Hostname: page-component-78c5997874-lj6df Total loading time: 0 Render date: 2024-11-10T12:14:28.206Z Has data issue: false hasContentIssue false

Profound metabolic acidosis from pyroglutamic acidemia: an underappreciated cause of high anion gap metabolic acidosis

Published online by Cambridge University Press:  21 May 2015

Thomas J. Green*
Affiliation:
Department of Emergency Medicine, University of British Columbia, Vancouver, BC
Jan Jaap Bijlsma
Affiliation:
Department of Emergency Medicine, University of British Columbia, Vancouver, BC
David D. Sweet
Affiliation:
Department of Emergency Medicine, University of British Columbia, Vancouver, BC Department of Medicine, University of British Columbia, Vancouver, BC Division of Critical Care Medicine, Vancouver General Hospital, Vancouver, BC
*
Department of Emergency Medicine, Vancouver General Hospital, 910 West 10th Ave., ground floor, JPPN, Vancouver BC V5Z 4E3; tomgreen@interchange.ubc.ca

Abstract

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.

The workup of the emergency patient with a raised anion gap metabolic acidosis includes assessment of the components of “MUDPILES” (methanol; uremia; diabetic ketoacidosis; paraldehyde; isoniazid, iron or inborn errors of metabolism; lactic acid; ethylene glycol; salicylates). This approach is usually sufficient for the majority of cases in the emergency department; however, there are many other etiologies not addressed in this mnemonic. Organic acids including 5-oxoproline (pyroglutamic acid) are rare but important causes of anion gap metabolic acidosis. We present the case of a patient with profound metabolic acidosis with raised anion gap, due to pyroglutamic acid in the setting of malnutrition and chronic ingestion of acetaminophen.

Type
Case Report • Rapport de cas
Copyright
Copyright © Canadian Association of Emergency Physicians 2010

References

REFERENCES

1.Wellner, VP, Sekura, R, Meister, A, et al. Glutathione synthetase deficiency, an inborn error of metabolism involving the gamma-glutamyl cycle in patients with 5-oxoprolinuria (pyroglutamic aciduria). Proc Natl Acad Sci U S A 1974;71:2505–9.Google Scholar
2.Larsson, A, Mattsson, B, Wauters, EA, et al. 5-oxoprolinuria due to hereditary 5-oxoprolinase deficiency in two brothers — a new inborn error of the gamma-glutamyl cycle. Acta Paediatr Scand 1981;70:301–8.CrossRefGoogle Scholar
3.Roesel, RA, Hommes, FA, Samper, L. Pyroglutamic aciduria (5-oxoprolinuria) without glutathione synthetase deficiency and with decreased pyroglutamate hydrolase activity. J Inherit Metab Dis 1981;4:8990.Google Scholar
4.Creer, MH, Lau, BW, Jones, JD, et al. Pyroglutamic acidemia in an adult patient. Clin Chem 1989;35:684–6.Google Scholar
5.Fenves, AZ, Kirkpatrick, HM III, Patel, VV, et al. Increased anion gap metabolic acidosis as a result of 5-oxoproline (pyroglutamic acid): a role for acetaminophen. Clin J Am Soc Nephrol 2006;1:441–7.Google Scholar
6.Pitt, JJ, Hauser, S. Transient 5-oxoprolinuria and high anion gap metabolic acidosis: clinical and biochemical findings in eleven subjects. Clin Chem 1998;44:1497–503.CrossRefGoogle ScholarPubMed
7.Dempsey, GA, Lyall, HJ, Corke, CF, et al. Pyroglutamic acidemia: a cause of high anion gap metabolic acidosis. Crit Care Med 2000;28:1803–7.Google Scholar
8.Bonham, JR, Rattenbury, JM, Meeks, A, et al. Pyroglutamicaciduria from vigabatrin. Lancet 1989;1:1452–3.Google Scholar
9.Croal, BL, Glen, AC, Kelly, CJ, et al. Transient 5-oxoprolin-uria (pyroglutamic aciduria) with systemic acidosis in an adult receiving antibiotic therapy. Clin Chem 1998;44:336–40.Google Scholar
10.Peter, JV, Rogers, N, Murty, S, et al. An unusual cause of severe metabolic acidosis. Med J Aust 2006;185:223–5.CrossRefGoogle ScholarPubMed
11.Brooker, G, Jeffery, J, Nataraj, T, et al. High anion gap metabolic acidosis secondary to pyroglutamic aciduria (5-oxoprolinuria): association with prescription drugs and malnutrition. Ann Clin Biochem 2007;44:406–9.Google Scholar
12.Rolleman, EJ, Hoorn, EJ, Didden, P, et al. Guilty as charged: unmeasured urinary anions in a case of pyroglutamic acidosis. Neth J Med 2008;66:351–3.Google Scholar
13.Kortmann, W, van Agtmael, MIA, van Diessen, J, et al. 5-Oxoproline as a cause of high anion gap metabolic acidosis: an uncommon cause with common risk factors. Neth J Med 2008;66:354–7.Google Scholar
14.Foot, CL, Fraser, JF, Mullany, DV. Pyroglutamic acidosis in a renal transplant patient. Nephrol Dial Transplant 2005;20:2836–8.CrossRefGoogle Scholar
15.Hodgman, MJ, Horn, JF, Stork, CM, et al. Profound metabolic acidosis and oxoprolinuria in an adult. J Med Toxicol 2007;3:119–24.CrossRefGoogle ScholarPubMed
16.Humphreys, BD, Forman, JP, Zandi-Nejad, K, et al. Acetaminophen-induced anion gap metabolic acidosis and 5-oxoprolinuria (pyroglutamic aciduria) acquired in hospital. Am J Kidney Dis 2005;46:143–6.Google Scholar
17.Pitt, JJ, Brown, GK, Clift, V, et al. Atypical pyroglutamic aciduria: possible role of paracetamol. J Inherit Metab Dis 1990;13:755–6.Google Scholar
18.Tailor, P, Raman, T, Garganta, CL, et al. Recurrent high anion gap metabolic acidosis secondary to 5-oxoproline (pyroglutamic acid). Am J Kidney Dis 2005;46:e4–10.Google Scholar
19.Proudfoot, AT, Wright, N, Proudfoot, AT, et al. Acute paracetamol poisoning. BMJ 1970;3:557–8.Google Scholar
20.O’Grady, JG, Alexander, GJ, Hayllar, KM, et al. Early indicators of prognosis in fulminant hepatic failure. Gastroenterology 1989;97:439–45.Google Scholar
21.Zezulka, A, Wright, N, Zezulka, A, et al. Severe metabolic acidosis early in paracetamol poisoning. Br Med J (Clin Res Ed) 1982;285:851–2.Google Scholar
22.Ghauri, FY, McLean, AE, Beales, D, et al. Induction of 5-oxoprolinuria in the rat following chronic feeding with N-acetyl 4-aminophenol (paracetamol). Biochem Pharmacol 1993;46:953–7.Google Scholar
23.Petersen, C. D-lactic acidosis. Nutr Clin Pract 2005;20:634–45.Google Scholar