ECE2023 Eposter Presentations Diabetes, Obesity, Metabolism and Nutrition (355 abstracts)
Mauriac syndrome: A case report
Abir El Amri , Ramla Mizouri , Ines Bani , Rym Ben Othman , Nadia Ben Amor , Faten Mahjoub , Berriche Olfa & Jamoussi Henda
National Institute of Nutrition, Tunis, Tunisia
Introduction: Mauriac syndrome is typically characterised by poor glycaemic control in type 1 diabetics. It is associated with diabetes, hepatomegaly, disturbed liver function and severe staturo-weight delay.
Observation: We report the case of a young type 1 diabetic patient referred to our department for further management of a glycaemic imbalance. The patient was 16 years old, type 1 diabetic for 10 years on insulin analogues, with a history of vitiligo since the age of 5 years, febrile convulsion at the age of 3 years, put on valproic acid for 2 years. The clinical examination showed a delay in growth and development and puberty (< 4 DS, Tanner stage 1), hepatomegaly with a liver arrow at 17 cm. The biological work-up showed an HbA1c of 11%, hepatic cytolysis at 3 times normal with negative hepatitis B and C serology. A strictly normal hormonal work-up including FSH, LH, testosteronemia, cortisol and GH with normal GH stimulation test. Celiac disease serology was negative with no villous atrophy on oesogastroduodenal fibroscopy. Abdominal CT scan showed homogeneous hepatomegaly with regular liver contours. Based on these clinical, biological and radiological arguments, Mauriac syndrome was strongly suspected, hence the indication for a liver biopsy, which was refused by the patient.
Conclusion: Our observation illustrates the importance of evoking Mauriac’s syndrome when hepatomegaly with statural delay is discovered in a type 1 diabetic. The diagnosis of certainty is histological and the treatment is based on the control of diabetes.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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