| Elevated hydroxyacylcarnitines in a carrier of LCHAD deficiency during acute liver disease of pregnancy - a common feature of the pregnancy complication? | |
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MedLine Citation:
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PMID: 20363656 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency is a mitochondrial fatty acid beta-oxidation defect characterized by accumulation of long-chain hydroxyacylcarnitine intermediates and female carriers of this disorder are in risk for pregnancy complications. We found elevated blood long-chain hydroxyacylcarnitine species in a carrier of LCHAD deficiency at 31weeks of pregnancy with a LCHAD deficient fetus during acute fatty liver of pregnancy-like liver involvement, but had been within the normal range at 25weeks of pregnancy. This finding supports the hypothesis of acylcarnitine accumulation in pathogenesis of AFLP in carriers of LCHAD and MTP deficiencies. |
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Authors:
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Petra M Eskelin; Kalevi A Laitinen; Tiina A Tyni |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, Non-U.S. Gov't Date: 2010-03-16 |
Journal Detail:
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Title: Molecular genetics and metabolism Volume: 100 ISSN: 1096-7206 ISO Abbreviation: Mol. Genet. Metab. Publication Date: 2010 Jun |
Date Detail:
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Created Date: 2010-05-17 Completed Date: 2010-08-09 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9805456 Medline TA: Mol Genet Metab Country: United States |
Other Details:
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Languages: eng Pagination: 204-6 Citation Subset: IM |
Affiliation:
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Department of Pediatric Neurology, Helsinki University Central Hospital, Helsinki, Finland. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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3-Hydroxyacyl CoA Dehydrogenases
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deficiency* Acute Disease Adult Carnitine / analogs & derivatives*, blood Fatty Liver / genetics Female Humans Lipid Metabolism, Inborn Errors / genetics Liver Diseases / genetics Multienzyme Complexes / genetics Pregnancy Pregnancy Complications / etiology, genetics* Pregnancy Trimester, Third |
| Chemical | |
Reg. No./Substance:
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0/Multienzyme Complexes; 0/acylcarnitine; 0/fatty acid beta-oxidation multienzyme complex; 541-15-1/Carnitine; EC 1.1.1.211/long-chain 3-hydroxyacyl CoA dehydrogenase; EC 1.1.1.35/3-Hydroxyacyl CoA Dehydrogenases |
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