Lanoy Charlotte, Bouckaert Yves
Intensive Care Unit, Centre Hospitalier Universitaire (CHU) de Tivoli, Avenue Max Buset 34, 7130, La Louvière, Belgium.
J Med Case Rep. 2016 Jun 23;10(1):184. doi: 10.1186/s13256-016-0964-x.
Frequent causes of high anion gap metabolic acidosis are well known: ethanol, methanol, and ethylene glycol intoxication; hyperglycemia; lactic or D-lactic acidosis; and impaired renal function. There are other causes, less frequent but also important. This report illustrates a rare case of a patient with increased anion gap metabolic acidosis due to a deficit of the γ-glutamyl cycle that led to 5-oxoproline (acid pyroglutamic) accumulation.
An 82-year-old white woman was admitted to our intensive care unit because of septic shock caused by right knee methicillin-sensitive Staphylococcus aureus-induced arthritis. She was treated for 10 days with flucloxacillin and rifampicin and developed metabolic acidosis with high anion gap. Her test results for methanol, ethanol, ethylene glycol, and acetylsalicylic acid were negative. Her glycemia, lactate level, and renal function were normal. However, the result of a urinary assay for pyroglutamate was positive. We concluded that the patient had metabolic acidosis induced by accumulation of 5-oxoproline. We modified her antibiotic treatment, administered acetylcysteine, and her acidosis resolved.
5-Oxoprolinuria (pyroglutamic acid accumulation) is a rare, probably underdiagnosed cause of transient metabolic acidosis with increased anion gap.
高阴离子间隙代谢性酸中毒的常见病因众所周知:乙醇、甲醇和乙二醇中毒;高血糖;乳酸或D - 乳酸酸中毒;以及肾功能受损。还有其他病因,虽不常见但也很重要。本报告阐述了一例罕见病例,患者因γ - 谷氨酰循环缺陷导致5 - 氧脯氨酸(酸性焦谷氨酸)蓄积,从而引发阴离子间隙增加的代谢性酸中毒。
一名82岁白人女性因右膝耐甲氧西林金黄色葡萄球菌引起的关节炎导致感染性休克入住我们的重症监护病房。她接受了10天的氟氯西林和利福平治疗,并出现了高阴离子间隙的代谢性酸中毒。她的甲醇、乙醇、乙二醇和乙酰水杨酸检测结果均为阴性。她的血糖、乳酸水平和肾功能正常。然而,尿焦谷氨酸检测结果呈阳性。我们得出结论,该患者因5 - 氧脯氨酸蓄积导致代谢性酸中毒。我们调整了她的抗生素治疗方案,给予乙酰半胱氨酸,她的酸中毒得到缓解。
5 - 氧脯氨酸尿症(焦谷氨酸蓄积)是导致短暂性阴离子间隙增加的代谢性酸中毒的一种罕见且可能未被充分诊断的病因。