Mauriac Syndrome in a child with type 1 diabetes mellitus and inappropriate glycemic control
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Abstract
Objective: This article aims to alert about the importance of adequate glycemic control in the regression of Mauriac Syndrome. Case description: CJOS, 9 years and 7 months, female. At eight years-old, she was hospitalized many times due to diabetic ketoacidosis and hyperglycemia, one of them in November 2014 at Hospital Universitário Antônio Pedro (HUAP). She had delayed growth and weight gain, below 15th percentile, but with body mass index (BMI) near of 50th percentile. She also presented glycohemoglobin (HbA1c) of 13.3%, hypertriglyceridemia, hypercholesterolemia, increase in transaminases and inversion of albumin and globulin standard. After adequate therapy and being discharged, she was hospitalized again in 2015, for lack of adhesion to treatment and maintenance of uncontrolled glycaemia. Abdominal ultrasound showed diffuse hepatomegaly with regular contours. Laboratory tests showed similar changes in 2014. The patient was treated with intravenous insulinization and was discharged with outpatient treatment plan. Discussion: Mauriac Syndrome is a rare form of type 1 diabetes mellitus, one of the most important diseases in pediatric patients. It is characterized by the triad hepatomegaly, growth delay and long term poorly controlled diabetes. The clinical case is explained by the loss of glycemic control and the endocrine and metabolic changes, which were caused by hyperglycemia and insulin therapy. The diagnosis is clinical, and the mainstay of treatment is the hard glycemic control, which may lead to regression of the syndrome symptoms.
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References
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