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Archives de pédiatrie
Volume 24, n° 8
pages 777-782 (août 2017)
Doi : 10.1016/j.arcped.2017.05.012
Received : 14 December 2016 ;  accepted : 29 May 2017
Stéatose hépatique aiguë gravidique et bêta-oxydation mitochondriale des acides gras : conséquences pour l’enfant
Acute fatty liver of pregnancy and mitochondrial fatty acid oxidation. Consequences for the offspring
 

B. Anon a, , C. Barbet b, C. Gendrot c, F. Labarthe b, Y. Bacq a
a Service d’hépato-gastroentérologie, hôpital Trousseau, CHRU de Tours, 37044 Tours cedex, France 
b Service de pédiatrie, hôpital Clocheville, CHRU de Tours, 37044 Tours cedex, France 
c Laboratoire de biochimie et biologie moléculaire, hôpital Bretonneau, CHRU de Tours, 37044 Tours cedex, France 

Auteur correspondant.
Résumé

La stéatose hépatique aiguë gravidique est une maladie rare spécifique de la grossesse pouvant entraîner une insuffisance hépatocellulaire et le décès de la mère et de l’enfant. Le pronostic maternel et fœtal a été transformé grâce à l’extraction fœtale précoce. Le diagnostic doit être suspecté au cours du troisième trimestre devant l’apparition de douleurs épigastriques, de vomissements ou d’une polyurie-polydipsie associés à une élévation des transaminases. La maladie peut être associée à un déficit au niveau de la bêta-oxydation mitochondriale des acides gras qui doit être recherché dès la naissance.

The full text of this article is available in PDF format.
Summary

Acute fatty liver of pregnancy (AFLP) is a rare liver disease unique to pregnancy that can lead to acute liver failure. The prognosis, initially often fatal for both mother and child, has been improved by prompt delivery. The diagnosis should be highly suspected if the mother presents epigastric pain, nausea and/or vomiting, or polyuria-polydipsia in the third trimester of pregnancy. AFLP has been found associated with a genetic deficiency of fatty acid beta-oxidation, which may cause sudden death in infancy. Consequently, the mother and her newborn should undergo screening for this deficiency.

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