title: Maternal mortality among women with sickle-cell disease in France, 1996-2009.
abstract: OBJECTIVE
  To describe maternal mortality among women with sickle-cell disease in France.
  
  
  METHODS
  Data from the national confidential enquiry into maternal deaths and from reference centres for sickle-cell disease were examined to identify women with this disease who died in France during 1996-2009. The maternal mortality ratio among women with sickle-cell disease was estimated and compared with the ratio in the general population. Characteristics of these women and their pregnancies and circumstances of their deaths were examined in detail.
  
  
  RESULTS
  Fifteen maternal deaths occurred among an estimated 3300 live births to women with sickle-cell disease, for a maternal mortality ratio of 454 per 100000 live births (95% CI [254; 750]), versus 9.4/100000 in the general population. Ten women were homozygous (SS) for sickle-cell disease, and five were composite heterozygotes. The episode leading to death appeared in the antepartum period for seven women (47%). Two women died of septic shock during pregnancy, one at 6 weeks, the other at 24 weeks. The other 13 women (87%) died postpartum. Thirteen deaths were directly attributable to sickle-cell disease. The other two maternal deaths, both considered direct obstetric causes, were due to amniotic fluid embolism and septic shock after post-amniocentesis chorioamnionitis. The expert committee on maternal mortality judged seven of these 15 deaths (47%) to be avoidable.
  
  
  CONCLUSIONS
  Sickle-cell disease is responsible for a major excess risk of maternal death in France, due mainly to direct complications of the disease.
authors.affiliation: Service de Gynécologie-Obstétrique, Hôpital Louis Mourier 178, rue des Renouillers, 92700 Colombes, Hôpitaux Universitaires Paris Nord Val de Seine, Assistance Publique Hôpitaux de Paris, Université Paris Diderot, Paris, France. Electronic address: ninonlesage@gmail.com.
authors.forename: N
authors.initials: N
authors.lastname: Lesage
authors.affiliation: INSERM, Obstetrical, Perinatal and Pediatric Epidemiology Research Team, Centre for Epidemiology and Biostatistics (U1153), DHU Risks in Pregnancy, Université Paris Descartes, Paris, France.
authors.forename: C
authors.initials: C
authors.lastname: Deneux Tharaux
authors.affiliation: INSERM, Obstetrical, Perinatal and Pediatric Epidemiology Research Team, Centre for Epidemiology and Biostatistics (U1153), DHU Risks in Pregnancy, Université Paris Descartes, Paris, France.
authors.forename: M
authors.initials: M
authors.lastname: Saucedo
authors.affiliation: Unité des Maladies Génétiques du Globule Rouge, Hôpital Henri-Mondor AP-HP, Université Paris Est, Créteil, Paris, France.
authors.forename: A
authors.initials: A
authors.lastname: Habibi
authors.affiliation: Unité des Maladies Génétiques du Globule Rouge, Hôpital Henri-Mondor AP-HP, Université Paris Est, Créteil, Paris, France.
authors.forename: F
authors.initials: F
authors.lastname: Galacteros
authors.affiliation: Centre de la drépanocytose, Hôpital Tenon, 4, rue de la Chine, 75020 Paris, France.
authors.forename: R
authors.initials: R
authors.lastname: Girot
authors.affiliation: INSERM, Obstetrical, Perinatal and Pediatric Epidemiology Research Team, Centre for Epidemiology and Biostatistics (U1153), DHU Risks in Pregnancy, Université Paris Descartes, Paris, France.
authors.forename: M H
authors.initials: MH
authors.lastname: Bouvier Colle
authors.affiliation: Service de Gynécologie-Obstétrique, Hôpital Louis Mourier 178, rue des Renouillers, 92700 Colombes, Hôpitaux Universitaires Paris Nord Val de Seine, Assistance Publique Hôpitaux de Paris, Université Paris Diderot, Paris, France; INSERM, Obstetrical, Perinatal and Pediatric Epidemiology Research Team, Centre for Epidemiology and Biostatistics (U1153), DHU Risks in Pregnancy, Université Paris Descartes, Paris, France.
authors.forename: G
authors.initials: G
authors.lastname: Kayem
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000235:genetics / Q000401:mortality*; D002423:Cause of Death; D005260:Female; D005602:France / Q000453:epidemiology; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D007231:Infant, Newborn; D050498:Live Birth / Q000453:epidemiology; D008428:Maternal Mortality; D019300:Medical Errors / Q000401:mortality*; D049590:Postpartum Period; D011247:Pregnancy; D011248:Pregnancy Complications / Q000401:mortality*; D011295:Prenatal Care; D050497:Stillbirth / Q000453:epidemiology; D055815:Young Adult
keywords: Confidential enquiry into maternal deaths; Maternal mortality; Sickle-cell disease