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胆汁淤积性疾病患儿的类二十烷酸合成

Eicosanoid synthesis in children with cholestatic disease.

作者信息

Dupont J, Amédée-Manesme O, Pepin D, Chambaz J

机构信息

Department of Food and Nutrition, Iowa State University, Ames 50011.

出版信息

J Inherit Metab Dis. 1990;13(2):212-8. doi: 10.1007/BF01799688.

Abstract

The possibility of malabsorption of triglycerides contained in the diets of children with cholestasis suggests a deficiency of essential fatty acids and, therefore, probable effects on eicosanoid metabolism. Children with either biliary atresia (BA) or syndromatic paucity of interlobular bile ducts (PILBD) were evaluated as to plasma and platelet total lipid fatty acid composition and synthesis of prostaglandins (PG) E1, PGE2, PGI2, PGF2, and thromboxane (TXB2) by whole blood incubated at 37 degrees C for 10 min. In both diseases linoleate deficiency was present as shown by low 18:2 fatty acids in plasma lipids. The children with BA had lower plasma arachidonate than controls but normal eicosanoid synthesis except for excess PGI2. Those with PILBD had low platelet arachidonate and were severely deficient in TXB2 synthesis (less than 10% of controls). Three children with PILBD were fed a supplement of structured triglyceride (Captex 810) intended to provide as much as 10% of energy as linoleate for 2-3 months. Results for these three cases suggested that insufficient linoleate was absorbed to increase plasma linoleate and differences in eicosanoids could not be attributed to linoleate supplementation.

摘要

胆汁淤积症患儿饮食中甘油三酯吸收不良的可能性表明必需脂肪酸缺乏,因此可能会影响类花生酸代谢。对患有胆道闭锁(BA)或综合征性小叶间胆管缺乏(PILBD)的儿童进行了评估,检测其血浆和血小板总脂质脂肪酸组成,以及通过在37℃孵育10分钟的全血合成前列腺素(PG)E1、PGE2、PGI2、PGF2和血栓素(TXB2)的情况。在这两种疾病中,血浆脂质中18:2脂肪酸含量低表明存在亚油酸缺乏。患有BA的儿童血浆花生四烯酸含量低于对照组,但除PGI2过量外,类花生酸合成正常。患有PILBD的儿童血小板花生四烯酸含量低,TXB2合成严重不足(低于对照组的10%)。三名患有PILBD的儿童接受了结构化甘油三酯(Captex 810)补充剂,旨在提供相当于能量10%的亚油酸,为期2至3个月。这三个病例的结果表明,亚油酸吸收不足,无法增加血浆亚油酸,类花生酸的差异不能归因于亚油酸补充。

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