| Pregnancy complications are frequent in long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency. | |
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MedLine Citation:
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PMID: 9539533 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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OBJECTIVE: Preeclampsia-related complications of pregnancy have been detected in carriers of long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency, a recently discovered disorder of mitochondrial fatty acid oxidation. Because no comprehensive study is available, we studied the frequency of pregnancy complications in mothers who had given birth to children with this disorder. STUDY DESIGN: Data of all pregnancies of 18 mothers to 28 diagnosed patients with long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency were reviewed retrospectively. From a total 79 pregnancies 16 early abortions were excluded; 63 pregnancies were included, and the fetus was affected in 29. RESULTS: One child born prematurely died neonatally but none of the mothers died. Preeclampsia, the syndrome of hemolysis, elevated liver enzymes, and low platelets, and acute fatty liver of pregnancy occurred in 31% and intrahepatic cholestasis in 10% of pregnancies with a long chain 3-hydroxyacyl-coenzyme A-deficient fetus but in none of the pregnancies with a healthy fetus. A total of 40% of affected neonates were born prematurely and 47% had growth restriction, whereas none of the healthy neonates were premature and growth restriction occurred in only 17% (p < 0.01). Prematurity and growth restriction could not be explained solely by the preeclampsia-related conditions. CONCLUSIONS: In pregnancies with a long-chain 3-hydroxyacyl-coenzyme A-deficient fetus the frequency of preeclampsia-related conditions is high. The results support the role of fatty acid accumulation in the pathogenesis of preeclampsia. Analysis for the prevalent mutation of this deficiency may be warranted in pregnancies with severe preeclampsia. |
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Authors:
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T Tyni; E Ekholm; H Pihko |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: American journal of obstetrics and gynecology Volume: 178 ISSN: 0002-9378 ISO Abbreviation: Am. J. Obstet. Gynecol. Publication Date: 1998 Mar |
Date Detail:
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Created Date: 1998-04-22 Completed Date: 1998-04-22 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 0370476 Medline TA: Am J Obstet Gynecol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 603-8 Citation Subset: AIM; IM |
Affiliation:
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Division of Child Neurology, Children's Hospital, University of Helsinki, Finland. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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3-Hydroxyacyl CoA Dehydrogenases
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deficiency* Cholestasis, Intrahepatic / complications Fatty Liver / complications Female Fetal Diseases / enzymology* HELLP Syndrome / complications Heterozygote* Humans Infant, Newborn Pre-Eclampsia / complications*, genetics* Pregnancy Pregnancy Complications Pregnancy Outcome* Retrospective Studies |
| Chemical | |
Reg. No./Substance:
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EC 1.1.1.35/3-Hydroxyacyl CoA Dehydrogenases |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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