Clinical and biochemical improvement of very long-chain acyl-CoA dehydrogenase deficiency in pregnancy.
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...
| Publicado en: | Journal of Perinatology Vol. 30; no. 8; pp. 558 - 563 |
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| Autores principales: | , , , , |
| Formato: | case study tables/charts Journal Article |
| Publicado: |
Springer Nature
Aug2010
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105068434&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 105068434 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 07438346 DZF jtl: Journal of Perinatology issn: 07438346 maglogo: Y pubinfo: dt: Aug2010 vid: 30 iid: 8 pid: 2579 pub: Springer Nature place: Dordrecht, <Blank> artinfo: ui: 105068434 2010731625 10.1038/jp.2009.198 NLM20668464 105068434 ppf: 558 ppct: 5 formats: fmt: @attributes: type: P tig: atl: Clinical and biochemical improvement of very long-chain acyl-CoA dehydrogenase deficiency in pregnancy. aug: au: Mendez-Figueroa H Shchelochkov OA Shaibani A Aagaard-Tillery K Shinawi MS affil: Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, TX, USA sug: subj: Deficiency Diseases In Pregnancy Enzymes Metabolism Adult Female Pregnancy Adult: 19-44 years Female ab: 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. pubtype: Academic Journal doctype: case study tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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