Bilirubin and Brain Injury

Surveillance of extreme hyperbilirubinaemia in Denmark. A method to identify the newborn infants

“…In the early phase of acute bilirubin encephalopathy, the jaundiced neonates become lethargic, hypotonic and suck poorly. This phase is reversible with relevant treatment (1–3). The intermediate phase is characterized by moderate stupor, irritability and a tendency for tone to increase.…”

“…Most newborn infants develop jaundice during their first days of life, and the lower the gestational age, the more frequent it is. The course is normally benign when relevant treatment is offered, but occasionally, the infants develop extreme hyperbilirubinaemia, that is, a serum concentration of unconjugated bilirubin above the limits of an exchange transfusion (1,2). In rare cases, the devastating condition kernicterus is seen (2–5).…”

“…The course is normally benign when relevant treatment is offered, but occasionally, the infants develop extreme hyperbilirubinaemia, that is, a serum concentration of unconjugated bilirubin above the limits of an exchange transfusion (1,2). In rare cases, the devastating condition kernicterus is seen (2–5). Only a few population‐based studies describe the incidence of extreme hyperbilirubinaemia and kernicterus (4,6–10).…”

Relation between serum bilirubin levels ≥450 μmol/L and bilirubin encephalopathy; a Danish population‐based study

“…In very rare cases it progresses to acute intermediate bilirubin encephalopathy characterized by stupor, irritability, jittering, hypertonia alternating with hypotonia, opistotonus, retrocollis and a high‐pitched cry. This condition is likely to progress to acute advanced bilirubin encephalopathy characterized by coma, pronounced opistotonus and retrocollis, pronation spasms of the upper extremities, setting sun sign, fever, apnoea, seizures, unability to feed and even respiratory failure and death (1). The infants progressing to the advanced phase most frequently suffer permanent brain damage, which is the chronic form of bilirubin encephalopathy (kernicterus) manifesting in form of hearing loss, dystonic or atetoid cerebral palsy, development delay, and gaze disturbance (1).…”

“…This condition is likely to progress to acute advanced bilirubin encephalopathy characterized by coma, pronounced opistotonus and retrocollis, pronation spasms of the upper extremities, setting sun sign, fever, apnoea, seizures, unability to feed and even respiratory failure and death (1). The infants progressing to the advanced phase most frequently suffer permanent brain damage, which is the chronic form of bilirubin encephalopathy (kernicterus) manifesting in form of hearing loss, dystonic or atetoid cerebral palsy, development delay, and gaze disturbance (1). Whether infants with acute intermediate bilirubin encephalopathy develop chronic bilirubin encephalopathy is not known.…”