Sodium–Glucose Cotransporter Inhibitors (SGLTi) are antidiabetic medications which act by inhibiting tubular glucose reabsorption. EDKA is a serious complication of their use. (HNFN1A)-MODY patients are characterized by reduced tubular glucose reabsorption. A single dose of dapagliflozin, an SGLT2i, has shown to induce higher glycosuria in (HNFN1A)-MODY compare to T2D patients.
A 51 years old female, diagnosed with (HNFN1A)-MODY at the age of 17, presented to the ER complaining of abdominal pain. She had HbA1c 7,6% and BMI=35,7 Kg/m2 (88 Kg) 45 days ago. Due to peripheral edema, her treatment changed from pioglitazone 45 mg/d, metformin 2000 mg/d and glimepiride 6 mg/d to dapagliflozin/metformin 10/2000/d and dulaglutide 1,5 mg/qw. During this period her SMBG was adequate, she lost 8 Kg and she experienced a remarkable loss of appetite.
On admission, she appeared dehydrated. Laboratory investigation revealed anion gap metabolic acidosis (pH: 6,9), Lact: 2,3 mmol/L, serious ketonuria, CRP: 0,2 mg/dL, blood glucose: 195 mg/dl and HbA1c 7%. She was diagnosed with EDKA and was hospitalized. After acid-base balance and dehydration were restored, and any infection or concomitant illness were excluded, she was discharged two days later, on treatment with insulin glargine 12 iu/d, metformin 2000mg/d and glimepiride 6mg/d. Two months later she continues with the same regimen (c-peptide: fasting 0.822 mg/dL and 6 min post-glucagon 1,38 mg/dL, HbA1c=6,9%).
We attributed this case of EDKA to dapagliflozine. Futhermore, the prone to ketogenesis diet with reduced calories might have played a precipitating role. Clinicians should use SGLT2i with great caution in (HNFN1A)-MODY patients.