Pregnancies in Women With Long‐Chain Fatty Acid Oxidation Disorders: Results of a European and North American Survey
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Le résumé fourni par la source
Long-chain fatty acid oxidation disorders (lcFAODs) are genetic disorders of energy metabolism that are associated with a risk of metabolic decompensation, especially during catabolic episodes. With improvement in diagnostics and treatment, more women with lcFAODs now reach child-bearing age. So far, little is known about the risk and outcome of pregnancies, particularly in women with more severe forms of lcFAODs. We performed an international web-based survey among health care professionals involved in the care of individuals with lcFAODs and collected data on 89 pregnancies in 39 women (mild VLCAD deficiency n = 8, severe VLCAD deficiency n = 10, LCHAD deficiency n = 4, CPT2 deficiency n = 14, CPT1 deficiency n = 3). There were 72 live births, 12 spontaneous miscarriages, and one stillbirth at 41 weeks of gestation. Four women were still pregnant at the time of the survey. In 25 women, the diagnosis was known before the first pregnancy, whereas 14 had at least one pregnancy before diagnosis. Most women remained metabolically stable during pregnancy, although 19% of women had at least one metabolic decompensation during pregnancy. Forty-one percent of babies were delivered by spontaneous vaginal delivery, 33% after induced labor, and 19% by an elective Caesarean section. Most deliveries were uncomplicated, with preventive i.v. glucose infusions given in 50%. However, 21% of mothers developed a metabolic decompensation in the postpartum period. No maternal deaths were reported. In conclusion, our data show that the outcome of pregnancies in lcFAOD patients is generally favorable, despite a significant risk of metabolic decompensation during the postpartum period.
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Le contrôle bibliographique ouvert
DOI retrouvé dans Crossref DOI retrouvé ; titre concordant.
- Titre Crossref
- Pregnancies in Women With Long‐Chain Fatty Acid Oxidation Disorders: Results of a European and North American Survey
- Date Crossref
- 01/01/2026
- Éditeur
- Wiley
- Type
- journal-article
Ce recoupement confirme des métadonnées liées au DOI. Il ne confirme ni la méthode ni les conclusions de l’étude, et il ne compte pas comme une seconde source scientifique indépendante.
Où se fait cette recherche
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University Medical Center Freiburg pays non établi dans la noticeÉtablissement de santé
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Amsterdam University Medical Centers pays non établi dans la noticeÉtablissement de santé
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Amsterdam Gastroenterology Endocrinology Metabolism pays non établi dans la noticeÉtablissement de santé
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Copenhagen University Hospital Department of Clinical Genetics pays non établi dans la noticeÉtablissement de santé
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Rigshospitalet pays non établi dans la noticeÉtablissement de santé
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Hacettepe University pays non établi dans la noticeUniversité ou école supérieure
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Salford Royal NHS Foundation Trust pays non établi dans la noticeÉtablissement de santé
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Salford Royal Hospital Adult Inherited Metabolic Disorders pays non établi dans la noticeÉtablissement de santé
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Northern Care Alliance NHS Foundation Trust pays non établi dans la noticeÉtablissement de santé
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Istanbul University-Cerrahpaşa pays non établi dans la noticeUniversité ou école supérieure
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Ludwig-Maximilians-Universität München pays non établi dans la noticeUniversité ou école supérieure
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LMU Klinikum pays non établi dans la noticeÉtablissement de santé
University Medical Center Freiburg, Amsterdam University Medical Centers et Amsterdam Gastroenterology Endocrinology Metabolism, avec 9 autres affiliations.
Une affiliation ne permet pas de déduire la nationalité d’un auteur.