A Case of Mauriac Syndrome: A Teenage Girl With Poorly Controlled Diabetes.

Abstract

Mauriac syndrome is a rare complication of longstanding, poorly controlled type 1 diabetes in pediatric patients. Mauriac syndrome is characterized by hepatomegaly and growth retardation. This case report discusses a 14-year-old girl with persistent, poorly controlled type 1 diabetes mellitus (T1DM) admitted to the pediatric intensive care unit (PICU), where she was ultimately diagnosed with Mauriac syndrome. The patient presented with severe hypoglycemia and a history of multiple admissions for diabetes ketoacidosis (DKA) despite insulin therapy. The patient had a history of poor glycemic control and growth retardation, and on physical exam, she was found to have hepatomegaly. Based on clinical presentation, history of poorly controlled diabetes hepatomegaly, imaging, and laboratory findings, the diagnosis of Mauriac syndrome was made. Management of the patient included diabetes education, optimizing insulin therapy, nutritional support, and closely monitoring labs in a multi-disciplinary approach. The patient responded well to insulin therapy and was started on closed-loop insulin administration. Liver enzymes and hepatomegaly normalized, and growth parameters improved over the subsequent months. This case emphasizes the importance of early recognition, monitoring, and management of an extremely rare syndrome that is crucial in preventing the short-term complications of lactic acidosis and rapidly progressing retinopathyand the long-term complications of hepatic dysfunction and growth impairment.