Resumen

CONSTANTINO, Cláudia et al. Mauriac Syndrome, Uncommon Presentation of a Common Disease. Nascer e Crescer [online]. 2010, vol.19, n.4, pp.278-281. ISSN 0872-0754.

Introduction: Type 1 diabetes mel­litus is a chronic metabolic disease whith increasing incidence. It has different kinds of manifestations, such as systemic disturbances like hepatomegaly and dwarfism, caused by poor glycemic control. Case presentation:A 14 yea-old girl with type 1 diabetes mellitus since she was three, with a poor glycemic control, admitted in an Intensive care Unit for severe ketoacidosis. On examination she had short stature, a non painful hepatomegaly and Tanner stage P1 M2. Laboratory data showed increased transaminases, cholesterol and tryglicerides. Discussion: Mauriac syndrome is characterized by poorly controlled type 1 diabetes mellitus, growth failure, delayed puberty, hypercholesterolemia, high levels of transaminases and hepatomegaly due to hepatic glycogen storage. The physiological mechanism is not completely understood, and this is due to a probably combination of various etio­logical factors. It is a rare clinical entity. The diagnosis, that is mostly clinical based, is very important because this is a revers­ible condition with optimization of insulin therapy.

Palabras clave : Type 1 Diabetes mellitus; Mauriac Syndrome; hepatomegaly; delayed puberty.

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