Mauriac syndrome in a 3-year-old boy.

Endocr Pract

Department of Pediatric Nephrology, Montefiore Medical Center, Bronx, New York, USA.

Published: April 2007

Objective: To report a case of Mauriac syndrome in a young child with poorly controlled type 1 diabetes mellitus.

Methods: We describe the typical features of Mauriac syndrome and review the clinical, laboratory, and ultrasound findings and follow-up results in our current patient.

Results: Hepatomegaly, growth impairment, and cushingoid features characterize Mauriac syndrome. Most frequently, it is seen in adolescents and young adults with a history of poor glycemic control. In our current patient, a 3-year-old boy with type 1 diabetes mellitus of 2 years' duration, Mauriac syndrome developed in the context of a disrupted social environment. His liver enzyme derangement, hepatomegaly, and growth improved once euglycemia was achieved.

Conclusion: Although Mauriac syndrome is currently uncommon, close monitoring of children with type 1 diabetes is important to maintain optimal glycemic control and prevent such an occurrence.

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Source
http://dx.doi.org/10.4158/EP.13.1.63DOI Listing

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