Korean J Pediatr.  2015 Jun;58(6):234-237. 10.3345/kjp.2015.58.6.234.

Hepatic glycogenosis in type 1 diabetes mellitus mimicking Mauriac syndrome

• Jung IA
• Cho WK
• Jeon YJ
• Kim SH
• Cho KS
• Park SH
• Jung MH
• Suh BK

• KMID: 1795121
• DOI: http://doi.org/10.3345/kjp.2015.58.6.234

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.

Keyword

Type 1 diabetes mellitus; Diabetes complications; Hypoglycemia; Hepatomegaly

MeSH Terms

Abdominal Pain
Adolescent
Biopsy
Blood Glucose
Diabetes Complications
Diabetes Mellitus, Type 1*
Diabetic Ketoacidosis
Female
Glucose
Glycogen Storage Disease*
Hepatomegaly
Humans
Hyperglycemia
Hypoglycemia
Insulin
Korea
Liver
Meals
Menarche
Physical Examination
Puberty, Delayed
Seoul
Starch
Blood Glucose
Glucose
Insulin
Starch