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Abstract: TH-PO397

Mind the Gap: Pyroglutamic Acidosis as a Not-so-Rare Cause of High Anion Gap Metabolic Acidosis (HAGMA)

Session Information

Category: Fluid, Electrolytes, and Acid-Base Disorders

  • 1102 Fluid, Electrolyte, and Acid-Base Disorders: Clinical


  • Beavin, Sam, University of Kentucky Medical Center, Lexington, Kentucky, United States
  • Ahmed, Sadiq, University of Kentucky Medical Center, Lexington, Kentucky, United States

In addition to the common causes of high-anion gap metabolic acdiosis (such as lactic acidosis, ketoacidosis, and toxic alcohol ingestion), pyroglutamic acidosis can also result in HAGMA. This case report illustrates a patient in which otherwise unexplained metabolic acidosis was attributed to 5-oxoproline accumulation in the setting of chronic acetaminophen exposure and malnutrition.

Case Description

A 39-year-old man with a history of paraplegia, malnutrition, and prior PEG tube placement was admitted for management of a gastro-cutaneous fistula involving abdominal wall takedown and partial omentectomy. His post-operative course was complicated by pneumonia, septic shock, and AKI-D requiring two sessions of iHD on hospital days 8 and 10. Following HD treatments, he recovered renal function with appropriate urine output and stable electrolytes. During this period, he was started on scheduled acetaminophen 4 g daily for pain control.

Over the course of subsequent hospital days 11–13, he developed worsening AGMA with a peak anion gap of 26. After initial respiratory compensation, he ultimately required intubation for tachypnea and worsening hypercarbia. The workup for common causes of AGMA was non-revealing with normal lactate, D-lactate, alcohol, and beta-hydroxybuterate levels. Organic acid analysis of urine showed significantly elevated levels of 5-oxoproline excretion (6797 mmol/mol Cr., reference range: <62). Acetaminophen was discontinued, with subsequent resolution of metabolic acidosis over the next 3 days.


This case demonstrates the development of HAGMA in a malnourished patient after several days of acetaminophen ingestion where the workup for common causes of HAGMA was non-revealing. In these situations, the accumulation of 5-oxoproline (pyroglutamic acid) is an important consideration. This rare condition can occur due to chronic acetaminophen exposure and subsequent glutathione depletion, particularly in the setting of poor nutritional status and a malfunctioning intestine.

Acidosis Trend
Hospital DayHCO3-Anion Gap
Day 10 (last iHD)2118
Day 111822
Day 121523
Day 13 (Acetaminophen stopped)1026
Day 141521
Day 152114