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Case Reports in Critical Care
Volume 2016, Article ID 6072909, 4 pages
Case Report

A Rare Case of Persistent Lactic Acidosis in the ICU: Glycogenic Hepatopathy and Mauriac Syndrome

1Department of Critical Care Medicine, South Health Campus ICU, 4448 Front Street SE, Calgary, AB, Canada T3M 1M4
2Department of Critical Care Medicine, University of Calgary, AB, Canada

Received 11 April 2016; Accepted 27 June 2016

Academic Editor: Gerhard Pichler

Copyright © 2016 Kirsten S. Deemer and George F. Alvarez. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


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 exacerbation of lactic acidosis in a patient with glycogenic hepatopathy treated for diabetic ketoacidosis with high dose insulin and dextrose.