{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T22:32:50Z","timestamp":1790375570394,"version":"4.1.0"},"reference-count":25,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2026,1,19]],"date-time":"2026-01-19T00:00:00Z","timestamp":1768780800000},"content-version":"vor","delay-in-days":18,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Long\u2010chain 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\u2010bearing 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\u2010based 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                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u20098, severe VLCAD deficiency\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200910, LCHAD deficiency\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u20094, CPT2 deficiency\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200914, CPT1 deficiency\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u20093). There were 72 live births, 12 spontaneous miscarriages, and one stillbirth at 41\u2009weeks 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\u2010one 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.\n                  <\/jats:p>","DOI":"10.1002\/jimd.70140","type":"journal-article","created":{"date-parts":[[2026,1,19]],"date-time":"2026-01-19T21:53:57Z","timestamp":1768859637000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Pregnancies in Women With Long\u2010Chain Fatty Acid Oxidation Disorders: Results of a European and North American Survey"],"prefix":"10.1002","volume":"49","author":[{"given":"Sarah C.","family":"Gr\u00fcnert","sequence":"first","affiliation":[{"name":"Children's Hospital, Department of Pediatrics, Adolescent Medicine and Neonatology, Faculty of Medicine Freiburg University Hospital  Freiburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9934-6831","authenticated-orcid":false,"given":"Mirjam","family":"Langeveld","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Metabolism Amsterdam University Medical Centers and Amsterdam Gastroenterology Endocrinology Metabolism (AGEM) Research Institute  Amsterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa","family":"Rudolph","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Metabolism Amsterdam University Medical Centers and Amsterdam Gastroenterology Endocrinology Metabolism (AGEM) Research Institute  Amsterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ute","family":"Spiekerkoetter","sequence":"additional","affiliation":[{"name":"Children's Hospital, Department of Pediatrics, Adolescent Medicine and Neonatology, Faculty of Medicine Freiburg University Hospital  Freiburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Allan M.","family":"Lund","sequence":"additional","affiliation":[{"name":"Centre Inherited Metabolic Diseases, Department of Clinical Genetics, Section\u00a04062 Copenhagen University Hospital  Rigshospitalet Denmark"},{"name":"Centre Inherited Metabolic Diseases, Department of Pediatrics, Section\u00a04062 Copenhagen University Hospital  Rigshospitalet Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annalisa","family":"Sechi","sequence":"additional","affiliation":[{"name":"Regional Coordinating Centre for Rare Diseases University Hospital of Udine  Udine Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1982-8046","authenticated-orcid":false,"given":"Halil Tuna","family":"Akar","sequence":"additional","affiliation":[{"name":"Department of Pediatric Metabolism and Nutrition Unit Hacettepe University Faculty of Medicine  Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0148-6332","authenticated-orcid":false,"given":"Karolina M.","family":"Stepien","sequence":"additional","affiliation":[{"name":"Adult Inherited Metabolic Disorders, Salford Royal Hospital Northern Care Alliance NHS Foundation Trust  Salford Greater Manchester UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Han\u0131m","family":"Aghakishili","sequence":"additional","affiliation":[{"name":"Department of Pediatric Nutrition and Metabolism, Cerrahpasa Medical Faculty Istanbul University\u2010Cerrahpasa  Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amelie S.","family":"Lotz\u2010Havla","sequence":"additional","affiliation":[{"name":"Dr. von Hauner Children's Hospital, LMU University Hospital Ludwig\u2010Maximilians\u2010Universit\u00e4t M\u00fcnchen  Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Klaus G.","family":"Parhofer","sequence":"additional","affiliation":[{"name":"Medical Department IV \u2013 Grosshadern LMU University Hospital, LMU Munich  Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8396-6764","authenticated-orcid":false,"given":"Saadet","family":"Mercimek\u2010Andrews","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics, Faculty of Medicine and Dentistry University of Alberta, Alberta Health Services  Edmonton Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Havva","family":"Yaz\u0131c\u0131","sequence":"additional","affiliation":[{"name":"Division of Metabolism and Nutrition, Department of Pediatrics Ege University Faculty of Medicine  \u0130zmir Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sema Kalkan","family":"U\u00e7ar","sequence":"additional","affiliation":[{"name":"Division of Metabolism and Nutrition, Department of Pediatrics Ege University Faculty of Medicine  \u0130zmir Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Scherer","sequence":"additional","affiliation":[{"name":"Division of Endocrinology and Metabolism, Department of Medicine III Medical University of Vienna  Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margreet","family":"Wagenmakers","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Erasmus MC Centre for Lysosomal and Metabolic Disease Erasmus University Medical Centre Rotterdam  Rotterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3151-3741","authenticated-orcid":false,"given":"Nur","family":"Arslan","sequence":"additional","affiliation":[{"name":"Division of Pediatric Metabolism, Department of Pediatrics Dokuz Eylul University Faculty of Medicine  Izmir Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5842-5912","authenticated-orcid":false,"given":"Irene","family":"Chang","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics University of California, San Francisco (UCSF)  San Francisco California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Allie","family":"LaTray","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics University of California, San Francisco (UCSF)  San Francisco California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vladimir","family":"Bzduch","sequence":"additional","affiliation":[{"name":"National Institute of Children's Diseases, Faculty of Medicine Comenius University Bratislava  Bratislava Slovakia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ivo","family":"Bari\u0107","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University Hospital Centre Zagreb  Zagreb Croatia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corina","family":"Weigel","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University Hospital of Erlangen  Erlangen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonja. L.","family":"van Ockenburg","sequence":"additional","affiliation":[{"name":"Department of Endocrinology University of Groningen, University Medical Center Groningen  Groningen the Netherlands"},{"name":"UMCG Center of Expertise for Carbohydrate, Fatty Acid Oxidation and Ketone Bodies Disorders University Medical Center Groningen  Groningen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander","family":"Rennings","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Radboud University Medical Center Nijmegen  Nijmegen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7259-1095","authenticated-orcid":false,"given":"Terry G. 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