Mauriac Syndrome; A Rare Complication of T1 DM

Mauriac syndrome (MS) is characterized by excessive intrahepatic glycogen deposition, attributed to severe hyperglycemia which diffuses by insulin-independent GLUT 2 to be trapped inside hepatocytes as G -6 phosphate. It can rarely occur in poorly controlled T1 diabetic adolescents and is complicated by hepatomegaly, short stature, delayed puberty and hypertriglyceridemia