Journal ArticleDOI
Pregnancy complications are frequent in long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency
TLDR
The results support the role of fatty acid accumulation in the pathogenesis of preeclampsia, and analysis for the prevalent mutation of this deficiency may be warranted in pregnancies with severe preeClampsia.About:
This article is published in American Journal of Obstetrics and Gynecology.The article was published on 1998-03-01. It has received 137 citations till now. The article focuses on the topics: Acute fatty liver of pregnancy & Pregnancy.read more
Citations
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Journal ArticleDOI
A Fetal Fatty-Acid Oxidation Disorder as a Cause of Liver Disease in Pregnant Women
Jamal A. Ibdah,Michael J. Bennett,Piero Rinaldo,Yiwen Zhao,Beverly Gibson,Harold F. Sims,Arnold W. Strauss +6 more
TL;DR: A small number of women diagnosed with acute fatty liver of pregnancy and the HELLP syndrome (hemolysis, elevated liver-enzyme levels, and a low platelet count) also have low iron levels, which are normal during pregnancy.
Journal ArticleDOI
Understanding and managing HELLP syndrome: the integral role of aggressive glucocorticoids for mother and child.
TL;DR: A critical review of published research about this variant form of severe preeclampsia, focused primarily on what is known about the pathogenesis of this disorder as it relates to patient experience with corticosteroids for its management, leads to the conclusion that there is maternal-fetal benefit realized when potent glucocorticoids are aggressively used for its treatment as mentioned in this paper.
Journal ArticleDOI
Disorders of mitochondrial long-chain fatty acid oxidation and the carnitine shuttle
Suzan J. G. Knottnerus,Suzan J. G. Knottnerus,Jeannette C. Bleeker,Jeannette C. Bleeker,Rob C. I. Wüst,Sacha Ferdinandusse,Lodewijk IJlst,Frits A. Wijburg,Ronald J.A. Wanders,Gepke Visser,Gepke Visser,Riekelt H. Houtkooper +11 more
TL;DR: This review aims to function as a comprehensive reference for clinical and laboratory findings for clinicians who are confronted with pediatric and adult patients with a possible diagnosis of a long-chain fatty acid oxidation disorder.
Journal ArticleDOI
Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency: Clinical Presentation and Follow-Up of 50 Patients
Margarethe E. J. den Boer,Ronald J.A. Wanders,Andrew A. M. Morris,Lodewijk IJlst,Hugo S. A. Heymans,Frits A. Wijburg +5 more
TL;DR: LCHAD deficiency often presents with a combination of chronic nonspecific symptoms, and survival can be improved by prompt diagnosis, but morbidity remains alarmingly high despite current therapeutic regimes.
Journal ArticleDOI
Imitators of severe preeclampsia
TL;DR: This review focuses on diagnosis, management, and counseling of women who develop these syndromes based on results of recent studies.
References
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Journal ArticleDOI
Placental bed spiral arteries in the hypertensive disorders of pregnancy.
Robert Pijnenborg,John Anthony,D. A. Davey,Alexandra Rees,Andrew Tiltman,Lisbeth Vercruysse,André Van Assche +6 more
TL;DR: The histology of the placental bed spiral arteries in normal pregnancy and in pregnancies complicated by hypertension, with or without proteinura is investigated.
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Novel fatty acid beta-oxidation enzymes in rat liver mitochondria. II. Purification and properties of enoyl-coenzyme A (CoA) hydratase/3-hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase trifunctional protein.
TL;DR: It is concluded that this enzyme is not long-chain 3-hydroxyacyl-CoA dehydrogenase; rather, it is enoyl- CoA hydratase/3-hydroxymethicone-like 3-ketoacyl -CoA thiolase trifunctional protein, which is free from the following enzymes.
Journal ArticleDOI
Pregnancy and fetal long-chain 3-hydroxyacyl coenzyme A dehydrogenase deficiency
TL;DR: There may be adverse effects on maternal liver function from a fetus with LCHAD deficiency, and Heterozygosity in the mother cannot alone account for the adverse effects because of the segregation of these effects with fetal LCHad status.
Journal ArticleDOI
The molecular basis of pediatric long chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with maternal acute fatty liver of pregnancy
Harold F. Sims,Jeffrey C. Brackett,Cynthia K. Powell,William R. Treem,Daniel E. Hale,Michael J. Bennett,Beverly Gibson,Scott Shapiro,Arnold W. Strauss +8 more
TL;DR: It is demonstrated that mutations in the LCHAD domain of the trifunctional protein alpha subunit in affected offspring are associated with maternal acute fatty liver of pregnancy and the initial delineation of the molecular basis of isolated LCHad deficiency is delineated.
Journal ArticleDOI
Molecular basis of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: identification of the major disease-causing mutation in the alpha-subunit of the mitochondrial trifunctional protein.
TL;DR: This work sequenced the cDNAs encoding the alpha- and beta-subunits of mitochondrial trifunctional protein and revealed one G-->C mutation at nucleotide position 1528 in the 3-hydroxyacyl-CoA dehydrogenase encoding region of thealpha-subunit.