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既往减重手术后发生胃肠道出血,进而引发尿素循环障碍所致的高氨血症性脑病

Hyperammonemia Encephalopathy due to Urea Cycle Disorder Precipitated by Gastrointestinal Bleed in the Setting of Prior Bariatric Surgery.

作者信息

Loeffler Jeffrey, Elfiky Ahmed, Al Moussawi Hassan, Ravindran Nishal

机构信息

Department of Medicine, Staten Island University Hospital, NY.

Department of Gastroenterology and Hepatology, Staten Island University Hospital, NY.

出版信息

ACG Case Rep J. 2023 Oct 3;10(10):e01164. doi: 10.14309/crj.0000000000001164. eCollection 2023 Oct.

Abstract

The urea cycle is a metabolic pathway that excretes nitrogenous waste products from the body. Urea cycle disorders (UCDs) result from enzymatic deficiencies within this pathway, which can lead to life-threatening hyperammonemia. Gastric bypass-related hyperammonemia in patients who have undergone Roux-en-Y gastric bypass surgery has been previously reported. UCDs have been implicated as a cause of gastric bypass-related hyperammonemia. In this report, we present the case of a patient with a history of bariatric surgery who experienced severe hyperammonemia encephalopathy triggered by a gastrointestinal bleed due to an undiagnosed UCD.

摘要

尿素循环是一种将体内含氮废物排出体外的代谢途径。尿素循环障碍(UCDs)是由该途径中的酶缺乏引起的,可导致危及生命的高氨血症。先前已有报道,接受了Roux-en-Y胃旁路手术的患者会出现与胃旁路相关的高氨血症。UCDs被认为是胃旁路相关高氨血症的一个病因。在本报告中,我们介绍了一例有减肥手术史的患者,该患者因未被诊断出的UCD导致胃肠道出血,进而引发了严重的高氨血症性脑病。

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本文引用的文献

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Hyperammonemic syndrome after Roux-en-Y gastric bypass.Roux-en-Y 胃旁路术后高氨血症综合征。
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A longitudinal study of urea cycle disorders.尿素循环障碍的纵向研究。
Mol Genet Metab. 2014 Sep-Oct;113(1-2):127-30. doi: 10.1016/j.ymgme.2014.08.001. Epub 2014 Aug 10.
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The incidence of urea cycle disorders.尿素循环障碍的发病率。
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