[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.

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