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Prospective cohort study of neurodevelopmental outcomes following extreme neonatal hyperbilirubinaemia in Australia
Angela J McGillivray1,2, Jan Polverino1, Nadia Badawi2,3
1Department of Newborn Care, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.
Insights
Extreme neonatal hyperbilirubinaemia in Australia has an estimated kernicterus incidence of 0.35 per 100,000 live births. Three infants experienced neurodevelopmental impairments, including cerebral palsy and autism spectrum disorder.
Area of Science:
- Neonatal care
- Neurodevelopmental pediatrics
- Public health
Background:
- Extreme neonatal hyperbilirubinaemia poses a risk for neurodevelopmental impairments.
- Kernicterus, a severe form of bilirubin encephalopathy, has significant long-term consequences.
Purpose of the Study:
- To determine the incidence and nature of neurodevelopmental outcomes in Australian infants with extreme neonatal hyperbilirubinaemia.
- To assess the prevalence of kernicterus in a contemporary Australian cohort.
Main Methods:
- Prospective cohort study of infants born 2010-2013 at ≥34 weeks gestation with total serum bilirubin ≥450 μmol/L or signs of acute bilirubin encephalopathy.
- Neurodevelopmental outcomes assessed up to 3 years using neurological examination, Bayley Scales of Infant and Toddler Development (3rd ed.), and Ages and Stages Questionnaire (3rd ed.).
Main Results:
- The estimated Australian incidence of kernicterus is 0.35 per 100,000 live births.
- Of 26 infants followed, three exhibited neurodevelopmental impairment: cerebral palsy (GMFCS levels 1 and 4), visual impairment, audiological deficiency, and autism spectrum disorder.
- Mean Bayley-III scores indicated generally average to slightly below average performance across cognitive, communication, and motor domains.
Conclusions:
- Australia's kernicterus rate is favorable compared to global estimates.
- Universal neonatal hyperbilirubinaemia assessment is recommended.
- Mandated adverse outcome reporting and investigation are crucial for prevention.
Aim:
This study aimed to establish the incidence and nature of neurodevelopmental outcomes following extreme neonatal hyperbilirubinaemia in an Australian cohort.
Methods:
A prospective cohort study of neurodevelopmental outcomes up to 3 years of age of infants born between 2010 and 2013 at ≥34 weeks gestation, with total serum bilirubin ≥450 μmol/L and/or clinical signs of acute bilirubin encephalopathy. Outcome measures comprised neurological examination, Bayley Scales of Infant and Toddler Development, 3rd edition and Ages and Stages Questionnaire, 3rd edition.
Results:
The Australian estimated incidence of kernicterus is 0.35 per 100 000 live births. Within the follow-up cohort of 26, three children have clinical neurodevelopmental impairment: one has gross motor function classification system level 4 cerebral palsy, audiological deficiency and visual impairment; the second has gross motor function classification system level 1 cerebral palsy and the third has global developmental delay with autism spectrum disorder. Mean Bayley Scales of Infant and Toddler Development, 3rd edition scores were: cognition 10.3 (SD 1.5), receptive communication 9.4 (SD 1.8), expressive communication 9.2 (SD 2.4), fine motor 10.4 (SD 2.6) and gross motor 9.2 (SD 2.3).
Conclusion:
The Australian national rate of kernicterus compares favourably with global estimates. Future preventative strategies in this context include universal neonatal hyperbilirubinaemia assessment and mandated adverse outcome reporting and investigation.

