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Pyroglutamic acidosis

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  • uncommon
  • high anion gap metabolic acidosis
  • associated with glutathione depletion
    • glutathione depletion results in abolition of feedback inhibition of gamma glutamylcysteine synthetase and increased production of gamma glutamylcysteine. Excess gamma glutamylcysteine is converted to pyroglutamic acid
    • glutathione depletion may occur in sepsis or due to paracetamol (acetaminophen) ingestion
  • inhibition of 5 oxoprolinase by flucloxacillin may also precipitate acidosis as may vigabatrin
  • may be congenital

  • consider diagnosis in patients with high anion gap acidosis in whom more common causes have been excluded. Measure urinary organic acids to demonstrate presence of pyroglutamic acid
  • use of N-acetylcysteine to replete glutathione should be considered
 

©Charles Gomersall, April, 2014 unless otherwise stated. The author, editor and The Chinese University of Hong Kong take no responsibility for any adverse event resulting from the use of this webpage.
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