ASN's Mission

To create a world without kidney diseases, the ASN Alliance for Kidney Health elevates care by educating and informing, driving breakthroughs and innovation, and advocating for policies that create transformative changes in kidney medicine throughout the world.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on X

Kidney Week

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

Authors

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

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.

Discussion

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