Pyroglutamic Acidosis - An Underrecognised Entity Associated with Acetaminophen Use

Wincy Wing-Sze Ng; Hok-Fung Tong; Wai-Yan Ng; Joshua Ka-Ho Yeung; Joyce Kit-Yu Young; Raymond Kam-Wing Woo; Maureen Mo-Lin Wong

doi:10.2478/rjaic-2023-0004

Pyroglutamic Acidosis - An Underrecognised Entity Associated with Acetaminophen Use

Rom J Anaesth Intensive Care. 2023 Apr 20;30(1):26-30. doi: 10.2478/rjaic-2023-0004. eCollection 2023 Apr.

Authors

Wincy Wing-Sze Ng¹, Hok-Fung Tong², Wai-Yan Ng², Joshua Ka-Ho Yeung³, Joyce Kit-Yu Young³, Raymond Kam-Wing Woo³, Maureen Mo-Lin Wong³

Affiliations

¹ Department of Intensive Care, Pamela Youde Nethersole Eastern Hospital, 3 Lok Man Road, Chai Wan, Hong Kong Special Administrative Region, China.
² Department of Pathology, Princess Margaret Hospital, 2-10 Princess Margaret Hospital Road, Lai Chi Kok, Hong Kong Special Administrative Region, Kowloon, China.
³ Department of Medicine & Geriatrics/ICU, Caritas Medical Centre, 111 Wing Hong Street, Sham Shui Po, Hong Kong Special Administrative Region, Kowloon, China.

Abstract

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.

Keywords: 5-oxoprolinuria; acetaminophen; paracetamol; pұroglutamic acidosis; voriconazole.