Pyroglutamic acidemia: a cause of high anion gap metabolic acidosis

Crit Care Med. 2000 Jun;28(6):1803-7. doi: 10.1097/00003246-200006000-00018.


Objective: To report four cases of pyroglutamic acidemia in adults causing clinically significant acidosis.

Data sources: Patients admitted to the intensive care units of the Alfred Hospital (a quaternary referral center) and Geelong Hospital (a major regional center) with an unexplained high anion gap acidosis.

Conclusions: Pyroglutamic acidemia (5-oxoprolinemia) is a rare cause of high anion gap metabolic acidosis that should be suspected in patients presenting with sepsis, hepatic, and/or renal dysfunction who are receiving drugs such as acetaminophen, flucloxacillin, and vigabatrin after the more common causes of a high anion gap acidosis have been excluded. Should pyroglutamic aciduria be present, known precipitants should be ceased, infection should be managed aggressively, and supportive management should be instituted.

Publication types

  • Case Reports

MeSH terms

  • Acidosis / etiology*
  • Acidosis / metabolism*
  • Aged
  • Aged, 80 and over
  • Female
  • Humans
  • Middle Aged
  • Pyrrolidonecarboxylic Acid / blood*


  • Pyrrolidonecarboxylic Acid