Pyroglutamic acidosis (PGA) is an underrecognized entity characterised by raised anion gap metabolic acidosis (RAGMA) and urinary hyper-excretion of pyroglutamic acid. It is frequently associated with chronic acetaminophen (APAP) ingestion. We report the case of a 73-year-old man with invasive pulmonary aspergillosis treated with voriconazole and APAP for analgesia with a cumulative dose of 160 g over 40 days. PGA was suspected as he developed severe RAGMA and common causes were excluded. Diagnosis was confirmed via urinary organic acid analysis which showed significant hyper-excretion of pyroglutamic acid. APAP was discontinued, and N-acetylcysteine (NAC) was administered. His RAGMA rapidly resolved following treatment.
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http://dx.doi.org/10.2478/rjaic-2023-0004 | DOI Listing |
Cureus
December 2024
Nephrology, NewYork-Presbyterian Queens, New York, USA.
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).
View Article and Find Full Text PDFA 60-year-old polymorbid patient developed severe metabolic acidosis during weeks of treatment with flucloxacillin and paracetamol. The acidosis was triggered by an accumulation of 5-oxoproline due to pre-existing risk factors for glutathione deficiency as well as a ketoacidosis due to starvation. After treatment with N-acetylcysteine and hemofiltration, the acidosis completely resolved.
View Article and Find Full Text PDFJ Clin Med
September 2024
Department of Anesthesia, Hôpital du Valais, 1951 Sion, Switzerland.
: Inborn errors of glutathione metabolism may cause high anion gap metabolic acidosis due to pyroglutamic acid accumulation. Since 1988, cases of this acidosis have been reported in individuals without these defects. : Given the poorly characterized predisposing factors, presentation, management, and prognosis of acquired pyroglutamic acidosis, we conducted a systematic review using the National Library of Medicine, Excerpta Medica, Web of Science, and Google Scholar databases.
View Article and Find Full Text PDFClin Genet
December 2024
Service de Génétique médicale, Nantes Université, CHU de Nantes, Nantes, France.
BMC Nephrol
August 2024
Department of Nephrology, Dialysis, and Transplantation, Bicêtre University Hospital, APHP, Paris Saclay University, 78 rue du Général Leclerc, Le Kremlin-Bicêtre, 94270, France.
Background: Pyroglutamic acidosis is a rare cause of high anion gap metabolic acidosis. Most cases of paracetamol related pyroglutamic acidosis are described in malnourished women and patients with kidney/liver failure, alcohol use or severe sepsis. In this report, we describe how pyroglutamic acidosis could be related to the use of chronic therapeutic paracetamol with only malnutrition as an associated risk factor.
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