title: Successful management of pregnancy with very-long-chain acyl-coenzyme A dehydrogenase deficiency.
abstract: Very-long-chain acyl-coenzyme A dehydrogenase deficiency (VLCADD) is a rare and life-threatening disease characterized by an enzymatic defect in the fatty acid β-oxidation pathway. A nulliparous woman with VLCADD showed improvements in serum levels of the long-chain acylcarnitine moiety (C14:1) during pregnancy and successfully delivered a healthy infant vaginally. Pregnancy and vaginal delivery can be successfully completed in patients with VLCADD with careful management.
authors: Department of Obstetrics and Gynecology, Osaka City University Graduate School of Medicine, Osaka, Japan.
authors: Hiroko
authors: H
authors: Yamamoto
authors: Department of Obstetrics and Gynecology, Osaka City University Graduate School of Medicine, Osaka, Japan.
authors: Daisuke
authors: D
authors: Tachibana
authors: Department of Pediatrics, Hiroshima University Graduate School of Biomedical and Health Sciences, Hiroshima, Japan.
authors: Go
authors: G
authors: Tajima
authors: Department of Health Science, University of Fukui, Fukui, Japan.
authors: Yosuke
authors: Y
authors: Shigematsu
authors: Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka, Japan.
authors: Takashi
authors: T
authors: Hamasaki
authors: Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka, Japan.
authors: Akemi
authors: A
authors: Tanaka
authors: Department of Obstetrics and Gynecology, Osaka City University Graduate School of Medicine, Osaka, Japan.
authors: Masayasu
authors: M
authors: Koyama
mesh_terms: D044942:Acyl-CoA Dehydrogenase, Long-Chain / Q000097:blood / Q000172:deficiency*; D001510:Bed Rest; D002648:Child; D003131:Combined Modality Therapy; D000080984:Congenital Bone Marrow Failure Syndromes; D057210:Delayed Diagnosis; D005229:Fatty Acids, Monounsaturated / Q000097:blood; D005260:Female; D006760:Hospitalization; D006801:Humans; D007231:Infant, Newborn; D007564:Japan; D007751:Labor, Induced; D008052:Lipid Metabolism, Inborn Errors / Q000097:blood / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*; D028361:Mitochondrial Diseases / Q000097:blood / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*; D009135:Muscular Diseases / Q000097:blood / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*; D063806:Myalgia / Q000209:etiology / Q000517:prevention & control; D011247:Pregnancy; D011248:Pregnancy Complications / Q000097:blood / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*; D018566:Pregnancy, High-Risk / Q000097:blood; D011296:Prenatal Diagnosis; D047929:Term Birth
chemical_list: D005229:Fatty Acids, Monounsaturated; D044942:Acyl-CoA Dehydrogenase, Long-Chain; C054211:9-tetradecenoic acid
keywords: placenta; pregnancy; very-long-chain acyl-coenzyme A dehydrogenase deficiency