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Archivos de Pediatría del Uruguay

versão On-line ISSN 1688-1249


MACHADO, Karina; PEREZ, María del Luján  e  MONTANO, Alicia. Síndrome de Mauriac: A propósito de un caso clínico. Arch. Pediatr. Urug. [online]. 2013, vol.84, n.3, pp.215-220. ISSN 1688-1249.

Type 1 diabetes mellitus (T1DM) is the most common chronic endocrinology disease in pediatric and probably the greatest health impact by the severity of its complications. The treatment, based on insulin, food and exercise, search the glycemic control, ensuring the prevention of complications and better quality of life. Education is the cornerstone in the care of diabetic patients and the key to successful treatment. The ultimate expression of poorly controlled T1DM is the Mauriac syndrome, integrated by short stature, delayed puberty, hepatomegaly, dyslipidemia and cushingoid phenotype. This syndrome is the most common cause of liver dysfunction in children and adolescents with T1DM and may revert optimizing treatment. We present a case of adolescent 14 years old with 8 years of evolution of T1DM. She had poor metabolic control, with failures in all pillars of diabetologic treatment. She showed failure of growth and pubertal development, hepatomegaly, cushingoid habit and dyslipidemia, which configured Mauriac syndrome in its full expression. These signs are due to sustained hyperglycemia, insulin deficiency and activation of counterregulatory hormones. The diagnosis of this syndrome is clinical. The treatment is based on adequate diabetológic treatment, which leads to good metabolic control. All symptoms and signs reverted achieving glycemic control. If this goal is not achieved, the prognosis depends on the medium and long term complications


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