Hepatomegaly and Deranged Liver Enzymes in a Patient With Poorly Controlled Type 1 Diabetes Mellitus

Glycogenic hepatopathy (GH) is a rare clinical entity characterized by glycogen accumulation in the liver which affects a minority of patients with poorly controlled diabetes mellitus. Its cardinal manifestations include hepatomegaly and elevated liver enzymes. Reaching the diagnosis requires an extensive workup, and typically a liver biopsy. GH is completely reversible with the restoration of good glycemic control. Herein, we report the case of a 20-year-old woman with type 1 diabetes and poor glycemic control, who presented hepatomegaly and a steep increase in transaminase levels while she was being treated for diabetic ketoacidosis. The patient was submitted to a comprehensive laboratory and imaging workup to rule out other possible causes for her deranged liver function and, finally, to a liver biopsy that confirmed the diagnosis of GH. Following the appropriate modifications to her insulin regimen, her glycemic control markedly improved, as did her liver function tests on follow-up.