P-446: Mauriac syndrome in the modern era: still present in cases of poorly controlled type 1 diabetes
K De Souza Oliveira, A Alves Cabral, B De Melo Nunes, B Barone, J Veiga Moreira Vasconcellos, I Aguiar Paiva, L Ignachiti Francisco, D Christian de Arruda Costa, L Salomão Tyszler, J Elmor MainczykAbstract
Introduction
Mauriac syndrome is a rare complication of poorly controlled type 1 diabetes mellitus, characterized by growth failure, delayed puberty, and hepatomegaly. Although uncommon in the era of modern insulin therapy, it remains clinically relevant and potentially reversible.
Objectives
To describe a case of Mauriac syndrome and highlight its clinical features and reversibility with improved glycemic control.
Methods
We report a case from a tertiary referral center of a female patient diagnosed with type 1 diabetes at 8 years of age, with long-standing poor glycemic control.
Results
The patient, now 19 years old, had persistently elevated glycated hemoglobin (HbA1c) levels, reaching 12.2%. She developed short stature, delayed bone age, and delayed puberty (Tanner stage M1P1 at 11 years). Physical examination revealed hepatomegaly up to 4 cm below the right costal margin, confirmed by ultrasound showing increased liver volume with preserved architecture. Liver enzymes were intermittently elevated, and other causes of liver disease were excluded. She also developed Graves’ disease. Following improvement in glycemic control, HbA1c decreased to 8.1%, with normalization of liver enzymes, suggesting partial reversibility.
Conclusions
Mauriac syndrome remains a relevant and preventable complication of chronic poor glycemic control in pediatric type 1 diabetes. Early recognition and appropriate management can lead to significant clinical improvement.