Mauriac syndrome or hepatic glycogenosis: A rare complication of unbalanced diabetes (about two clinical cases)

Abstract

Unbalanced diabetes can lead to many complications related to insulin deficiency. Hepatocytic glycogenic overload, formerly known as Mauriac syndrome, is one of them. It is a rare syndrome initially described in children with type 1 diabetes (T1D) who have a history of high blood glucose followed by high doses of insulin. It is exceptionally described among adults. We report two cases of Mauriac syndrome diagnosed in two DT1 patients, the first admitted for abdominal distension with a subictus and the second admitted for abdominal distension with a failure to thrive. The clinical examination objectified a distended abdomen with hepatomegaly in both patients, subictus in the 1st and a very noticeable growth failure in the 2nd patient. Biological tests showed hyperglycemia, hepatic cytolysis in the first patient with a negative etiological work-up. The diagnosis of hepatic glycogenosis was retained in both patients on a bundle of anamnestic and clinical arguments, and this in the absence of other abnormalities responsible for liver disturbances in the 1st patient. The diagnosis of certainty is histological, and treatment is based on diabetes control.

Keywords

• Mauriac syndrome
• Hepatic glycogenosis
• Hepatomegaly
• Type 1 diabetes