title: A Rare Case of Persistent Lactic Acidosis in the ICU: Glycogenic Hepatopathy and Mauriac Syndrome.
abstract: 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 demonstratesexacerbationof lactic acidosis in a patient with glycogenic hepatopathy treated for diabetic ketoacidosis with high dose insulin and dextrose.
authors.affiliation: Department of Critical Care Medicine, South Health Campus ICU, 4448 Front Street SE, Calgary, AB, Canada T3M 1M4.
authors.forename: Kirsten S
authors.identifier: 0000-0002-3674-3522
authors.initials: KS
authors.lastname: Deemer
authors.affiliation: Department of Critical Care Medicine, University of Calgary, AB, Canada.
authors.forename: George F
authors.initials: GF
authors.lastname: Alvarez