Screening for Severe Neonatal Hyperbilirubinemia

Vinod K. Bhutani

Disclosures

Pediatr Health. 2009;3(4):369-379. 

In This Article

Target Conditions

Kernicterus is predominantly a preventable brain injury in infants with severe neonatal jaundice that often manifests as irreversible posticteric sequelae. The hallmark sign, usually at autopsy, is the icteric (yellow) staining of the basal ganglia, specifically the globus pallidus. It can also now be evidenced as increased signals on MRI scans. It results from a TB level that exceeds the infant's neuroprotective defenses and results in neuronal injury, primarily in the basal ganglia, central and peripheral auditory pathways, hippocampus, diencephalon, subthalamic nuclei, midbrain, pontine and brain stem nuclei for oculomotor function, respiratory, neurohumoral and electrolyte control as well as the cerebellum.[17,18]

Acute bilirubin encephalopathy (ABE) includes progressive changes in the infant's mental (behavioral) status, muscle tone, cry, increasing hypertonia, especially of extensor muscles, retrocollis and opisthotonos, along with varying degrees of drowsiness, poor feeding and alternating tone (Box 1). Acute stage mortality (approximately 7%) is due to respiratory failure and progressive coma or intractable seizures. The rate of progression of clinical signs depends on the rate of bilirubin rise, duration of hyperbilirubinemia, host susceptibility and presence of comorbidities.[19,20]

Bilirubin-induced neurologic dysfunction (BIND) consists of a wider spectrum of disorders that includes kernicterus, ABE and isolated sensori-neural hearing loss. Although not yet proven, some experts believe that there may be milder and subtler neurological manifestations of BIND, with signs of awkwardness, incoordination, gait abnormalities, fine tremors and exaggerated extrapyramidal reflexes.[21] These subtle signs are difficult to diagnose owing to their delayed clinical expression and may be the only residual sequelae of ABE. Clinical predisposition to BIND includes prematurity, rapid rate of bilirubin rise (usually associated with hemolysis), asphyxia, sepsis, hypoalbuminemia, lethargy and temperature instability.[1]

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