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Korean J Pediatr 2015 June;58(6) :234-237.
Published online 2015 June 15.       
Hepatic glycogenosis in type 1 diabetes mellitus mimicking Mauriac syndrome
In Ah Jung, Won Kyoung Cho, Yeon Jin Jeon, Shin Hee Kim, Kyoung Soon Cho, So Hyun Park, Min Ho Jung, Byung-Kyu Suh
Department of Pediatrics, The Catholic University of Korea College of Medicine, Seoul, Korea
Corresponding Author: Byung-Kyu Suh ,Tel: +82-2-2258-6185, Fax: +82-2-532-6185, Email:
Copyright © 2015 by The Korean Pediatric Society
Hepatic glycogenosis in type 1 diabetes mellitus (DM) can be caused by poor glycemic control due to insulin deficiency, excessive insulin treatment for diabetic ketoacidosis, or excessive glucose administration to control hypoglycemia. Mauriac syndrome, which is characterized by hepatomegaly due to hepatic glycogenosis, growth retardation, delayed puberty, and Cushingoid features, is a rare diabetic complication. We report a case of hepatic glycogenosis mimicking Mauriac syndrome. A 14-year-old girl with poorly controlled type 1 DM was admitted to The Catholic University of Korea, Seoul St. Marys Hospital for abdominal pain and distension. Physical examination revealed hepatomegaly and a Cushingoid face. The growth rate of the patient had decreased, and she had not yet experienced menarche. Laboratory findings revealed elevated liver enzyme levels. A liver biopsy confirmed hepatic glycogenosis. Continuous glucose monitoring showed hyperglycemia after meals and frequent hypoglycemia before meals. To control hyperglycemia, we increased insulin dosage by using an insulin pump. In addition, we prescribed uncooked cornstarch to prevent hypoglycemia. After strict blood glucose control, the patients liver functions and size normalized. The patient subsequently underwent menarche. Hepatic glycogenosis is a complication of type 1 DM that is reversible with appropriate glycemic control.
Keywords: Type 1 diabetes mellitus | Diabetes complications | Hypoglycemia | Hepatomegaly
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Hepatic glycogenosis in a patient with poorly controlled type 1 diabetes mellitus  2009 November;52(11)
Type 2 diabetes mellitus and metabolic syndrome  2006 July;49(7)
Type 2 Diabetes Mellitus in Children  2002 May;45(5)
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