S2817 Glycopenic Hepatopathy in a Patient With Recurrent Diabetic Ketoacidosis: A Rare Complication of Poorly Controlled Type 1 Diabetes Mellitus

Abstract

Introduction: Glycogenic hepatopathy (GH) is a rare condition predominately seen in patients with poorly controlled type 1 diabetes mellitus. This condition develops due to excessive accumulation of glycogen in the hepatocytes, leading to significant hepatomegaly and transient liver dysfunction with elevated liver enzymes. Case Description/Methods: A 24-year-old female with a history of poorly controlled type 1 diabetes mellitus complicated by multiple admissions for diabetic ketoacidosis (DKA) presented with acute onset abdominal pain, nausea and vomiting. Physical exam was significant for tender hepatomegaly. Admission laboratory results were significant for serum glucose 189 mg/dL, bicarbonate 5 mmol/L, anion gap 35 mmol/L, beta-hydroxybutyrate 6.5 mmol/L, and HbA1c 11.4%. Admission hepatic panel was significant for alanine aminotransferase 31 U/L, aspartate aminotransferase 56 U/L, alkaline phosphatase 138 U/L with normal bilirubin and prothrombin time. Patient was treated with insulin and intravenous fluids with resolution of her DKA. However, her clinical course was complicated by persistent abdominal pain with an acute rise in liver enzymes (alanine aminotransferase 216 U/L, aspartate aminotransferase 249 U/L, alkaline phosphatase 116 U/L with normal bilirubin and prothrombin time). Triglyceride level was elevated at 214mg/dL. Abdominal computed tomography was notable for hepatomegaly with liver size of 24 cm. Workup for hepatitis, HIV, COVID-19, autoimmune hepatitis, hemochromatosis, and Wilson's disease were negative. Patient underwent liver biopsy that demonstrated diffuse hepatocyte cytoplasmic clearing, consistent with GH. The patient was medically managed with strict glycemic control, diabetic diet and endocrinology input throughout admission. One week after hospital discharge, the patient remained asymptomatic and liver function tests returned to normal limits. Discussion: GH is a rare condition that should be considered in patients with poorly controlled diabetes mellitus who present with hepatomegaly and acute liver injury. While GH presents similarly to NAFLD, it can only be differentiated and definitely diagnosed by liver biopsy. This condition should not be overlooked as it is a favorable diagnosis with a good prognosis compared to other liver disease associated with diabetes mellitus. Further studies are needed to better understand the characteristics of this disease, and future development of non-invasive, rapid diagnostic testing could help avoid extensive and costly workups. (Figure Presented).