Case Report: Case analysis of a juvenile type 1 diabetes mellitus patient with Mauriac syndrome
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By
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Rong Fu
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Mengmeng Yang
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Yanyan Guo
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Yiyun Huang
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Xiaoze Li
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Mingying Zhang
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July 15, 2026
Clinical Scorecard: Clinical Case Study: Evaluation of a Young Patient with Type 1 Diabetes Mellitus and Mauriac Syndrome
At a Glance
| Category | Detail |
|---|
| Condition | Mauriac Syndrome |
| Key Mechanisms | Inadequate management of type 1 diabetes leading to glycogenic hepatopathy and metabolic complications. |
| Target Population | Pediatric and adolescent patients with type 1 diabetes mellitus. |
| Care Setting | Clinical case management in a hospital setting. |
Key Highlights
- Patient presented with abdominal pain and vomiting, leading to a diagnosis of DKA.
- Liver biopsy confirmed glycogenic hepatopathy, establishing Mauriac syndrome.
- Patient had a history of recurrent DKA and poor insulin adherence.
- Treatment included continuous intravenous insulin therapy and dietary guidance.
- Patient was readmitted two months later for DKA, with persistent hepatomegaly.
Guideline-Based Recommendations
Diagnosis
- Comprehensive evaluation including medical history, physical examination, and laboratory tests.
Management
- Initiate continuous intravenous insulin therapy and provide diabetes education.
Monitoring & Follow-up
- Regular monitoring of liver function and blood glucose levels.
Risks
- Potential for recurrent DKA and long-term health impacts due to Mauriac syndrome.
Patient & Prescribing Data
14-year-old female with type 1 diabetes and Mauriac syndrome.
Initial treatment with continuous intravenous insulin, later switched to a subcutaneous regimen.
Clinical Best Practices
- Monitor liver function and growth parameters in pediatric patients with long-standing type 1 diabetes.
- Consider Mauriac syndrome in patients with hepatomegaly and abnormal liver function tests.
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