Hundemer Gregory L, Fenves Andrew Z
Division of Nephrology, Massachusetts General Hospital (Hundemer, Fenves) and the Division of Renal Medicine, Brigham and Women's Hospital (Hundemer), Boston, Massachusetts.
Proc (Bayl Univ Med Cent). 2017 Apr;30(2):169-170. doi: 10.1080/08998280.2017.11929570.
Acquired 5-oxoprolinemia is increasingly recognized as a cause of anion gap metabolic acidosis. It predominantly occurs in chronically ill, malnourished women with impaired renal function and chronic acetaminophen ingestion. Depletion of glutathione and cysteine stores leads to elevated 5-oxoproline levels. N-acetylcysteine, given its effect in repleting glutathione and cysteine stores, has been proposed as a potential treatment for 5-oxoprolinemia, though reports of its successful use are lacking. We present a case of 5-oxoproline metabolic acidosis that persisted despite discontinuation of acetaminophen. However, the acidosis rapidly resolved with N-acetylcysteine administration.
获得性5-氧脯氨酸血症越来越被认为是阴离子间隙代谢性酸中毒的一个原因。它主要发生在患有慢性疾病、营养不良、肾功能受损且长期服用对乙酰氨基酚的女性身上。谷胱甘肽和半胱氨酸储备的耗竭导致5-氧脯氨酸水平升高。鉴于N-乙酰半胱氨酸具有补充谷胱甘肽和半胱氨酸储备的作用，有人提出将其作为5-氧脯氨酸血症的一种潜在治疗方法，不过缺乏其成功应用的报道。我们报告一例5-氧脯氨酸代谢性酸中毒病例，尽管停用了对乙酰氨基酚，但酸中毒仍持续存在。然而，给予N-乙酰半胱氨酸后酸中毒迅速得到缓解。
