High anion gap metabolic acidosis (HAGMA) is a common biochemical abnormality in hospitalized patients, often linked to conditions such as lactic acidosis, renal failure, or drug toxicity. A rare etiology, 5-oxoprolinuria, resulting from acetaminophen use, malnutrition, and sepsis, is increasingly recognized in critically ill patients. We report a 29-year-old male with a history of intellectual disability and normal baseline kidney function who was admitted with acute necrotizing pancreatitis and developed severe metabolic acidosis and acute kidney injury (AKI).
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