Elevated Lactate Levels in Patients With Poorly Regulated Type 1 Diabetes and Glycogenic Hepatopathy: A New Feature of Mauriac Syndrome

Glycogenic hepatopathy is a rare but probably underdiagnosed feature of poorly controlled diabetes, in particular type 1 diabetes. It is characterized by excessive hepatic glycogen storage as first described in 1930 by Mauriac as a part of a syndrome comprising growth retardation, delayed puberty, and Cushingoid features in young patients with type 1 diabetes (1). Recent case reports have demonstrated that glycogenic hepatopathy can be the sole presenting feature of Mauriac syndrome (reviewed in ref. 2). Here, we describe four strikingly similar cases of young adults with poorly controlled type 1 diabetes who presented with excess hepatic glycogen storage and increased plasma lactate levels. The combination of these metabolic abnormalities led to the initial presumption …