Patient Reference Age Gender Nutritional status/ comorbidities pH Anion Gap Lactate Urine 5-Oxoprolinea     (yrs)       (mEq/l) (mmol/l) (mmol/mmolCreatinine) 1 Creer et al [4] 52 F Albumin low, partial gastrectomy 7.17 27 2.2 15 2 Pitt et al [5] 34 F Vegetarian 7.17 N/A N/A 13 3 Pitt and Hauser [9] 33 F Pregnant 7.12 33 N/A 17 4 Pitt and Hauser [9] 54 F Alcohol abuse 7.23 26 N/A 13.8 5 Pitt and Hauser [9] 60 F Subtotal gastrectomy 7.14 39 N/A 11 6 Pitt and Hauser [9] 57 F Multiple abdominal surgeries 7.16 31 N/A 10 7 Pitt and Hauser [9] 17 F Spina bifida 7.38 31 N/A 20.4 8 Pitt and Hauser [9] 73 F   7.31 31 N/A 22 9 Pitt and Hauser [9] 84 M   7.15 21 N/A 23.6 10 Pitt and Hauser [9] 57 M Alcohol abuse 7.09 37 N/A 5.7 11 Dempsey et al [12] 80 F Chronic infection 7.27 35 0.5 Elevated 12 Dempsey et al [12] 60 F Gastrectomy 7.14 38 1.6 13.7 13 Dempsey et al [12] 64 F Lymphoma 6.8 33 1 4 14 Dempsey et al [12] 54 F Alcohol abuse 7.26 22 1.1 13.8 15 Humphreys et al [18] 41 F   N/A 29 Normal Elevated 17 Tailor et al [19] 40 F   7.24- 7.32 20-36 1.5-5.2 8.9-20.7 18 Foot et al [20] 57 F Kidney transplant 6.99 31 1.6 [370mm/L] 19 Fenves et al [10] 36 F Metastatic cancer N/A 47 N/A 7 20 Fenves et al [10] 46 F   6.88 33 N/A N/A 21 Fenves et al [10] 74 F Alcohol abuse 7.16 24 2.5 1 22 Fenves et al [10] 55 F Albumin low, poor oral intake 7.44 35 4.3 24.7 23 Kortmann et al [11] 72 F Albumin low, poor oral intake 7.12 30 0.7 16.6 24 Kortmann et al [11] 56 F Alcohol abuse 7.2 28 1.3 4.1 25 Kortmann et al [11] 79 F Chronic infection 7.29 29 1.6 N/A 26 Howie et al [17] 21 F Pregnant 7.26 25 1.6 4.9 27 Green et al [21] 43 F Eating disorder, opioid abuse 7.11 34 1.7 17.2 28 Duewell et al [22] 39 F Chronic infections 7.09 22 0.4 8.5 29 McGregor et al [3] 63 F Pancreatitis, poor oral intake 7.03 37 1.5 Elevated 30 McGregor et al [3] 83 F Alcohol abuse, poor oral intake 7.39 29 1 Elevated 31 Current report 62 M Crohn's disease, chronic infection 7.37 18 1.3 Elevated 32 Current report 62 M Alcohol abuse, poor oral intake 7.24 23 2.1 Elevated

aNormal reference range for urine 5-Oxoproline<0.1mmol/mmolCreatinine

Table 1: Patient demographics and biochemical data of 32 patients with 5-oxoproline-associated high anion gap matabolic acidosis.