Fenves Andrew Z, Kirkpatrick Haskell M, Patel Viralkumar V, Sweetman Lawrence, Emmett Michael
Department of Internal Medicine, Baylor University Medical Center, 3500 Gaston, Dallas, TX 75246, USA.
Clin J Am Soc Nephrol. 2006 May;1(3):441-7. doi: 10.2215/CJN.01411005. Epub 2006 Apr 19.
The endogenous organic acid metabolic acidoses that occur commonly in adults include lactic acidosis; ketoacidosis; acidosis that results from the ingestion of toxic substances such as methanol, ethylene glycol, or paraldehyde; and a component of the acidosis of kidney failure. Another rare but underdiagnosed cause of severe, high anion gap metabolic acidosis in adults is that due to accumulation of 5-oxoproline (pyroglutamic acid). Reported are four patients with this syndrome, and reviewed are 18 adult patients who were reported previously in the literature. Twenty-one patients had major exposure to acetaminophen (one only acute exposure). Eighteen (82%) of the 22 patients were women. Most of the patients were malnourished as a result of multiple medical comorbidities, and most had some degree of kidney dysfunction or overt failure. The chronic ingestion of acetaminophen, especially by malnourished women, may generate high anion gap metabolic acidosis. This undoubtedly is an underdiagnosed condition because measurements of serum and/or urinary 5-oxoproline levels are not readily available.
成年人中常见的内源性有机酸代谢性酸中毒包括乳酸酸中毒、酮症酸中毒、因摄入甲醇、乙二醇或副醛等有毒物质导致的酸中毒以及肾衰竭酸中毒的一部分。另一个导致成年人严重高阴离子间隙代谢性酸中毒的罕见但诊断不足的原因是5-氧脯氨酸（焦谷氨酸）的蓄积。本文报告了4例患有该综合征的患者，并对先前文献报道的18例成年患者进行了回顾。21例患者曾大量接触对乙酰氨基酚（1例仅为急性接触）。22例患者中有18例（82%）为女性。大多数患者因多种内科合并症而营养不良，且大多数患者有一定程度的肾功能不全或明显的肾衰竭。长期摄入对乙酰氨基酚，尤其是营养不良的女性，可能会引发高阴离子间隙代谢性酸中毒。这无疑是一种诊断不足的疾病，因为血清和/或尿液中5-氧脯氨酸水平的检测方法并不容易获得。
