Hepatic glycogenosis in type 1 diabetes mellitus mimicking Mauriac syndrome

• Published in: Clinical and experimental pediatrics Vol. 58; no. 6; pp. 234 - 237
• Main Authors: Jung, In Ah, Cho, Won Kyoung, Jeon, Yeon Jin, Kim, Shin Hee, Cho, Kyoung Soon, Park, So Hyun, Jung, Min Ho, Suh, Byung-Kyu
• Format: Journal Article
• Language: English
• Published: Korea (South) Clinical and Experimental Pediatics / Korean Pediatric Society 01.06.2015; The Korean Pediatric Society; Korean Pediatric Society; 대한소아청소년과학회
• Subjects: Abdomen; Antibodies; Biopsy; Case Report; Conflicts of interest; Diabetes; Diabetes complications; Fatty acids; Glucose; Hemoglobin; Hepatitis; Hepatomegaly; Hormones; Hyperglycemia; Hypoglycemia; Insulin resistance; Insulin-like growth factors; Liver; Meals; Metabolism; Ovaries; Patients; Puberty; Type 1 diabetes mellitus; 소아과학; Diabetes complications; Hepatomegaly; Hypoglycemia; Type 1 diabetes mellitus
• ISSN: 1738-1061; 2092-7258; 2713-4148
• DOI: 10.3345/kjp.2015.58.6.234

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. Mary's 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 patient's 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.