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KMID : 0918520180180030069
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Journal of the Korean Society of Inherited Metabolic Disease
2018 Volume.18 No. 3 p.69 ~ p.77
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Defects in Ketone Body Metabolism and Pregnancy
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Fukao Toshiyuki
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Abstract
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Pregnancy and delivery pose a high risk of developing metabolic decompensation in women with defects of ketone body metabolism. In this review, the available reported cases in pregnancy are summarized. It is very important to properly manage women with defects of ketone body metabolism during pregnancy, especially nausea and vomiting in the first trimester of pregnancy, and during labor and delivery. Pregnant women with deficiencies of HMG-CoA lyase or succinyl-CoA:3-ketoacid CoA transferase (SCOT) often experience metabolic decompensations with nausea and vomiting of pregnancy, often requiring hospitalization. For successful delivery and to reduce stresses, vaginal delivery with epidural anesthesia or elective cesarean delivery with epidural or spinal anesthesia are recommended for women with HMG-CoA lyase and SCOT deficiency. In beta-ketothiolase deficiency, four pregnancies in three patients had favorable outcomes without severe metabolic problems.
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KEYWORD
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Pregnancy, Labor, Delivery, Ketone body metabolism, HMG-CoA lyase, HMG-CoA synthase, Succinyl-CoA:3-ketoacid CoA transferase, Beta-ketothiolase
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