Glycogenic Hepatopathy: Persistent Lactic Acidosis in Poorly Controlled Type 1 Diabetic

Abstract

Mauriac syndrome is a rare complication of poorly controlled type 1 diabetes mellitus and remains underdiagnosed. Hepatomegaly is a typical sign and appears in most patients. The clinical signs considered typical of this syndrome (short stature, obesity, and hepatomegaly) are often incomplete, and lactic acidosis can be exacerbated by high-dose insulin and glucose therapy, as occurs during the treatment of diabetic ketoacidosis. Glycogen storage liver disease must be differentiated from metabolic dysfunction-associated steatotic liver disease as a cause of hepatomegaly and liver function abnormalities in patients with type 1 diabetes mellitus, as these conditions require different therapeutic approaches and have distinct prognoses. The authors describe a clinical case of persistent lactic acidosis during a diabetic ketoacidosis episode in a woman with poorly controlled type 1 diabetes, hepatomegaly, and glycogen storage liver disease, which was diagnosed through liver biopsy.