterus Registry now rec- ognizes a syndrome of bilirubin-induced neurologic dys- function (BIND), which includes kernicterus and a wide spectrum of disorders caused by excessive hyperbiliru- binemia. Using the Registry eligibility criteria, Johnson et al. (1 ) have documented the reemergence of kernicterus and outlined a series of lapses in care that are amenable to system-based correction. The common insult in all cases of BIND results from a total serum bilirubin (TSB) concentration that exceeds the infant's neuroprotective defenses and causes neuronal injury leading to impaired oculomotor function (primarily through damage to the basal ganglia, central and periph- eral auditory pathways, hippocampus, diencephalon, subthalamic nuclei, midbrain, cerebellum, and pontine and brain-stem nuclei) and impaired respiratory function (through damage to neurohumoral and electrolyte control and the cerebellum). The manifestations of acute bilirubin encephalopathy and chronic kernicteric sequelae may be minimal to severe and occur as various combinations (or possibly, isolated findings) of extrapyramidal disorders, neuromotor abnormalities, sensorineural hearing loss, and visual disability. Although not yet demonstrated, some experts believe that milder and subtler neurologic manifestations of BIND exist. The current reemergence of kernicterus in babies dis-