Management of Multiple Acyl-CoA Dehydrogenase Deficiency (MADD) in Pregnancy.

IF 3.7 3区生物学 Q2 BIOCHEMISTRY & MOLECULAR BIOLOGY

Metabolites Pub Date : 2025-06-24 DOI:10.3390/metabo15070432

Matthew A Shear, Allie LaTray, Irene J Chang, Annalisa Post, Renata C Gallagher

引用次数: 0

Abstract

Multiple acyl-CoA dehydrogenase deficiency (MADD), also known as glutaric acidemia/glutaric aciduria type II (GA II), is an inborn error of fatty acid, amino acid, and choline metabolism. The chronic management of MADD involves both dietary fat and protein restriction to reduce the substrates of the dehydrogenases affected, the avoidance of prolonged fasting as in any fat metabolism disorder, and monitoring for potential complications. Due to its rarity, there is little published experience on the management of MADD in pregnancy. Herein, we report the successful management of a pregnancy in a patient with late-onset or type III MADD, with considerations for preconception, antepartum, intrapartum, and postpartum care.

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妊娠期多重酰基辅酶a脱氢酶缺乏症（MADD）的处理。

多重酰基辅酶a脱氢酶缺乏症（MADD），也称为戊二酸血症/戊二酸尿症II型（GA II），是一种先天性脂肪酸、氨基酸和胆碱代谢错误。MADD的慢性管理包括限制饮食中的脂肪和蛋白质，以减少受影响的脱氢酶的底物，避免长时间禁食，如在任何脂肪代谢紊乱中，并监测潜在的并发症。由于其罕见性，很少有关于妊娠期MADD管理的出版经验。在此，我们报告了一个成功的管理怀孕的迟发型或III型MADD患者，考虑到孕前，产前，产时和产后护理。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Metabolites Biochemistry, Genetics and Molecular Biology-Molecular Biology

CiteScore

5.70

自引率

7.30%

发文量

1070

审稿时长

17.17 days

期刊介绍： Metabolites (ISSN 2218-1989) is an international, peer-reviewed open access journal of metabolism and metabolomics. Metabolites publishes original research articles and review articles in all molecular aspects of metabolism relevant to the fields of metabolomics, metabolic biochemistry, computational and systems biology, biotechnology and medicine, with a particular focus on the biological roles of metabolites and small molecule biomarkers. Metabolites encourages scientists to publish their experimental and theoretical results in as much detail as possible. Therefore, there is no restriction on article length. Sufficient experimental details must be provided to enable the results to be accurately reproduced. Electronic material representing additional figures, materials and methods explanation, or supporting results and evidence can be submitted with the main manuscript as supplementary material.