SESSION TITLE: Student/Resident Critical Care SESSION TYPE: Student/Resident Case Report Slide PRESENTED ON: Tuesday, October 31, 2017 at 04:30 PM - 05:30 PM INTRODUCTION: Tissue hypoperfusion is the most common cause of lactic acidosis in critically ill patients. We present a rare case of persistent lactic acidosis in the absence of tissue hypoperfusion after resolution of diabetic ketoacidosis (DKA) in a patient with long standing uncontrolled diabetes mellitus type-1 (DM-1) due to glycogenic hepatopathy (Mauriac syndrome). CASE PRESENTATION: A 19-year-old male with uncontrolled DM-1 and recurrent DKA presented with abdominal pain, nausea and vomiting. Physical exam was normal except for mildly tender hepatomegaly. He was in DKA and was started on intravenous insulin in the intensive care unit. Lactic acid on presentation was 2.5 mmol/L, but increased to 7.5 mmol/L after resolution of DKA. Insulin drip was restarted and he was managed empirically for suspected sepsis. Abdominal ultrasonography demonstrated hepatomegaly with a liver span of 20 cm. Antibiotics were discontinued after a negative infectious work-up. However, the patient had persistent lactic acidosis. Liver enzymes, bilirubin, albumin and coagulation studies were normal. The patient was transitioned to subcutaneous insulin. He had documented hepatomegaly for 3 years. Hepatitis profile, thiamine level, alpha 1 antitrypsin, ceruloplasmin, Anti Nuclear Antibody, Extractable Nuclear Antigen antibody screen, anti-smooth muscle antibody, anti Liver Kidney Microsomal antibody, rheumatoid factor, anti-CCP antibody, anti-tissue transglutaminase were all negative. Liver biopsy showed edematous hepatocytes with glycogenated nuclei. Periodic Acid Schiff stain showed intense staining of glycogen in the hepatocytes with diastase pretreatment showing disappearance of glycogen. There was no steatosis, inflammation, fibrosis, necrosis or Mallory bodies. A diagnosis of glycogenic hepatopathy was made and the patient was sent home on insulin. DISCUSSION: Glycogenic hepatopathy is an under-recognized reversible cause of infiltrative hepatomegaly and lactic acidosis in patients with poorly controlled diabetes. The concomitant presence of persistent hyperglycemia and insulin leads to hepatic glycogen storage. Clinical features in adults include abdominal pain, nausea, vomiting, hepatomegaly, and lactic acidosis. Lactic acidosis is due to decrease in gluconeogenesis and failure to convert pyruvate into glucose and can be acutely worsened by high dose insulin. Liver biopsy is the definitive method of diagnosis. Rigorous glucose control reverses this condition. CONCLUSIONS: Awareness of glycogenic hepatopathy should help the clinician to not treat lactic acidosis based on numbers and expose patients to unnecessary sepsis treatment in the right clinical setting. Reference #1: Ross, J. J., et al. (2011). “Interactive medical case. A sweet source of abdominal pain.” N Engl J Med 364(15): e30 Reference #2: Deemer, K. S. and G. F. Alvarez (2016). “A Rare Case of Persistent Lactic Acidosis in the ICU: Glycogenic Hepatopathy and Mauriac Syndrome.” Case Rep Crit Care 2016: 6072909. DISCLOSURE: The following authors have nothing to disclose: Aditya Kotecha, Dongping Shi, Mohamed Siddique, Saraschandra Vallabhajosyula, Vivek Iyer, Geetha Krishnamoorthy No Product/Research Disclosure Information