Neonatal hemochromatosis: Management, outcome, and prevention

Enrico Lopriore*, M. Luisa Mearin, Dick Oepkes, Roland Devlieger, Peter F. Whitington

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

50 Scopus citations

Abstract

Neonatal hemochromatosis (NH) is a rare disorder but the most common cause of acute liver failure in neonates. NH is characterized by severe hepatic injury and iron overload and is associated with high perinatal mortality and morbidity rates. NH is often preceded by oligohydramnios and intrauterine growth restriction, suggesting an important impact of NH during fetal life. Stillbirth and prematurity are not uncommon. During the last decade, major discoveries on the etiology of NH have radically changed the management and outcome of this disease. NH is now regarded as an alloimmune disease and is, as such, often referred to as gestational alloimmune liver disease. Antenatal treatment with intravenous immunoglobulins starting at 14weeks' gestation has been shown to prevent the development of NH in subsequent pregnancies. Postnatal treatment, previously based on the use of anti-oxidants and chelation therapy, has now successfully been replaced by exchange transfusions and intravenous immunoglobulins substitution. This review summarizes the latest discoveries on the etiology of NH and the new recommendations concerning its management and prevention.

Original languageEnglish (US)
Pages (from-to)1221-1225
Number of pages5
JournalPrenatal Diagnosis
Volume33
Issue number13
DOIs
StatePublished - Dec 2013

ASJC Scopus subject areas

  • Genetics(clinical)
  • Obstetrics and Gynecology

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