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Anion Gap Metabolic Acidosis Secondary to 5-Oxopro ...
Anion Gap Metabolic Acidosis Secondary to 5-Oxoproline and Starvation Ketoacidosis
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This case describes a 44-year-old man hospitalized for septic arthritis and severe diarrhea who developed worsening anion gap metabolic acidosis (AGMA). He received acetaminophen throughout admission, first as needed and later at 4 g/day, while also having poor oral intake. His anion gap was elevated starting on hospital day 8. Initially, glucose remained normal and beta-hydroxybutyrate (BHB) was normal, suggesting that ketosis was not yet the main driver.<br /><br />By hospital day 16, BHB had risen to 2.2 mmol/L despite therapeutic acetaminophen levels, and acetaminophen was stopped. The next day, the patient became acutely altered, with severe acidosis: anion gap 28, bicarbonate 5 mEq/L, pH 7.18, and BHB 8.7 mmol/L. A urine organic acid screen later revealed markedly elevated pyroglutamic acid (5-oxoproline), confirming 5-oxoproline acidosis. He was treated with fluids, dextrose, sodium bicarbonate, N-acetylcysteine, and thiamine, and his labs normalized within 24 hours.<br /><br />The authors emphasize that AGMA is often multifactorial and requires systematic evaluation. In this case, the acidosis had two phases: first, 5-oxoproline (pyroglutamic) acidosis from chronic therapeutic acetaminophen use, and later starvation ketoacidosis due to poor intake, reflected by rising BHB. 5-oxoproline acidosis is underrecognized, difficult to test for promptly, and usually occurs in sick patients taking normal doses of acetaminophen rather than in overdose. Because urine organic acid testing takes days, high suspicion and empiric treatment are important.<br /><br />The report highlights the usefulness of the GOLD MARK mnemonic for AGMA evaluation, which includes both oxoproline and ketoacidosis, unlike the older MUDPILES mnemonic.
Asset Subtitle
Jess Hatfield
Meta Tag
Author List
Jess Hatfield, Kadilee Adams, Michael McNeal, Nickolas Underhill
Category
Clinical Vignettes
Concept
Anion Gap Metabolic Acidosis
Concept
Pyroglutamic Acidosis
Concept
GOLD MARK
Concept
Starvation Ketoacidosis
Concept
N-Acetylcysteine
Distinguished
Non-Finalist
Presenter Organization
Texas A&M School of Medicine
Presenting Author
Jess Hatfield
Track
Adult
Keywords
anion gap metabolic acidosis
5-oxoproline acidosis
pyroglutamic acidosis
acetaminophen
starvation ketoacidosis
beta-hydroxybutyrate
N-acetylcysteine
urine organic acids
GOLD MARK mnemonic
septic arthritis
Anion Gap Metabolic Acidosis
Pyroglutamic Acidosis
GOLD MARK
Starvation Ketoacidosis
N-Acetylcysteine
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