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Chapter: Paediatrics: Neonatology

Paediatrics: Bilirubin encephalopathy (kernicterus)

A clinical syndrome resulting from the development of excessive neuro-toxic unconjugated bilirubin levels.

Bilirubin encephalopathy (kernicterus)

 

A clinical syndrome resulting from the development of excessive neuro-toxic unconjugated bilirubin levels. Toxic levels lead to selective damage of the cerebellum, basal ganglia, and brainstem auditory pathways. It may occur in the healthy neonate if serum bilirubin is >360µmol/L , but usually only occurs at significantly higher serum levels (>430µmol/L after 48hr of life) unless:

Infant is <24hr old.

 

Infant is preterm.

 

Infant is severely ill (any cause).

 

Infant is acidotic.

 

Caused by iso-immunization haemolytic disease.

 

Reduced albumin binding caused by drugs or hypoalbuminanaemia.

 

Presentation

 

Lethargy progressing to hypertonia then hypotonia.

 

Poor feeding.

 

Fever.

 

High-pitched cry.

 

Opisthotonos.

 

Seizures and coma.

 

Main differential diagnosis is meningoencephalitis/sepsis. Neonatal tetany may also present with opisthotonos.

 

Treatment

 

Supportive (likely to require full intensive care).

 

Urgent reduction of serum bilirubin by intensive phototherapy and exchange transfusion.

 

Give IV immunoglobulin.

 

Treat underlying cause.

 

Prognosis

 

Majority survives, but there is a high risk of athetoid cerebral palsy, deaf-ness, and low IQ.

 

 

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Paediatrics: Neonatology


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