Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Hepatic glycogenosis in type 1 diabetes mellitus mimicking Mauriac syndrome

  • Jung, In Ah (Department of Pediatrics, The Catholic University of Korea College of Medicine) ;
  • Cho, Won Kyoung (Department of Pediatrics, The Catholic University of Korea College of Medicine) ;
  • Jeon, Yeon Jin (Department of Pediatrics, The Catholic University of Korea College of Medicine) ;
  • Kim, Shin Hee (Department of Pediatrics, The Catholic University of Korea College of Medicine) ;
  • Cho, Kyoung Soon (Department of Pediatrics, The Catholic University of Korea College of Medicine) ;
  • Park, So Hyun (Department of Pediatrics, The Catholic University of Korea College of Medicine) ;
  • Jung, Min Ho (Department of Pediatrics, The Catholic University of Korea College of Medicine) ;
  • Suh, Byung-Kyu (Department of Pediatrics, The Catholic University of Korea College of Medicine)
  • Received : 2014.09.12
  • Accepted : 2015.01.05
  • Published : 2015.06.10
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Abstract

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.

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References

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