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Published on: May 6, 2013
[Mauriac syndrome--a rare complication of type 1 diabetes mellitus]
Anne V N Schmetz1, Mariëlle H Dekker-Maas, Marianne P den Breejen
1Sophia Kinderziekenhuis, afd. IC kinderen, Erasmus MC, Rotterdam, the Netherlands. anne_schmetz@hotmail.com
Insights
Mauriac syndrome, a complication of type 1 diabetes in children, is characterized by enlarged liver and growth issues. Improved metabolic control can reverse these symptoms, highlighting the importance of patient compliance in diabetes management.
Area of Science:
- Pediatric Endocrinology
- Metabolic Disorders
Background:
- Type 1 diabetes mellitus treatment has advanced, yet metabolic control remains challenging due to patient compliance.
- Poor metabolic control in children with type 1 diabetes increases the risk of Mauriac syndrome.
Observation:
- Two pediatric patients presented with classic Mauriac syndrome symptoms: hepatomegaly, growth retardation, and cushingoid features.
- Mauriac syndrome is a rare complication, presenting similarly to glycogenic hepatopathy in adults.
Findings:
- Achieving acceptable metabolic control led to the reduction of hepatomegaly and resolution of cushingoid features.
- These findings demonstrate the reversibility of Mauriac syndrome with effective diabetes management.
Implications:
- Increased awareness of Mauriac syndrome is vital for early diagnosis and intervention, even with modern insulin therapies.
- Emphasizes the critical role of patient adherence in managing type 1 diabetes and preventing its complications.
Abstract:
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.
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