Het syndroom van Mauriac

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.

Conflict of interest: M.P. den Breejen received compensation for educating and conference visits from Bosman, Medtronic, Mediq, Lilly and Novo Nordisk. M.H. Dekker-Maas received compensation from Teva for lecturing. T.C.J. Sas received compensation for lecturing, authoring and conference visits from Novo Nordisk, Pfizer and Lilly. Financial support for this article: none declared.