Re-emergence of a rare syndrome: Mauriac syndrome

Mauriac syndrome (MS) is one of the rare complications of poorly controlled Type 1 Diabetes Mellitus (T1DM). It is characterized by poor glycemic control, short stature hepatomegaly, Cushingoid features and delayed puberty [1]. Since the introduction and easy availability of longer acting insulin and insulin analogues, cases of Mauriac syndrome are hardly reported now [2]. This syndrome is more frequently encountered in children and adolescents with poor glycemic control and increases susceptibility of micro vascular complications of Diabetes like diabetic retinopathy and diabetic nephropathy. It is the most common cause of hepatic dysfunction in children and adolescents with Type 1 diabetes mellitus [3]. A classical case of Mauriac syndrome (MS) in an adolescent girl of uncontrolled Type 1 Diabetes Mellitus is being presented.