Unique presentation of Mauriac syndrome with associated celiac disease in an adolescent with type 1 diabetes mellitus: A case report

Abstract

We report an adolescent girl with Mauriac syndrome diagnosed in merely 3 years of diagnosis of type 1 diabetes mellitus with poor glycemic control and associated celiac disease. A 11-year-old girl with poor metabolic control characterized by recurrent hypoglycemia and hyperglycemia presented with Mauriac syndrome comprising stunting, hepatosplenomegaly, and elevated serum transaminases without cushingoid features, within 3 years of diagnosis of type 1 diabetes mellitus. Serum tissue transglutaminase antibodies were elevated. The girl also had coexisting celiac disease. She improved with adequate glycemic control along with a gluten-free diet, iron, and other supplements with regression in the size of the liver and reduction of liver enzymes. Mauriac syndrome should be considered in type 1 diabetic patients with poor metabolic control and hepatomegaly irrespective of the duration of diabetes mellitus. Recurrent hypoglycemia and the presence of Mauriac syndrome are signs of associated autoimmune disorders.