Successful management of pregnancy with very-long-chain acyl-coenzyme A dehydrogenase deficiency

Hiroko Yamamoto; Daisuke Tachibana; Go Tajima; Yosuke Shigematsu; Takashi Hamasaki; Akemi Tanaka; Masayasu Koyama

doi:10.1111/jog.12672

Successful management of pregnancy with very-long-chain acyl-coenzyme A dehydrogenase deficiency

J Obstet Gynaecol Res. 2015 Jul;41(7):1126-8. doi: 10.1111/jog.12672. Epub 2015 Feb 6.

Authors

Hiroko Yamamoto¹, Daisuke Tachibana¹, Go Tajima², Yosuke Shigematsu³, Takashi Hamasaki⁴, Akemi Tanaka⁴, Masayasu Koyama¹

Affiliations

¹ Department of Obstetrics and Gynecology, Osaka City University Graduate School of Medicine, Osaka, Japan.
² Department of Pediatrics, Hiroshima University Graduate School of Biomedical and Health Sciences, Hiroshima, Japan.
³ Department of Health Science, University of Fukui, Fukui, Japan.
⁴ Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka, Japan.

PMID: 25655073
DOI: 10.1111/jog.12672

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.

Keywords: placenta; pregnancy; very-long-chain acyl-coenzyme A dehydrogenase deficiency.

Publication types

Case Reports

MeSH terms

Acyl-CoA Dehydrogenase, Long-Chain / blood
Acyl-CoA Dehydrogenase, Long-Chain / deficiency*
Bed Rest
Child
Combined Modality Therapy
Congenital Bone Marrow Failure Syndromes
Delayed Diagnosis
Fatty Acids, Monounsaturated / blood
Female
Hospitalization
Humans
Infant, Newborn
Japan
Labor, Induced
Lipid Metabolism, Inborn Errors / blood
Lipid Metabolism, Inborn Errors / diagnosis
Lipid Metabolism, Inborn Errors / physiopathology
Lipid Metabolism, Inborn Errors / therapy*
Mitochondrial Diseases / blood
Mitochondrial Diseases / diagnosis
Mitochondrial Diseases / physiopathology
Mitochondrial Diseases / therapy*
Muscular Diseases / blood
Muscular Diseases / diagnosis
Muscular Diseases / physiopathology
Muscular Diseases / therapy*
Myalgia / etiology
Myalgia / prevention & control
Pregnancy
Pregnancy Complications / blood
Pregnancy Complications / diagnosis
Pregnancy Complications / physiopathology
Pregnancy Complications / therapy*
Pregnancy, High-Risk* / blood
Prenatal Diagnosis
Term Birth

Substances

Fatty Acids, Monounsaturated
Acyl-CoA Dehydrogenase, Long-Chain
9-tetradecenoic acid

Supplementary concepts

VLCAD deficiency