Deemer Kirsten S, Alvarez George F
Department of Critical Care Medicine, South Health Campus ICU, 4448 Front Street SE, Calgary, AB, Canada T3M 1M4.
Department of Critical Care Medicine, University of Calgary, AB, Canada.
Case Rep Crit Care. 2016;2016:6072909. doi: 10.1155/2016/6072909. Epub 2016 Jul 25.
Mauriac syndrome is a rare disorder that can present with the single feature of glycogenic hepatopathy in children and adults with poorly controlled diabetes mellitus. An often underrecognized finding of glycogenic hepatopathy is lactic acidosis and hyperlactatemia. Primary treatment of glycogenic hepatopathy is improved long-term blood glucose control. Resolution of symptoms and hepatomegaly will occur with improvement in hemoglobin A1C. We present here a case of a young adult female presenting to the intensive care unit with Mauriac syndrome. This case demonstrates of lactic acidosis in a patient with glycogenic hepatopathy treated for diabetic ketoacidosis with high dose insulin and dextrose.
莫里亚克综合征是一种罕见的疾病，在糖尿病控制不佳的儿童和成人中，可能仅表现为糖原性肝病这一特征。糖原性肝病一个常未被充分认识的表现是乳酸酸中毒和高乳酸血症。糖原性肝病的主要治疗方法是改善长期血糖控制。随着糖化血红蛋白水平的改善，症状和肝肿大将会缓解。我们在此报告一例年轻成年女性因莫里亚克综合征入住重症监护病房的病例。该病例展示了一名患有糖原性肝病的患者在接受高剂量胰岛素和葡萄糖治疗糖尿病酮症酸中毒时出现乳酸酸中毒的情况。
