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Acute bilirubin encephalopathy and its progression to kernicterus: current perspectives

Authors Usman F, Diala UM, Shapiro SM, Le Pichon JB, Slusher TM

Received 4 October 2017

Accepted for publication 20 December 2017

Published 7 March 2018 Volume 2018:8 Pages 33—44

DOI https://doi.org/10.2147/RRN.S125758

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Colin Mak

Peer reviewer comments 2

Editor who approved publication: Dr Robert Schelonka


Fatima Usman,1,2,* Udochukwu Michael Diala,3,4,* Steven M Shapiro,5–7 Jean Baptiste Le Pichon,5–7 Tina M Slusher8,9

1Department of Pediatrics, Bayero University Kano, Kano, Nigeria; 2Department of Pediatrics, Aminu Kano Teaching Hospital, Kano, Nigeria; 3Department of Pediatrics, University of Jos, Jos, Plateau State, Nigeria; 4Department of Pediatrics, Jos University Teaching Hospital, Jos, Plateau State, Nigeria; 5Department of Paediatrics, Children’s Mercy Hospital, Kansas City, MO, USA; 6Department of Paediatrics, University of Missouri-Kansas City, Kansas City, MO, USA; 7Department of Paediatrics, University of Kansas, Kansas City, MO, USA; 8Department of Paediatrics, Division of Global Healthy, University of Minnesota, Minneapolis, MN, USA; 9Pediatric Intensive Care Faculty, Hennepin County Medical Center, University of Minnesota, Minneapolis, MN, USA

*These authors contributed equally to this work


Abstract: Acute bilirubin encephalopathy (ABE) remains a significant cause of morbidity and mortality throughout the world, especially in low-middle-income countries where it can account for up to 15% of neonatal death. The pathophysiology of this acute life-threatening event of infancy and its potential evolution to kernicterus remain poorly understood. In this review, we start by reviewing the terminology of hyperbilirubinemia and its clinical consequences, ABE and later kernicterus spectrum disorder (KSD). We then review the pathogenesis of ABE and discuss clinical factors that can contribute to its pathogenicity. We examine in detail the clinical correlates of ABE and KSD. We present a comprehensive approach to its diagnosis and conclude with a set of simple clinical interventions ranging between primary preventive and rehabilitative measures that may help reduce the incidence of this largely preventable disease.

Keywords: acute bilirubin encephalopathy, neonatal jaundice, kernicterus, kernicterus spectrum disorder, low-middle-income countries

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