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 typically resolves with supervision and appropriate nutritional intake, can progress to severe hyperbilirubinemia in 8–10% of healthy newborn infants. Severe hyperbilirubinemia 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, as described in neonates, refers to the icteric (yellow) staining of the basal ganglia, specifically the globus pallidus (4 ). The voluntary Pilot Kernicterus Registry now recognizes a syndrome of bilirubin-induced neurologic dysfunction (BIND), which includes kernicterus in its most severe acute and chronic forms. Using the Registry eligibility criteria, Johnson et al. (1 ) have documented the reemergence of kernicterus in a population of term and near-term “healthy” infants after its near eradication following prevention of Rh sensitization and widespread availability of phototherapy. The common insult in all cases of BIND results from a total serum bilirubin (TSB) concentration that exceeds the infant’s neuroprotective defenses and leads to neuronal injury, primarily in the basal ganglia, central and peripheral auditory pathways, hippocampus, diencephalon, subthalamic nuclei, midbrain, cerebellum and pontine and brain-stem nuclei for oculomotor function and for respiratory, neurohumoral, and electrolyte control. 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 discharged as healthy from US hospitals represents a crisis of credibility and calls into question our ability to measure TSB with accuracy and precision. There is a need to address the societal demand for patient safety and to respond to calls for a public health policy to better manage a preventable injury. After reviewing lapses in care and their root causes, we provide here empiric evidence on which to model a practical, family-centered, system-based approach to monitor and manage hyperbilirubinemia to prevent acute kernicterus and the clinical spectrum of BIND. After a workshop on Strategies for a System-wide Approach in the Management of Hyperbilirubinemia to Prevent Kernicterus [held at Pennsylvania Hospital with the joint sponsorship of Parents of Infants and Children with Kernicterus (PICK) in February 2001], several organizations reviewed their roles, and suggestions for action were considered. Alerts and updates for prevention of severe hyperbilirubinemia and kernicterus were subsequently disseminated by The Joint Commission for Accreditation of Hospital Organizations (2 ), the CDC (3 ), and the American Academy of Pediatrics (5 ). The National Quality Forum (at the Agency for Healthcare Research for Quality) has since declared kernicterus and/or TSB concentrations 300 mg/L (30 mg/dL, or 513 mol/L) as a “never-event”. It is the only pediatric condition on this list (6 ).