Diagnosis of Mauriac Syndrome in Resource-Limited Settings

Abstract

Abstract Mauriac syndrome (MS) is a rare complication, seen in pediatric patients with uncontrolled type 1 diabetes mellitus (DM) characterized by growth failure and hepatomegaly and is associated with microvascular complications. We describe the case report of a 13-year-old girl with type 1 DM with MS due to inadequate insulin treatment with only short-acting insulin. She had growth failure, hepatomegaly with raised liver enzymes, neuropathy, very high HbA1c, and was in Tanner’s stage 1 sexual maturity. Her condition improved after switching to premixed insulin twice daily with additional short acting insulin before the lunch. Her enzymes normalized after 2 weeks of insulin intensification. Glycogen deposition in the liver due to uncontrolled DM is the cause of liver injury in MS. Thorough monitoring of growth and microvascular complications during each encounter with health-care personnel, along with tackling of social, mental, and emotional problems of children with type 1 DM is needed to prevent MS in resource-limited settings.