Review of bilirubin neurotoxicity I: molecular biology and neuropathology of disease.

Abstract:

:Despite the availability of successful prevention strategies to prevent excessive hyperbilirubinemia, the neurological sequelae of bilirubin neurotoxicity (BNTx) still occur throughout the world. Kernicterus, encephalopathy due to BNTx, is now understood to be a spectrum of severity and phenotypes known as kernicterus spectrum disorder (KSD). A better understanding of the selective neuropathology and molecular biology of BNTx and using consistent clinical definitions of KSDs as outcome measure can lead to more accurately predicting the risk and causes of BNTx and KSDs. In Part I of our two-part review, we will summarize current and recent advances in the understanding of the selective neuropathology and molecular biology of the disease. Herein we emphasize the role of unbound, free unconjugated bilirubin as well as genetic contributions to the susceptibility BNTx and the development of KSDs. In Part II, we focus on current and possible novel methods to prevent BNTx and ABE and treat ABE and KSDs.

journal_name

Pediatr Res

journal_title

Pediatric research

authors

Riordan SM,Shapiro SM

doi

10.1038/s41390-019-0608-0

subject

Has Abstract

pub_date

2020-01-01 00:00:00

pages

327-331

issue

2

eissn

0031-3998

issn

1530-0447

pii

10.1038/s41390-019-0608-0

journal_volume

87

pub_type

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