Pyroglutamic acidemia: a cause of high anion gap metabolic acidosis.
OBJECTIVE
To report four cases of pyroglutamic acidemia in adults causing clinically significant acidosis.


METHODS
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.
Dempsey
G A
GA
The Alfred Hospital, Prahran, Melbourne, Australia.
Lyall
H J
HJ
Corke
C F
CF
Scheinkestel
C D
CD
D000138:Acidosis / Q000209:etiology* / Q000378:metabolism*; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D008875:Middle Aged; D011761:Pyrrolidonecarboxylic Acid / Q000097:blood*
D011761:Pyrrolidonecarboxylic Acid