Glycogenic Hepatopathy

Glycogenic hepatopathy is excessive intrahepatic glycogen accumulation. It is a rare complication of long-standing, poorly controlled type 1 diabetes mellitus. We report a case of a 19-year-old woman with a history of poorly controlled diabetes mellitus and frequent admissions for diabetic ketoacidosis, who presented with abdominal pain, nausea, vomiting, and hepatomegaly. She was found to have diabetic ketoacidosis with persistently elevated serum lactate that did not improve with insulin infusions. She eventually underwent a liver biopsy, which showed excessive intracytoplasmic glycogen accumulation consistent with glycogenic hepatopathy.