Yeoh Cindy, Teng Howard, Jackson Jacob, Hingula Lee, Irie Takeshi, Legler Aron, Levine Corrine, Chu Iris, Chai Casey, Tollinche Luis
Department of Anesthesiology and Critical Care Medicine, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA.
Department of Anesthesiology, Weill Cornell Medical College and New York Presbyterian Hospital, New York, NY, USA.
Curr Anesthesiol Rep. 2019 Sep;9(3):340-359. doi: 10.1007/s40140-019-00345-w. Epub 2019 Jul 12.
Metabolic disorders encompass a group of inherited inborn errors of metabolism that are uncommonly encountered but can pose challenges when encountered during the perioperative period. Hence, it is paramount that anesthesiologists are experienced and familiar with management of these conditions.
Hundreds of inborn errors of metabolism have already been identified, yet new metabolic disorders continue to be discovered with advancements in genomic science.
In our general review, we define the more common metabolic disorders encountered in perioperative medicine and discuss the perioperative anesthetic considerations and challenges associated with each disorder. The following disorders are covered in our review: disorders of carbohydrate metabolism, disorders of amino acid metabolism, disorders of branched-chain amino acid metabolism, organic acidemias, mitochondrial disorders, lysosomal storage disorders, metal metabolism disorders, and urea cycle disorders.
代谢紊乱包括一组遗传性先天性代谢缺陷，虽然不常见，但在围手术期遇到时可能会带来挑战。因此，麻醉医生有处理这些情况的经验并熟悉相关管理至关重要。
数百种先天性代谢缺陷已被识别，但随着基因组科学的发展，仍不断有新的代谢紊乱被发现。
在我们的综述中，我们定义了围手术期医学中更常见的代谢紊乱，并讨论了与每种紊乱相关的围手术期麻醉注意事项和挑战。我们的综述涵盖以下紊乱：碳水化合物代谢紊乱、氨基酸代谢紊乱、支链氨基酸代谢紊乱、有机酸血症、线粒体疾病、溶酶体贮积症、金属代谢紊乱和尿素循环紊乱。
