title: Transient neonatal liver disease after maternal antenatal intravenous Ig infusions in gestational alloimmune liver disease associated with neonatal haemochromatosis.
abstract: OBJECTIVE
  Neonatal haemochromatosis is a rare gestational disease that results in severe foetal liver disease with extrahepatic iron overload, sparing the reticuloendothelial system. Recurrence can be prevented with intravenous immunoglobulin (IVIG) infusions during pregnancy, supporting an alloimmune aetiology. The aim of the study was to assess the effect of antenatal treatment with IVIG infusion on the outcome of pregnancies in women with a history of documented neonatal haemochromatosis likely owing to gestational alloimmune disease and to analyse IVIG tolerance.
  
  
  METHODS
  From 2004 to 2012, 8 pregnant women were treated with IVIG at 1 g/kg body weight weekly from 18 weeks' gestation until birth in a prospective multicentre study.
  
  
  RESULTS
  All 8 neonates born to the treated women survived. Five developed mild neonatal liver disease with hepatomegaly (n = 1), hyperechogenic liver (n = 2), abnormal liver function tests (n = 1), raised serum ferritin (n = 3) and α-fetoprotein (n = 5) levels, or mild iron overload on liver magnetic resonance imaging (n = 1). Ferritin and α-fetoprotein levels normalised before 14 days and 2 months, respectively. A per-mother-basis analysis comparing outcomes of treated (n = 8) and untreated (n = 9) gestations showed a significant improvement in the survival of neonates with gestational IVIG therapy (survival 8/8 vs 0/9, P < 0.001). Adverse effects of IVIG infusion occurred in 5 mothers leading to discontinuation of treatment in 1 case. Preterm neonates born before 37 weeks' gestation had a decreased risk of neonatal liver disease (P = 0.04).
  
  
  CONCLUSIONS
  Antenatal treatment with IVIG infusion in women at risk for gestational alloimmune disease recurrence improves the outcome of pregnancies despite mild signs of transient neonatal liver disease.
authors: *Pediatric Hepatology and Inborn Metabolic Diseases Unit, University Children's Hospital, Toulouse †Department of Pediatric Gastroenterology, Hepatology and Nutrition ‡Pathology Department, Hopital Femme Mère Enfant, Bron §Pathology Department, Institut Gustave Roussy ||Sorbonne Universités, UPMC Univ Paris 06, Paris ¶Internal Medicine Department #Special Care Baby Unit, Poissy-Saint Germain en Laye Hospital, Poissy **Clinical Genetics, Hopital Femme Mère Enfant, Bron ‡‡Pediatric Hepatology and Gastroenterology Unit §§Special Care Baby Unit, University Children's Hospital, Bordeaux ||Fetal Medicine, Saint Nicolas Hospital, Blaye ¶¶Neonatal Intensive Care Unit, Armand Trousseau Hospital, Paris ##Neonatal Intensive Care Unit, General Hospital, Saint-Brieuc ***Pediatric Surgery, Saint-Brieuc †††Clinical Genetics, University Hospital, Rennes, France ‡‡‡Metabolic Medicine Department, Great Ormond Street Hospital, London, UK §§§Department of Pediatric Surgery, Hepatology, and Transplantation, Necker Enfants Malades Hospital, Paris ||||||Pediatrics, University Hospital, Limoges, France.
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mesh_terms: D000328:Adult; D005260:Female; D005293:Ferritins / Q000097:blood; D006432:Hemochromatosis / Q000188:drug therapy* / Q000276:immunology; D006529:Hepatomegaly; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000009:adverse effects* / Q000627:therapeutic use*; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000097:blood / Q000209:etiology* / Q000473:pathology; D007262:Infusions, Intravenous; D007501:Iron / Q000097:blood; D008099:Liver / Q000473:pathology*; D008107:Liver Diseases / Q000097:blood / Q000209:etiology* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D011247:Pregnancy; D011295:Prenatal Care; D011446:Prospective Studies; D012306:Risk; D013534:Survival; D000509:alpha-Fetoproteins / Q000378:metabolism
chemical_list: D016756:Immunoglobulins, Intravenous; D000509:alpha-Fetoproteins; D005293:Ferritins; D007501:Iron