MON-LB126 A Benign and Favorable Diagnosis: Glycogen Hepatopathy Causing Transient Transaminitis During Diabetic Ketoacidosis in Type 1 Diabetes Mellitus

Abstract

Background: Transient transaminitis is a rarely discussed complication of uncontrolled diabetes mellitus (DM). Known as glycogenic hepatopathy (GH), it is belived to be caused by build-up of glycogen in hepatocytes. Recognized as benign and reversible, GH is associated with hepatomegaly (>90% cases) and primarily seen in patients with type 1 DM during periods of inadequate hyperglycemic control. Differential diagnoses include glycogen storage diseases, nonalcoholic fatty liver disease, hepatosclerosis, autoimmune hepatitis, hemochromatosis, Wilson disease, and acute viral hepatitis.1 Case Report: A 26-year-old African American female with type 1 DM and sickle cell presented on multiple occasions to the emergency department with abdominal pain associated with nausea, vomiting and diarrhea. Initial labs consistently included glucose levels >600 mg/dL (70-105 mg/dL), elevated anion gap ranging 20-40s mEq/L (5-15 mEq), and severe metabolic acidosis reflective of diabetic ketoacidosis (DKA). Labs were also significant for repeated mild transaminitis despite adequate fluid hydration.After several admissions, we observed a distinct pattern of mild transaminitis that directly fluctuated with her levels of blood glucose. With some minor lag, the patient’s liver enzymes normalized when her glucose levels normalized and DKA resolved.Further work-up ruled out more common etiologies of liver injury. Multiple abdominal ultrasounds and CT scans showed a normal sized liver without obvious structural abnormalities. Labs were significant for negative hepatitis B and hepatitis C; several negative anti-smooth muscle, anti-nuclear antibody, centromere antibody, and liver kidney microsomal type 1 antibody; normal levels of ceruloplasmin and alpha 1 anti-trypsin; low iron levels 23 ug/dL (60-180 ug/dL); borderline low IgG 627 mg/dL (700-1600 mg/dL).We hypothesized that the patient likely had GH by exclusion of other liver pathologies and given the context of transient transaminitis during DKA. Conclusion: GH is a benign and favorable diagnosis in diabetic patients with elevated transaminases.1 Given the small number of cases of GH reported, there is a need to record and analyze more patients with likely GH in order to better understand the condition. Appropriate clinician awareness of GH can also eliminate the need for time consuming and costly workup.