The treatment of children with type 1 diabetes mellitus has improved dramatically over the last few decades. The maintenance of acceptable metabolic control, nevertheless, remains challenging because the success of treatment is so dependent on patient compliance. Children with type 1 diabetes and poor metabolic control are at risk of developing Mauriac syndrome, a condition characterised by hepatomegaly, growth retardation and cushingoid features. A similar complication may occur in type-1 or type-2 adult diabetics; namely, glycogenic hepatopathy. We describe two children, a 12-year-old girl and a 16-year-old boy, who presented with classic symptoms of Mauriac syndrome. After metabolic control was achieved, reduction of hepatomegaly and the disappearance of cushingoid features were observed, proving the reversibility of the syndrome. Awareness that this syndrome still exists despite improved insulin therapy is crucial for earlier recognition and treatment.
Nederlands Tijdschrift voor Geneeskunde
Erasmus MC: University Medical Center Rotterdam

Schmetz, A. V. N., Dekker-Maas, M. H., Den Breejen, M. P., & Sas, T. (2012). [Mauriac syndrome--a rare complication of type 1 diabetes mellitus]. Nederlands Tijdschrift voor Geneeskunde, 156(28). Retrieved from