We report an 18 years old female with hepatomegaly and elevated liver function tests. She had been diagnosed with Type 1 diabetes mellitus when she was 13 years old. She was referred to our division because of diabetic ketoacidosis. Evaluation of her previous records revealed the presence of liver function abnormalities for 4 months. At physical examination she had an enlarged liver. A1c level was 13%. She was treated initially with intravenous insulin. Following the achievement of acceptable plasma glucose levels, negative urinary ketone bodies and normal bicarbonate levels, insulin detemir and insulin aspart were suggested. Ultrasonography revealed the presence of hepatomegaly with 200 mm longitudinal axis. Viral hepatitis markers including hepatitis B, hepatitis C and CMV, ANA and AMA were negative. Serum alpha-1 AT, ceruloplasmin, copper, iron and ferritin levels were in normal ranges. Liver biopsy revealed glycogen deposition that was consistent with hepatic glycogenosis. Subsequent to the achievement of glycemic control, liver enzymes started to decline and 50% reduction was achieved in ALT in 1 week.
Hepatic glycogenosis is associated with poor metabolic control and high amount of insulin that is required to maintain euglycemia. Hepatic glycogenosis may resolve following glycemic control.
25 - 29 Apr 2009
European Society of Endocrinology