MON-133 Exacerbation of Lactic Acidosis During DKA in Adolescents with Type 1 Diabetes Mellitus with Mauriac Syndrome

Abstract

Background: Glycogenic hepatopathy (GH: deposition of glycogen in the liver) and lactic acidosis (LA) in poorly controlled type 1 diabetes (T1D) can be the only presentation of Mauriac syndrome (hypercholesterolemia, growth retardation, delayed puberty, and cushingoid features). LA in the background of DKA is the result of poor tissue perfusion due to dehydration and hypophosphatemia. Intensive insulin therapy during management of DKA inhibits glycogenolysis and gluconeogenesis leading to the anaerobic conversion of pyruvate to lactate, thereby worsening the LA. Case series: Patient #1 had ten DKA admissions and had LA in two admissions nine months apart [mean + SD-age: 12.5+/-0.7 years; BMI-28.7+/-0.33 kg/m2, HbA1c:10.5 +/- 0.4%; duration of diabetes: 6.5+/-0.7 years]. Patient #2 had five DKA admissions (age:14 years; BMI: 23.7 kg/m2, HbA1c: 11.7; duration of diabetes: 6 years). Clinical and biochemical characteristics:- initial presentation- pH:7.11+/- 0.06; HCO3: 6.5+/- 0.6 mmol/l, glucose: 807+/-50.4 mg/dl, phos: 5.3+/-0.4, lactate: 3.11+/-1.33 with hepatomegaly on exam. DKA resolved in 27.6 +/- 2.5 hours. Lactate levels peaked to 7.8+/-0.3mmol/l at 13+/-11.5 hours after resolution of DKA and the anion gap at the time of the peak was 13.6 +/- 7.5mmol/l. Liver enzymes were within normal limits on admission and after [AST-21.6 +/-4.04; (5-34 U/L); ALT- 23.6 +/- 9.6 (0-55 U/L)]. Liver ultrasound showed hepatomegaly (18.65 +/-2.33 cm; Normal:8.5-14 cm) suggestive of glycogen versus fat deposition. Basal bolus insulin was continued and the lactate levels declined in 29+/-6.02 hours after the peak to 2.7 +/-1.18mmol/l at the time of discharge. Since the liver enzymes were normal and the LA was improving, no liver biopsy was performed in either of our patients. Conclusions: The lactate levels were high on admission and the levels continued to rise during and after resolution of the DKAin both the patients. Since the patients have a history of poorly controlled diabetes and had hepatomegaly on ultrasound, GH was considered the most likely cause of the hepatomegaly. Our cases illustrate the following points: a. hepatomegaly in poorly controlled T1DM is likely GH. b. elevated lactate levels favor GH as elevated lactate levels are not seen in steatohepatitis, the caveat being that ultrasound cannot discriminate between the two conditions c. DKA can unmask this lactic acidemia which can be present in half of the patients with Mauriac’s syndrome d. liver biopsy is the gold standard for diagnosis of GH though gradient-dual-echo liver MRI can be non-invasive alternative for diagnosis of GH.