Document Detail


Clinical and biochemical improvement of very long-chain acyl-CoA dehydrogenase deficiency in pregnancy.
MedLine Citation:
PMID:  20668464     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is an enzymatic defect of the fatty acid (FA) beta oxidation pathway. In catabolic states, such as labor and early postpartum period, patients are potentially prone to metabolic decompensation and subsequent rhabdomyolysis with increased risk for myoglobinuria and renal insufficiency. We report a 21-year-old primigravida with a previously characterized VLCAD deficiency, who experienced frequent and unprovoked episodes of rhabdomyolysis before pregnancy. As there was no published experience to guide her management, a detailed multidisciplinary care plan was established to minimize the potential morbidity. Although there is little known about the antenatal course of gravidae affected by VLCAD, we predicted that placental and fetal beta-oxidation in an unaffected pregnancy may temporize or even improve maternal FA beta-oxidation. Consistent with our prediction, we observed a significant clinical and biochemical improvement throughout her pregnancy, and she delivered vaginally with an uncomplicated postpartum course. We conclude that although VLCAD deficiency can present a therapeutic challenge during pregnancy, the beneficial placento-maternal metabolic interactions and the implementation of a proper peripartum management reassure a successful antenatal and perinatal outcome.
Authors:
H Mendez-Figueroa; O A Shchelochkov; A Shaibani; K Aagaard-Tillery; M S Shinawi
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of perinatology : official journal of the California Perinatal Association     Volume:  30     ISSN:  1476-5543     ISO Abbreviation:  J Perinatol     Publication Date:  2010 Aug 
Date Detail:
Created Date:  2010-07-29     Completed Date:  2010-11-22     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8501884     Medline TA:  J Perinatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  558-62     Citation Subset:  IM    
Affiliation:
Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, TX 63110, USA.
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MeSH Terms
Descriptor/Qualifier:
Acyl-CoA Dehydrogenase, Long-Chain / deficiency*
Carnitine / analogs & derivatives,  blood
Female
Humans
Infant, Newborn
Lipid Metabolism, Inborn Errors / complications*,  physiopathology
Male
Pregnancy
Pregnancy Complications / physiopathology*
Prenatal Care
Rhabdomyolysis / etiology*
Term Birth
Young Adult
Chemical
Reg. No./Substance:
0/acylcarnitine; 541-15-1/Carnitine; EC 1.3.99.13/Acyl-CoA Dehydrogenase, Long-Chain

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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