Mauriac Syndrome: Case Report and Review of the Literature

Topaloglu O., Sezer S. D., Demir B., Akarken D.

TURKISH JOURNAL OF ENDOCRINOLOGY AND METABOLISM, vol.21, no.4, pp.131-135, 2017 (ESCI) identifier identifier


This is a case report of a young male with poorly controlled type 1 diabetes mellitus who presented with the clinical features of diabetic ketoacidosis. Once the patient was stabilized, he was examined for hepatomegaly and elevated liver enzymes. Along with the other clinical features, the patient was diagnosed as a case of Mauriac Syndrome. Mauriac Syndrome, initially described by Mauriac in 1930, is one of the causes of hepatomegaly and elevated liver enzymes in poorly controlled diabetic patients. However, hepatomegaly, growth retardation and other clinical features of the syndrome have been found to be reversible with optimization of insulin therapy. In patients with poorly controlled diabetes, Type 1 diabetic patients must be closely observed for sexual maturation and growth. After optimal therapy has been given, close follow-up is essential to observe the regression of clinical features.