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Updated: Jun 12, 2026
05:07
Multimodality Diagnosis of Mesenteric Ischemia
Published on: July 21, 2023
Mauriac syndrome--a modern reality
1Academic Unit of Child Health, Sheffield Children's Hospital, Sheffield, UK. cjelder@doctors.org.uk
Summary
Mauriac syndrome, a rare complication of poorly controlled diabetes mellitus, presents with liver enlargement and growth issues in adolescents. Early detection through regular checks is crucial for managing this condition in young diabetic patients.
Area of Science:
- Pediatric Endocrinology
- Metabolic Disorders
- Hepatology
Background:
- Diabetes Mellitus (DM) management in adolescents requires vigilant monitoring for complications.
- Mauriac syndrome is a rare but significant condition associated with long-standing, poorly controlled DM.
- Early identification of endocrine and growth abnormalities is essential in pediatric diabetes care.
Observation:
- Two adolescent females with poorly controlled diabetes mellitus (DM) presented with significant hepatomegaly during routine annual reviews.
- Associated clinical features included short stature in one patient, delayed puberty, and a Cushingoid habitus.
- These findings prompted the diagnosis of Mauriac syndrome.
Findings:
- The cases highlight the clinical presentation of Mauriac syndrome, characterized by hepatomegaly and specific growth/developmental delays in adolescents with poorly controlled DM.
- Persistent high HbA1c levels (>9.5%) were a key indicator in identifying at-risk individuals.
- The syndrome underscores the systemic impact of chronic hyperglycemia on adolescent development.
Implications:
- Incorporating regular abdominal examinations into routine care for pediatric patients with persistently high HbA1c levels is recommended.
- This proactive screening approach can facilitate earlier diagnosis and intervention for Mauriac syndrome.
- Improved management strategies for poorly controlled diabetes in adolescents are needed to prevent such complications.
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