Mauriac syndrome: A case report

Abstract

INTRODUCTION: Mauriac syndrome is an uncommon condition characterized by growth retardation, hepatomegaly and obesity in children with type 1 diabetes. CASE REPORT: We describe an 11 years old female patient with type 1 diabetes of 8 years duration treated with glargine and rapid-acting insulin. She presented gradually increased abdominal volume of approximately 3 months duration and poor glycemic control over 6 months. In addition, she exhibited polydipsia and polyuria. Physical examination revealed growth retardation, pubertal delay and hepatomegaly. Markers of viral or autoimmune hepatitis were negatives. Finally, liver biopsy reported abundant glycogen deposition. CONCLUSION: Although the Mauriac syndrome is an unusual condition, it should be suspected in patients with uncontrolled type 1 diabetes in order to establish a timely diagnosis and treatment.