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Critical care medicine · Jun 2000
Case ReportsPyroglutamic acidemia: a cause of high anion gap metabolic acidosis.
- G A Dempsey, H J Lyall, C F Corke, and C D Scheinkestel.
- The Alfred Hospital, Prahran, Melbourne, Australia.
- Crit. Care Med. 2000 Jun 1;28(6):1803-7.
ObjectiveTo report four cases of pyroglutamic acidemia in adults causing clinically significant acidosis.Data SourcesPatients 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.ConclusionsPyroglutamic 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.
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