HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

Recurrent high anion gap metabolic acidosis secondary to 5-oxoproline (pyroglutamic acid).

Abstract
High anion gap metabolic acidosis in adults is a severe metabolic disorder for which the primary organic acid usually is apparent by clinical history and standard laboratory testing. We report a case of recurrent high anion gap metabolic acidosis in a 48-year-old man who initially presented with anorexia and malaise. Physical examination was unrevealing. Arterial pH was 6.98, P co 2 was 5 mm Hg, and chemistry tests showed a bicarbonate level of 3 mEq/L (3 mmol/L), anion gap of 32 mEq/L (32 mmol/L), and a negative toxicology screen result, except for an acetaminophen (paracetamol) level of 7.5 mug/mL. Metabolic acidosis resolved with administration of intravenous fluids. Subsequently, he experienced 5 more episodes of high anion gap metabolic acidosis during an 8-month span. Methanol, ethylene glycol, acetone, ethanol, d -lactate, and hippuric acid screens were negative. Lactate levels were modestly elevated, and acetaminophen levels were elevated for 5 of 6 admissions. These episodes defied explanation until 3 urinary organic acid screens, obtained on separate admissions, showed striking elevations of 5-oxoproline levels. Inborn errors of metabolism in the gamma-glutamyl cycle causing recurrent 5-oxoprolinuria and high anion gap metabolic acidosis are rare, but well described in children. Recently, there have been several reports of apparent acquired 5-oxoprolinuria and high anion gap metabolic acidosis in adults in association with acetaminophen use. Acetaminophen may, in susceptible individuals, disrupt regulation of the gamma-glutamyl cycle and result in excessive 5-oxoproline production. Suspicion for 5-oxoproline-associated high anion gap metabolic acidosis should be entertained when the cause of high anion gap metabolic acidosis remains poorly defined, the anion gap cannot be explained reasonably by measured organic acids, and there is concomitant acetaminophen use.
AuthorsPrayus Tailor, Tuhina Raman, Cheryl L Garganta, Runa Njalsson, Katarina Carlsson, Ellinor Ristoff, Hugh B Carey
JournalAmerican journal of kidney diseases : the official journal of the National Kidney Foundation (Am J Kidney Dis) Vol. 46 Issue 1 Pg. e4-10 (Jul 2005) ISSN: 1523-6838 [Electronic] United States
PMID15983950 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Bicarbonates
  • Acetaminophen
  • Glutathione Synthase
  • Pyrrolidonecarboxylic Acid
Topics
  • Acetaminophen (adverse effects)
  • Acid-Base Equilibrium
  • Acidosis (chemically induced, drug therapy, etiology)
  • Amino Acid Metabolism, Inborn Errors (diagnosis, genetics, metabolism)
  • Anorexia (etiology)
  • Bicarbonates (blood, therapeutic use)
  • Dyspnea (etiology)
  • Fatigue (etiology)
  • Glutathione Synthase (blood)
  • Humans
  • Male
  • Middle Aged
  • Pyrrolidonecarboxylic Acid (urine)
  • Urinalysis (methods)

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research graph!


Choose Username:
Email:
Password:
Verify Password:
Enter Code Shown: