Predictive and diagnostic measures for kernicterus spectrum disorder: a prospective cohort study

Rose Gelineau-Morel, Fatima Usman, Saadatu Shehu, Hung Wen Yeh, Mohammad A. Suwaid, Mohammed Abdulsalam, Yasir Jibril, Katherine M. Satrom, Steven M. Shapiro, Timothy P. Zinkus, Hayden W. Head, Tina M. Slusher, Jean Baptiste Le Pichon, Zubaida L. Farouk

Research output: Contribution to journalArticlepeer-review


Background: Kernicterus spectrum disorder (KSD) resulting from neonatal hyperbilirubinemia remains a common cause of cerebral palsy worldwide. This 12-month prospective cohort study followed neonates with hyperbilirubinemia to determine which clinical measures best predict KSD. Methods: The study enrolled neonates ≥35 weeks gestation with total serum bilirubin (TSB) ≥ 20 mg/dl admitted to Aminu Kano Hospital, Nigeria. Clinical measures included brain MRI, TSB, modified bilirubin-induced neurologic dysfunction (BIND-M), Barry-Albright Dystonia scale (BAD), auditory brainstem response (ABR), and the modified KSD toolkit. MRI signal alteration of the globus pallidus was scored using the Hyperbilirubinemia Imaging Rating Tool (HIRT). Results: Of 25 neonates enrolled, 13/25 completed 12-month follow-up and six developed KSD. Neonatal BIND-M ≥ 3 was 100% sensitive and 83% specific for KSD. Neonatal ABR was 83% specific and sensitive for KSD. Neonatal HIRT score of 2 was 67% sensitive and 75% specific for KSD; this increased to 100% specificity and sensitivity at 12 months. BAD ≥ 2 was 100% specific for KSD at 3–12 months, with 50–100% sensitivity. Conclusions: Neonatal MRIs do not reliably predict KSD. BIND-M is an excellent screening tool for KSD, while the BAD or HIRT score at 3 or 12 months can confirm KSD, allowing for early diagnosis and intervention. Impact: The first prospective study of children with acute bilirubin encephalopathy evaluating brain MRI findings over the first year of life.Neonatal MRI is not a reliable predictor of kernicterus spectrum disorders (KSD).Brain MRI at 3 or 12 months can confirm KSD.The modified BIND scale obtained at admission for neonatal hyperbilirubinemia is a valuable screening tool to assess risk for developing KSD.The Barry Albright Dystonia scale and brain MRI can be used to establish a diagnosis of KSD in at-risk infants as early as 3 months.

Original languageEnglish (US)
JournalPediatric Research
StateAccepted/In press - 2023

Bibliographical note

Funding Information:
R.G.-M. is supported by the NICHD (grant number T32HD069038). She has received a travel grant from the American Society of Clinical Pharmacology and Therapeutics and has a provisional patent for a novel drug for the treatment of movement disorders. F.U. and S.S. declared internal funding from Children’s Mercy Hospital to support patient MRI costs for the study. T.S. declared internal funding for travel and hotel only from Children’s Mercy Hospital related to this study. J.-B.L.P. declared internal funding from Children’s Mercy Hospital to support the costs of this study. He also serves on the editorial board of Pediatric Neurology and Annals of Child Neurology Society and is a clinical advisory round table member for the Firefly Fund for Neimann Pick type C. J.-B.L.P. also provides medical expertize on an occasional basis to various law firms. All other authors declare no conflicts of interest.

Funding Information:
This work was funded by a Children’s Mercy Hospital internal grant to J.B.L.P. R.G.-M. is supported by the Eunice Kennedy Shriver National Institute of Child Health and Human Development (grant number T32HD069038). The funding sources had no role in study design, in the collection, analysis, and interpretation of data, in the writing of the report, or in the decision to submit the paper for publication.

Publisher Copyright:
© 2023, The Author(s), under exclusive licence to the International Pediatric Research Foundation, Inc.

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