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Open AccessArticle
Drug-Related Pyroglutamic Acidosis: Systematic Literature Review
by
Tessa Scafetta
Tessa Scafetta 1,†,
Orsolya Kovacs
Orsolya Kovacs 1,2,†,
Gregorio P. Milani
Gregorio P. Milani 3,4,
Gabriel Bronz
Gabriel Bronz 1,
Sebastiano A. G. Lava
Sebastiano A. G. Lava 5,6,
Céline Betti
Céline Betti 7,
Federica Vanoni
Federica Vanoni 8,9,
Mario G. Bianchetti
Mario G. Bianchetti 1,*,
Pietro B. Faré
Pietro B. Faré 1,‡ and
Pietro Camozzi
Pietro Camozzi 2,‡
1
Family Medicine Institue, Faculty of Biomedical Sciences, Università della Svizzera Italiana, 6900 Lugano, Switzerland
2
Department of Anesthesia, Hôpital du Valais, 1951 Sion, Switzerland
3
Pediatric Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy
4
Department of Clinical Sciences and Community Health, Università degli Studi di Milano, 20122 Milan, Italy
5
Pediatric Cardiology Unit, Department of Pediatrics, Centre Hospitalier Universitaire Vaudois, University of Lausanne, 1011 Lausanne, Switzerland
6
Clinical Pharmacology Service, Centre Hospitalier Universitaire Vaudois, University of Lausanne, 1011 Lausanne, Switzerland
7
Pediatric Emergency Department, University Children’s Hospital Zurich, 8032 Zurich, Switzerland
8
Pediatric Institute of Southern Switzerland, Ente Ospedaliero Cantonale, 6500 Bellinzona, Switzerland
9
Faculty of Biomedical Sciences, Università Della Svizzera Italiana, 6900 Lugano, Switzerland
*
Author to whom correspondence should be addressed.
†
These authors contributed equally to this work.
‡
These authors also contributed equally to this work.
J. Clin. Med. 2024, 13(19), 5781; https://doi.org/10.3390/jcm13195781 (registering DOI)
Submission received: 17 August 2024
/
Revised: 13 September 2024
/
Accepted: 17 September 2024
/
Published: 27 September 2024
Abstract
Background: 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. Methods: 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. Results: A total of 131 cases were found. Most patients were females (79%), adults (92%) aged 51 years or older (66%) with pre-existing conditions (74%) such as undernutrition, alcohol-use disorder, or kidney disease, and had an ongoing infection (69%). The clinical features included diminished consciousness (60%), Kussmaul breathing (56%), and nausea or vomiting (27%). At least 92% of patients were on paracetamol therapy for >10 days at an appropriate dose, 32% on a β-lactamase-resistant penicillin, and 2.3% on vigabatrin. Besides severe anion gap acidosis, patients also presented with hypokalemia (24%) and kidney function deterioration (41%). Management involved discontinuing the offending drug (100%), bicarbonate (63%), acetylcysteine (42%), and acute kidney replacement therapy (18%). The fatality rate was 18%, which was higher without acetylcysteine (24%) compared to with it (11%). Conclusions: Acquired pyroglutamic acidosis is a rare, potentially fatal metabolic derangement, which usually occurs after paracetamol use, frequently combined with a β-lactamase-resistant penicillin or vigabatrin. This condition predominantly affects adults, especially women with factors like undernutrition, alcohol-use disorder, or kidney disease, often during infection. Increased awareness of this rare condition is necessary.
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MDPI and ACS Style
Scafetta, T.; Kovacs, O.; Milani, G.P.; Bronz, G.; Lava, S.A.G.; Betti, C.; Vanoni, F.; Bianchetti, M.G.; Faré, P.B.; Camozzi, P.
Drug-Related Pyroglutamic Acidosis: Systematic Literature Review. J. Clin. Med. 2024, 13, 5781.
https://doi.org/10.3390/jcm13195781
AMA Style
Scafetta T, Kovacs O, Milani GP, Bronz G, Lava SAG, Betti C, Vanoni F, Bianchetti MG, Faré PB, Camozzi P.
Drug-Related Pyroglutamic Acidosis: Systematic Literature Review. Journal of Clinical Medicine. 2024; 13(19):5781.
https://doi.org/10.3390/jcm13195781
Chicago/Turabian Style
Scafetta, Tessa, Orsolya Kovacs, Gregorio P. Milani, Gabriel Bronz, Sebastiano A. G. Lava, Céline Betti, Federica Vanoni, Mario G. Bianchetti, Pietro B. Faré, and Pietro Camozzi.
2024. "Drug-Related Pyroglutamic Acidosis: Systematic Literature Review" Journal of Clinical Medicine 13, no. 19: 5781.
https://doi.org/10.3390/jcm13195781
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