Liver Dysfunction due to Hepatic Glycogenosis in a Girl with Type 1 Diabetes / 대한소아내분비학회지

ABSTRACT

Hepatic glycogenosis is an under-recognized cause of hepatomegaly and elevated serum aminotransferase levels in patients with type 1 diabetes; further, most cases of hepatic glycogenosis are reported to be associated with poor glycemic control. In this report, we describe the case of a 12-year-old girl with hepatic glycogenosis, who presented with elevated liver enzymes. She was diagnosed with type 1 diabetes at the age of 6 years, and her diabetes was very "brittle" with recurrent episodes of hypoglycemic attacks. Her recent hemoglobin A1C (HbA1c) level was 8.4%, and the average HbA1c level during the last 2 years was 8.7%. Her aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels were persistently elevated during the last year, up to 700 and 258 U/L, respectively. Her growth rate and pubertal development were normal. Her serum was negative for hepatitis viral markers, and the plasma levels of ceruloplasmin and ferritin were also normal. Ultrasound examination revealed hepatomegaly with increased hepatic echogenicity. Liver biopsy demonstrated irregular glycogen deposition in hepatocytes. Recurrent hypoglycemia was resolved with continuous subcutaneous insulin infusion, and after 3 weeks, her AST and ALT levels decreased to 47 and 33 U/L, respectively. We conclude that hepatic glycogenosis should be suspected as a cause of abnormal liver function tests in patients with poorly controlled or brittle type 1 diabetes. After excluding other causes of hepatic dysfunction, a 1-month trial for achieving improved glycemic control, while avoiding hypoglycemia, is recommended before proceeding with invasive investigation of the patient.