European Journal of Pediatrics

, Volume 168, Issue 4, pp 427–429

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

  • Simon Hannam
  • Paul Moriaty
  • Helen O’Reilly
  • John S. Craig
  • Michael A. Heneghan
  • Alastair Baker
  • Anil Dhawan
Original Paper

DOI: 10.1007/s00431-008-0763-5

Cite this article as:
Hannam, S., Moriaty, P., O’Reilly, H. et al. Eur J Pediatr (2009) 168: 427. doi:10.1007/s00431-008-0763-5

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.

Keywords

Jaundice Kernicterus Bilirubin Unconjugated Neurodevelopment 

Abbreviations

CN-1

Crigler-Najjar Type 1

UDPGT

Uridinediphosphate glucuronosyltransferase

PCV

Packed cell volume

AABR

Automated auditory brainstem response

MRI

Magnetic resonance image

Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  • Simon Hannam
    • 1
  • Paul Moriaty
    • 2
  • Helen O’Reilly
    • 1
  • John S. Craig
    • 2
  • Michael A. Heneghan
    • 3
  • Alastair Baker
    • 1
  • Anil Dhawan
    • 1
  1. 1.Department of Child HealthKings College HospitalLondonUK
  2. 2.Regional Neonatal UnitRoyal Maternity HospitalBelfastUK
  3. 3.Institute of Liver StudiesKings College HospitalLondonUK

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