Literature DB >> 16735790

Kernicterus associated with hereditary spherocytosis and UGT1A1 promoter polymorphism.

Alberto Berardi1, Licia Lugli, Fabrizio Ferrari, Giancarlo Gargano, Maria D'Apolito, Agnese Marrone, Achille Iolascon.   

Abstract

INTRODUCTION: An apparent re-emergence of kernicterus has been recently reported, with some cases occurring in otherwise healthy breastfed newborn.
METHODS: We describe a case of kernicterus in a term Caucasian newborn.
RESULTS: An exceptional polymorphism of UGT1A1 gene promoter co-existed with asymptomatic inherited spherocytosis, due to erythroid anion exchange (band-3) deficiency. Both concurred to the development of severe neonatal hyperbilirubinaemia.
CONCLUSION: As some cases of kernikterus remain unresolved, haemolytic diseases and bilirubin metabolism disorders should be carefully investigated in unexplained severe neonatal hyperbilirubinaemia. Copyright (c) 2006 S. Karger AG, Basel.

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Year:  2006        PMID: 16735790     DOI: 10.1159/000093668

Source DB:  PubMed          Journal:  Biol Neonate        ISSN: 0006-3126


  4 in total

1.  Causes of hemolysis in neonates with extreme hyperbilirubinemia.

Authors:  R D Christensen; R H Nussenzveig; H M Yaish; E Henry; L D Eggert; A M Agarwal
Journal:  J Perinatol       Date:  2014-04-24       Impact factor: 2.521

Review 2.  A pediatrician's practical guide to diagnosing and treating hereditary spherocytosis in neonates.

Authors:  Robert D Christensen; Hassan M Yaish; Patrick G Gallagher
Journal:  Pediatrics       Date:  2015-06       Impact factor: 7.124

3.  Evaluating eosin-5-maleimide binding as a diagnostic test for hereditary spherocytosis in newborn infants.

Authors:  R D Christensen; A M Agarwal; R H Nussenzveig; N Heikal; M A Liew; H M Yaish
Journal:  J Perinatol       Date:  2014-11-06       Impact factor: 2.521

Review 4.  Old and new insights into the diagnosis of hereditary spherocytosis.

Authors:  Olga Ciepiela
Journal:  Ann Transl Med       Date:  2018-09
  4 in total

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