Endocrine Abstracts

Introduction: Mauriac syndrome is a rare pathology characterized by the presence in an unbalanced type 1 diabetic (T1DM) of delayed stature-weight and puberty, and hepatomegaly with disturbance of the liver balance. We report a case of Mauriac syndrome in a young diabetic to recall the syndrom, as well as the diagnostic criteria.Medical observationThis was a 17-year-old patient, type 1 diabetic since the age of 2, with recurrent ketoacidosis. Admitted to the endocrinology department post ketoacidosis. On clinical examination, abdominal distension was noted, with a liver arrow greater than 12 cm. Height 136 cm (-4 DS) and weight 30 kg. The pubertal stage according to Tanner's classification was P2G2. Biology revealed poorly balanced diabetes (HbA1c 12%) and moderate cytolysis. Viral serologies and immunological work-up were negative; thyroid work-up was normal. Abdominal ultrasound showed´ hepatomegaly (hepatic arrow FH: 17cm) homogeneous and hyperechoic. Liver biopsy concluded glycogenic overload.ConclusionMauriac syndrome was first described by Mauriac in 1930, in children with type 1 diabetes mellitus presenting with stunted growth and puberty, hepatomegaly and elevated transaminases. Differential diagnoses include toxic causes, viral hepatitis, Wilson's disease, autoimmune hepatitis, celiac disease and minor glycogenosis. Paraclinical investigations confirm clinical suspicion and establish the diagnosis. The gold standard is liver biopsy, to rule out autoimmune hepatitis. Balanced diabetes allows normalization of liver function and recovery of growth and puberty.