Pyroglutamic acidosis as a cause for high anion gap metabolic acidosis: a prospective study

Itai Gueta,Eias Massalha,Eldar Priel,Ronen Loebstein,Yuval Shafir,Shir Raibman Spector,Noa Markovits,Haim Mayan

doi:10.1038/s41598-019-39257-4

Itai Gueta, Eias Massalha + Show 6 more

Open Access

https://doi.org/10.1038/s41598-019-39257-4

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Abstract

5-oxoprolinemia (pyroglutamic acid, PGA) in the absence of acetaminophen use has been rarely reported as a cause for high anion gap metabolic acidosis. We investigated the prevalence and risk factors for elevated PGA concentrations among hospitalized patients with high anion gap metabolic acidosis: We prospectively enrolled patients with high anion gap metabolic acidosis hospitalized in the department of medicine. For each patient we collected the main diagnosis, concurrent medications and laboratory parameters. Spot urine samples were tested for PGA concentration. Levels ≥63 µmol/mmol creatinine were considered elevated. Overall, forty patients were prospectively followed. Mean age was 66.9 (17.9) years. Four (6.3%) patients had a high urine PGA level and demonstrated also lower blood pH (7.2 vs 7.3, p = 0.05) and lower serum lactate concentration (17.5 mg/dl vs 23.0 mg/dl, p = 0.04). Additionally, the high PGA level group consisted of more patients with septic shock [2/4 (50%) vs 3/36 (8.3%)] with a trend towards significance (p = 0.07). In conclusion, PGA might have a role in patients with septic shock and acidosis. Being a treatable condition, PGA should be taken into consideration particularly when no other cause for high anion gap is identified.

Highlights

5-oxoprolinemia in the absence of acetaminophen use has been rarely reported as a cause for high anion gap metabolic acidosis
Elevated pyroglutamic acid (PGA) concentration is a known cause for high anion gap metabolic acidosis
Glutathione depletion states secondary to conditions in which metabolic stress is prominent has been associated with increased PGA levels

Summary

Introduction

5-oxoprolinemia (pyroglutamic acid, PGA) in the absence of acetaminophen use has been rarely reported as a cause for high anion gap metabolic acidosis. Four (6.3%) patients had a high urine PGA level and demonstrated lower blood pH (7.2 vs 7.3, p = 0.05) and lower serum lactate concentration (17.5 mg/dl vs 23.0 mg/dl, p = 0.04). High anion gap metabolic acidosis (HAGMA) is a common acid-base disturbance encountered in hospitalized patients. In cases where no other explanation is found, less common etiologies such as accumulation of D-lactate or pyroglutamic acid (PGA) should be suspected[1,2] The latter has been mostly reported in the presence of chronic acetaminophen use. To our department a 77-year-old female was admitted due to reduced level of consciousness, polyuria and fever of 38.8 °C Her past medical history was significant for osteoporosis and a recent hip replacement with a complicated course that required its removal due to Pseudomonas MDR infection.

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