To outline the complexities in managing adolescent patients with type 1 diabetes and the management strategies for Mauriac syndrome.
Approach:
Patient Presentation: A 14-year-old female with a six-year history of type 1 diabetes presented with abdominal pain and vomiting, demonstrating poor adherence to insulin therapy and blood glucose monitoring.
Diagnostic Evaluation: Diagnostic tests revealed diabetic ketoacidosis (DKA), hepatomegaly, and abnormal liver function, leading to a diagnosis of Mauriac syndrome confirmed by liver biopsy.
Treatment Protocol: The patient received continuous intravenous insulin therapy, diabetes education, dietary guidance, and later transitioned to an insulin pump and then a four-injection regimen.
Key Findings:
Recurrent episodes of DKA and abnormal liver function were observed.
Liver biopsy confirmed glycogenic hepatopathy.
Whole-exome sequencing identified a homozygous HFE mutation.
Interpretation:
Mauriac syndrome is a rare but significant complication of poorly managed type 1 diabetes, necessitating careful monitoring and comprehensive treatment strategies.
Limitations:
The case study is based on a single patient, limiting generalizability.
Long-term outcomes and treatment effectiveness were not fully assessed.
Conclusion:
This case highlights the recognition of Mauriac syndrome in pediatric patients with type 1 diabetes.
