Elevated hydroxyacylcarnitines in a carrier of LCHAD deficiency during acute liver disease of pregnancy - a common feature of the pregnancy complication?

Eskelin PM; Laitinen KA; Tyni TA

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Elevated hydroxyacylcarnitines in a carrier of LCHAD deficiency during acute liver disease of pregnancy - a common feature of the pregnancy complication?

机译：妊娠急性肝病期间LCHAD缺乏症携带者中的羟酰基肉碱含量升高-妊娠并发症的共同特征是？

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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.

机译：长链3-羟酰基辅酶A脱氢酶（LCHAD）缺乏症是线粒体脂肪酸β-氧化缺陷，其特征在于长链羟酰基肉碱中间体的积累，该疾病的女性携带者有妊娠并发症的风险。我们发现，在怀孕31周时，LCHAD缺乏症携带者的血液中长链羟基酰基肉碱物种升高，而在妊娠样脂肪肝受累的急性脂肪肝中，LCHAD缺乏胎儿，但在怀孕25周时处于正常范围。这一发现支持了LCHAD和MTP缺陷携带者中AFLP发病过程中酰基肉碱积累的假说。

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《Molecular genetics and metabolism》 |2010年第2期|共3页
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Eskelin PM; Laitinen KA; Tyni TA;
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正文语种 eng
中图分类内分泌腺疾病及代谢病;
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1. Elevated hydroxyacylcarnitines in a carrier of LCHAD deficiency during acute liver disease of pregnancy - a common feature of the pregnancy complication? [J] . Eskelin PM, Laitinen KA, Tyni TA Molecular genetics and metabolism . 2010,第2期

机译：妊娠急性肝病期间LCHAD缺乏症携带者中的羟酰基肉碱含量升高-妊娠并发症的共同特征是？
2. Acute fatty liver of pregnancy and neonatal long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency. [J] . Gutierrez-Junquera C, Balmaseda E, Gil E, European journal of pediatrics . 2009,第1期

机译：妊娠急性脂肪肝和新生儿长链3-羟酰基辅酶A脱氢酶（LCHAD）缺乏。
3. Acute fatty liver of pregnancy and neonatal long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency [J] . Carolina Gutiérrez Junquera, Elena Balmaseda, Esther Gil, European Journal of Pediatrics . 2009,第1期

机译：妊娠急性脂肪肝和新生儿长链3-羟酰基辅酶A脱氢酶（LCHAD）缺乏症
4. Imaging of Acute Maternal Diseases in Pregnancy [C] . Andrew J. LeRoy, the Radiological Society of North America Scientific Assembly and Annual Meeting of the Radiological Society of North America . 2006

机译：妊娠期急性母体疾病的成像
5. Gentamicin-modified Nanocarriers for Placental Targeted Drug Delivery to Treat Pregnancy-related Complications [D] . ?Alfaifi, Ali 2019

机译：用于胎盘针对性药物递送的庆大霉素改性纳米载体治疗妊娠相关的并发症
6. EFFECT OF FEEDING EXERCISE AND GENOTYPE ON PLASMA 3-HYDROXYACYLCARNITINES IN CHILDREN WITH LCHAD DEFICIENCY [O] . Melanie B. Gillingham, Dietrich Matern, Cary O. Harding -1

机译：取食锻炼效果和基因型对血浆3 HYDROXYaCYLCaRNITINEs患儿LCHaD缺乏症
7. EFFECT OF FEEDING, EXERCISE AND GENOTYPE ON PLASMA 3-HYDROXYACYLCARNITINES IN CHILDREN WITH LCHAD DEFICIENCY [O] . Melanie B. Gillingham, Dietrich Matern, Cary O. Harding 2015

机译：喂养，运动和基因型对LCHaD缺乏症儿童血浆3-羟基亚喹啉的影响

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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