EC Gastroenterology and Digestive System

The Mauriac syndrome is a rare complication of poorly controlled diabetes mellitus in adolescence. We report the case of a 17-year-old patient with type 1 diabetes who got admitted for severe hyperglycemic imbalance. The clinical examination showed failure to thrive, hepatomegaly. Biological analyses showed hyperglycaemia, major cytolysis, and anicteric cholestasis. The management was based on insulin and fluid therapy. The etiological investigation of the liver dysfunctions was negative. Clinical and biological evolution was favorable. The diagnosis of hepatic glycogenesis was retained based on a bundle of anamnestic and clinical arguments and the absence of other abnormalities causing by hepatic disorders. Blood sugar was very high. Blood transaminases were also high. Abdominal ultrasound showed homogeneous hepatomegaly. Viral hepatitis serology, immunological tests were negative, liver biopsy was in favor of hepatocyte ballooning. Given the favorable evolution under intensive insulin therapy, the diagnosis of Mauriac syndrome was retained.

Keywords: Mauriac Syndrome; Hepatic Glycogenosis; Diabetes Mellitus; Failure to Thrive; Hepatomegaly

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