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Eur J Obstet Gynecol Reprod Biol. 2015 Nov;194:183-8. doi: 10.1016/j.ejogrb.2015.09.016. Epub 2015 Sep 25.

Maternal mortality among women with sickle-cell disease in France, 1996-2009.

Author information

1
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.
2
INSERM, Obstetrical, Perinatal and Pediatric Epidemiology Research Team, Centre for Epidemiology and Biostatistics (U1153), DHU Risks in Pregnancy, Université Paris Descartes, Paris, France.
3
Unité des Maladies Génétiques du Globule Rouge, Hôpital Henri-Mondor AP-HP, Université Paris Est, Créteil, Paris, France.
4
Centre de la drépanocytose, Hôpital Tenon, 4, rue de la Chine, 75020 Paris, France.
5
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.

Abstract

OBJECTIVE:

To describe maternal mortality among women with sickle-cell disease in France.

STUDY DESIGN:

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.

CONCLUSION:

Sickle-cell disease is responsible for a major excess risk of maternal death in France, due mainly to direct complications of the disease.

KEYWORDS:

Confidential enquiry into maternal deaths; Maternal mortality; Sickle-cell disease

PMID:
26431903
DOI:
10.1016/j.ejogrb.2015.09.016
[Indexed for MEDLINE]

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