TY - JOUR AU - Lombardo, Fortunato AU - Passanisi, Stefano AU - Gasbarro, Albino AU - Tuccari, Giovanni AU - Ieni, Antonio AU - Salzano, Giuseppina PY - 2019 DA - 2019/01/07 TI - Hepatomegaly and type 1 diabetes: a clinical case of Mauriac’s syndrome JO - Italian Journal of Pediatrics SP - 3 VL - 45 IS - 1 AB - Hepatic glycogenosis is characterized by excessive glycogen accumulation in hepatocytes and represents a complication of poor controlled type 1 diabetes. It can be caused by excessive insulin doses or recurrent ketoacidosis episodes. Mauriac’s syndrome is a rare disease, which includes short stature, growth maturation delay, dyslipidemia, moon facies, protuberant abdomen, hepatomegaly with transaminase elevation. It has become even less common after the emergence of advances on diabetes treatment, but still exists. Recent reports described glycogenosis without the full spectrum of Mauriac’s syndrome in both adults and children with brittle diabetes. Clinical, laboratory and histological abnormalities are reversible with appropriate glycemic control. SN - 1824-7288 UR - https://doi.org/10.1186/s13052-018-0598-2 DO - 10.1186/s13052-018-0598-2 ID - Lombardo2019 ER -