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01-04-2009 | Original Paper

Normal neurological outcome in two infants treated with exchange transfusions born to mothers with Crigler-Najjar Type 1 disorder

Authors: Simon Hannam, Paul Moriaty, Helen O’Reilly, John S. Craig, Michael A. Heneghan, Alastair Baker, Anil Dhawan

Published in: European Journal of Pediatrics | Issue 4/2009

Abstract

Patients with Crigler-Najjar Type 1 (CN-1) disorder have an unconjugated hyperbilirubinaemia due to the complete absence in activity of uridinediphosphate glucuronosyltransferase, a bilirubin-conjugating enzyme. In pregnant women with CN-1, the foetus is at high risk of being adversely affected by the bilirubin, as unconjugated bilirubin can cross the placenta and is potentially neurotoxic. We report the long-term outcomes of two infants born to women with CN-1. These infants had exchange transfusions soon after birth and have normal neurodevelopmental outcomes at 18 months and four years of age, respectively. We propose that this intervention might have improved the neurological outcome of these infants.

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Title: Normal neurological outcome in two infants treated with exchange transfusions born to mothers with Crigler-Najjar Type 1 disorder
Authors: Simon Hannam
Paul Moriaty
Helen O’Reilly
John S. Craig
Michael A. Heneghan
Alastair Baker
Anil Dhawan
Publication date: 01-04-2009
Publisher: Springer-Verlag
Published in: European Journal of Pediatrics / Issue 4/2009
Print ISSN: 0340-6199
Electronic ISSN: 1432-1076
DOI: https://doi.org/10.1007/s00431-008-0763-5

At a glance: The STEP trials

Springer Medicine

Normal neurological outcome in two infants treated with exchange transfusions born to mothers with Crigler-Najjar Type 1 disorder

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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